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Sample records for models including spinocerebellar

  1. Ataxias and Cerebellar or Spinocerebellar Degeneration

    Science.gov (United States)

    ... and conducts a broad range of basic and clinical research on cerebellar and spinocerebellar degeneration, including work aimed at finding the cause(s) of ataxias and ways to ... Publications Definition Ataxia ...

  2. Clinical neurogenetics: autosomal dominant spinocerebellar ataxia.

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    Shakkottai, Vikram G; Fogel, Brent L

    2013-11-01

    The autosomal dominant spinocerebellar ataxias are a diverse and clinically heterogeneous group of disorders characterized by degeneration and dysfunction of the cerebellum and its associated pathways. Clinical and diagnostic evaluation can be challenging because of phenotypic overlap among causes, and a stratified and systematic approach is essential. Recent advances include the identification of additional genes causing dominant genetic ataxia, a better understanding of cellular pathogenesis in several disorders, the generation of new disease models that may stimulate development of new therapies, and the use of new DNA sequencing technologies, including whole-exome sequencing, to improve diagnosis. Copyright © 2013 Elsevier Inc. All rights reserved.

  3. Research progress of spinocerebellar ataxia type 1

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    Lin-wei ZHANG

    2014-05-01

    Full Text Available Spinocerebellar ataxia type 1 (SCA1 is a kind of autosomal dominant genetic neurodegenerative disorder. To date, the pathogenesis of SCA1 remains unclear. Studies in numerous SCA1 experimental models, including transgenic mice, transgenic drosophila and induced pluripotent stem cells, have shown that phosphorylation of S776 in mutant ataxin-1, molecular chaperones, ubiquitin-proteasome system and down-regulation of several components of RAS-MAPK-MSK1 pathway may involve in the pathogenesis of SCA1. In this review, the clinical and pathological features of SCA1, and the latest advances of pathogenesis, model systems and therapeutic exploration will be briefly summarized. doi: 10.3969/j.issn.1672-6731.2014.05.017

  4. Phenotype variability and early onset ataxia symptoms in spinocerebellar ataxia type 7: comparison and correlation with other spinocerebellar ataxias

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    Marcus Vinicius Cristino de Albuquerque

    2015-01-01

    Full Text Available The spinocerebellar ataxias (SCA are a group of neurodegenerative disorders characterized by heterogeneous clinical presentation. Spinocerebellar ataxia type 7 (SCA7 is caused by an abnormal CAG repeat expansion and includes cerebellar signs associated with visual loss and ophthalmoplegia. Marked anticipation and dynamic mutation is observed in SCA7. Moreover, phenotype variability and very early onset of symptoms may occur. In this article, a large series of Brazilian patients with different SCA subtypes was evaluated, and we compared the age of onset of SCA7 with other SCA. From the 26 patients with SCA7, 4 manifested their symptoms before 10-year-old. Also, occasionally the parents may have the onset of symptoms after their children. In conclusion, our study highlights the genetic anticipation phenomenon that occurs in SCA7 families. Patients with very early onset ataxia in the context of a remarkable family history, must be considered and tested for SCA7.

  5. Properties of bilateral spinocerebellar activation of cerebellar cortical neurons

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    Pontus eGeborek

    2014-10-01

    Full Text Available We aimed to explore the cerebellar cortical inputs from two spinocerebellar pathways, the spinal border cell-component of the ventral spinocerebellar tract (SBC-VSCT and the dorsal spinocerebellar tract (DSCT, respectively, in the sublobule C1 of the cerebellar posterior lobe. The two pathways were activated by electrical stimulation of the contralateral lateral funiculus (coLF and the ipsilateral LF (iLF at lower thoracic levels. Most granule cells in sublobule C1 did not respond at all but part of the granule cell population displayed high-intensity responses to either coLF or iLF stimulation. As a rule, Golgi cells and Purkinje cell simple spikes responded to input from both LFs, although Golgi cells could be more selective. In addition, a small population of granule cells responded to input from both the coLF and the iLF. However, in these cases, similarities in the temporal topography and magnitude of the responses suggested that the same axons were stimulated from the two LFs, i.e. that the axons of individual spinocerebellar neurons could be present in both funiculi. This was also confirmed for a population of spinal neurons located within known locations of SBC-VSCT neurons and dorsal horn DSCT neurons. We conclude that bilateral spinocerebellar responses can occur in cerebellar granule cells, but the VSCT and DSCT systems that provide the input can also be organized bilaterally. The implications for the traditional functional separation of VSCT and DSCT systems and the issue whether granule cells primarily integrate functionally similar information or not are discussed.

  6. Origin, course, and laterality of spinocerebellar axons in the North American opossum, Didelphis virginiana.

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    Terman, J R; Wang, X M; Martin, G F

    1998-08-01

    Spinocerebellar axons have been studied extensively in placental mammals, but there have been no full reports on their origin, laterality, or spinal course in any marsupial. We have used the North American opossum (Didelphis virginiana) to obtain such information and to ask whether any spinocerebellar neurons innervate both the anterior and posterior lobes of the cerebellum through axonal collaterals. To identify spinal neurons that project to the cerebellum, we employed the retrograde transport of Fluoro-Gold (FG) from the anterior lobe, the main target of spinocerebellar axons. In some cases, cerebellar injections of FG were combined with hemisections of the rostral cervical or midthoracic spinal cord, so that laterality of spinocerebellar connections could be established. To determine whether single neurons project to both the anterior lobe and the posterior lobe, injections of Fast Blue (FB) into the anterior lobe were combined with injections of Diamidino yellow (DY) or rhodamine B dextran (RBD) into the posterior lobe, or vice versa. Following injections of FG into the anterior lobe, neurons were labeled throughout the length of the spinal cord, which differed in laminar distribution and laterality of their projections. Among other areas, neurons were labeled in the central cervical nucleus, the nucleus centrobasalis, Clarke's nucleus, the dorsal horn dorsal spinocerebellar tract area, the spinal border region, and Stilling's nucleus. When anterior lobe injections of FB were combined with injections of RBD or DY into the posterior lobe, or vice versa, some double-labeled neurons were present in all major spinocerebellar groups. Cerebellar injections of FG also retrogradely labeled spinocerebellar axons, allowing us to document their locations in the gray matter as well as within the periphery of the lateral and ventral funiculi at all spinal levels. A few spinocerebellar axons also were found in the dorsal funiculus (a dorsal column-spinocerebellar tract

  7. Missense mutations in ITPR1 cause autosomal dominant congenital nonprogressive spinocerebellar ataxia

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    Huang Lijia

    2012-09-01

    Full Text Available Abstract Background Congenital nonprogressive spinocerebellar ataxia is characterized by early gross motor delay, hypotonia, gait ataxia, mild dysarthria and dysmetria. The clinical presentation remains fairly stable and may be associated with cerebellar atrophy. To date, only a few families with autosomal dominant congenital nonprogressive spinocerebellar ataxia have been reported. Linkage to 3pter was demonstrated in one large Australian family and this locus was designated spinocerebellar ataxia type 29. The objective of this study is to describe an unreported Canadian family with autosomal dominant congenital nonprogressive spinocerebellar ataxia and to identify the underlying genetic causes in this family and the original Australian family. Methods and Results Exome sequencing was performed for the Australian family, resulting in the identification of a heterozygous mutation in the ITPR1 gene. For the Canadian family, genotyping with microsatellite markers and Sanger sequencing of ITPR1 gene were performed; a heterozygous missense mutation in ITPR1 was identified. Conclusions ITPR1 encodes inositol 1,4,5-trisphosphate receptor, type 1, a ligand-gated ion channel that mediates calcium release from the endoplasmic reticulum. Deletions of ITPR1 are known to cause spinocerebellar ataxia type 15, a distinct and very slowly progressive form of cerebellar ataxia with onset in adulthood. Our study demonstrates for the first time that, in addition to spinocerebellar ataxia type 15, alteration of ITPR1 function can cause a distinct congenital nonprogressive ataxia; highlighting important clinical heterogeneity associated with the ITPR1 gene and a significant role of the ITPR1-related pathway in the development and maintenance of the normal functions of the cerebellum.

  8. Maculopathy and spinocerebellar ataxia type 1

    DEFF Research Database (Denmark)

    Lebranchu, Pierre; Le Meur, Guylène; Magot, Armelle

    2013-01-01

    Autosomal dominant cerebellar ataxia is a rare heterogeneous group of diseases characterized by cerebellar symptoms, often associated with other multisystemic signs. Mild optic neuropathy has been associated with spinocerebellar ataxia type 1 (SCA1), but macular dysfunction has been reported...

  9. Simulating spinal border cells and cerebellar granule cells under locomotion--a case study of spinocerebellar information processing.

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    Anton Spanne

    Full Text Available The spinocerebellar systems are essential for the brain in the performance of coordinated movements, but our knowledge about the spinocerebellar interactions is very limited. Recently, several crucial pieces of information have been acquired for the spinal border cell (SBC component of the ventral spinocerebellar tract (VSCT, as well as the effects of SBC mossy fiber activation in granule cells of the cerebellar cortex. SBCs receive monosynaptic input from the reticulospinal tract (RST, which is an important driving system under locomotion, and disynaptic inhibition from Ib muscle afferents. The patterns of activity of RST neurons and Ib afferents under locomotion are known. The activity of VSCT neurons under fictive locomotion, i.e. without sensory feedback, is also known, but there is little information on how these neurons behave under actual locomotion and for cerebellar granule cells receiving SBC input this is completely unknown. But the available information makes it possible to simulate the interactions between the spinal and cerebellar neuronal circuitries with a relatively large set of biological constraints. Using a model of the various neuronal elements and the network they compose, we simulated the modulation of the SBCs and their target granule cells under locomotion and hence generated testable predictions of their general pattern of modulation under this condition. This particular system offers a unique opportunity to simulate these interactions with a limited number of assumptions, which helps making the model biologically plausible. Similar principles of information processing may be expected to apply to all spinocerebellar systems.

  10. Spinocerebellar ataxia-10 with paranoid schizophrenia

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    Bhavesh Trikamji

    2015-01-01

    Full Text Available Spino-cerebellar ataxia type 10 (SCA10 is an autosomal dominant disorder that is characterized by cerebellar ataxia, seizures and nystagmus with a fragmented pursuit. Schizophrenia has been reported with SCAs 1 and 2 yet in SCA 10, psychiatric manifestations are uncommon. We report a Hispanic family involving a father and his four children with SCA10 genetic mutation. Two of his children, a 20-year-old female and a 23-year-old male, presented with gradually progressive spino-cerebellar ataxia and paranoid schizophrenia. Neurological examination revealed ocular dysmetria, dysdiadokinesia, impaired finger-to-nose exam, gait ataxia and hyperreflexia in both the cases. Additionally, they had a history of psychosis with destructive behavior, depression and paranoid delusions with auditory hallucinations. Serology and CSF studies were unremarkable and MRI brain revealed cerebellar volume loss. Ultimately, a test for ATAXIN-10 mutation was positive thus confirming the diagnosis of SCA10 in father and his four children. We now endeavor to investigate the association between schizophrenia and SCA10.

  11. Clinical and CT features in cases of spinocerebellar degeneration occurring at infancy

    International Nuclear Information System (INIS)

    Eda, Isematsu; Nakano, Chizuko; Kawahara, Hitoshi; Takeshita, Kenzo

    1985-01-01

    Clinical symptoms and CT findings were evaluated in 7 patients in whom symptoms compatible with spinocerebellar degeneration had occurred at infancy. CT findings included atrophy of the vermis, hemisphere and brain-stem, and dilation of the forth ventricle. These were in accordance with those reported in adult cases of spinocerebellar degeneration. There was some correlation between the severity of clinical symptoms and CT findings. Clinical lesions tended to coincide with CT findings. None of the patients had typical adult types such as delayed cerebello-cortical atrophy and olivo-pontocerebellar atrophy. There were great differences in clinical symptoms among the patients. Many of the patients had complications such as pigmentary degeneration of the retina, optic atrophy and cataract, which are rare in adult cases. It is therefore considered impossible to categorize these cases, except for two cases of familial convulsive paraplegia, as a given type. (Namekawa, K.)

  12. Organization of spinocerebellar projection map in three types of agranular cerebellum: Purkinje cells vs. granule cells as organizer element

    International Nuclear Information System (INIS)

    Arsenio Nunes, M.L.; Sotelo, C.; Wehrle, R.

    1988-01-01

    The organization of the spinocerebellar projection was analysed by the anterograde axonal WGA-HRP (horseradish peroxidase-wheat germ agglutinin conjugate) tracing method in three different types of agranular cerebellar cortex either induced experimentally by X-irradiation or occurring spontaneously in weaver (wv/wv) and staggerer (sg/sg) mutant mice. The results of this study show that in the X-irradiated rat and weaver mouse, in both of which the granule cells are directly affected and die early in development, the spinal axons reproduce, with few differences, the normal spinocerebellar pattern. Conversely, in staggerer mouse, in which the Purkinje cells are intrinsically affected and granule neurons do not seem to be primarily perturbed by the staggerer gene action, the spinocerebellar organization is severely modified. These findings appear somewhat paradoxical because if granule cells, the synaptic targets of mossy spinocerebellar fibers, were necessary for the organization of spinocerebellar projection, the staggerer cerebellum would exhibit a much more normal projectional map than the weaver and the X-irradiated cerebella. It is, therefore, obvious that granule cells, and even specific synaptogenesis, are not essential for the establishment of the normal spinocerebellar topography. On the other hand, the fact that the Purkinje cells are primarily affected in the unique agranular cortex in which the spinocerebellar organization is severely modified suggests that these neurons could be the main element in the organization of the spinocerebellar projection map. This hypothesis is discussed in correlation with already-reported findings on the zonation of the cerebellar cortex by biochemically different clusters of Purkinje cells

  13. Speech in spinocerebellar ataxia.

    Science.gov (United States)

    Schalling, Ellika; Hartelius, Lena

    2013-12-01

    Spinocerebellar ataxias (SCAs) are a heterogeneous group of autosomal dominant cerebellar ataxias clinically characterized by progressive ataxia, dysarthria and a range of other concomitant neurological symptoms. Only a few studies include detailed characterization of speech symptoms in SCA. Speech symptoms in SCA resemble ataxic dysarthria but symptoms related to phonation may be more prominent. One study to date has shown an association between differences in speech and voice symptoms related to genotype. More studies of speech and voice phenotypes are motivated, to possibly aid in clinical diagnosis. In addition, instrumental speech analysis has been demonstrated to be a reliable measure that may be used to monitor disease progression or therapy outcomes in possible future pharmacological treatments. Intervention by speech and language pathologists should go beyond assessment. Clinical guidelines for management of speech, communication and swallowing need to be developed for individuals with progressive cerebellar ataxia. Copyright © 2013 Elsevier Inc. All rights reserved.

  14. Direct and indirect spino-cerebellar pathways: shared ideas but different functions in motor control

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    Juan eJiang

    2015-07-01

    Full Text Available The impressive precision of mammalian limb movements relies on internal feedback pathways that convey information about ongoing motor output to cerebellar circuits. The spino-cerebellar tracts (SCT in the cervical, thoracic and lumbar spinal cord have long been considered canonical neural substrates for the conveyance of internal feedback signals. Here we consider the distinct features of an indirect spino-cerebellar route, via the brainstem lateral reticular nucleus (LRN, and the implications of this pre-cerebellar ‘detour’ for the execution and evolution of limb motor control. Both direct and indirect spino-cerebellar pathways signal spinal interneuronal activity to the cerebellum during movements, but evidence suggests that direct SCT neurons are mainly modulated by rhythmic activity, whereas the LRN also receives information from systems active during postural adjustment, reaching and grasping. Thus, while direct and indirect spino-cerebellar circuits can both be regarded as internal copy pathways, it seems likely that the direct system is principally dedicated to rhythmic motor acts like locomotion, while the indirect system also provides a means of pre-cerebellar integration relevant to the execution and coordination of de

  15. Motor Decline in Clinically Presymptomatic Spinocerebellar Ataxia Type 2 Gene Carriers

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    Velázquez-Perez, Luis; Díaz, Rosalinda; Pérez-González, Ruth; Canales, Nalia; Rodríguez-Labrada, Roberto; Medrano, Jacquelín; Sánchez, Gilberto; Almaguer-Mederos, Luis; Torres, Cira; Fernandez-Ruiz, Juan

    2009-01-01

    Background Motor deficits are a critical component of the clinical characteristics of patients with spinocerebellar ataxia type 2. However, there is no current information on the preclinical manifestation of those motor deficits in presymptomatic gene carriers. To further understand and characterize the onset of the clinical manifestation in this disease, we tested presymptomatic spinocerebellar ataxia type 2 gene carriers, and volunteers, in a task that evaluates their motor performance and their motor learning capabilities. Methods and Findings 28 presymptomatic spinocerebellar ataxia type 2 gene carriers and an equal number of control volunteers matched for age and gender participated in the study. Both groups were tested in a prism adaptation task known to be sensible to both motor performance and visuomotor learning deficits. Our results clearly show that although motor learning capabilities are intact, motor performance deficits are present even years before the clinical manifestation of the disease start. Conclusions The results show a clear deficit in motor performance that can be detected years before the clinical onset of the disease. This motor performance deficit appears before any motor learning or clinical manifestations of the disease. These observations identify the performance coefficient as an objective and quantitative physiological biomarker that could be useful to assess the efficiency of different therapeutic agents. PMID:19401771

  16. Automated home cage assessment shows behavioral changes in a transgenic mouse model of spinocerebellar ataxia type 17.

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    Portal, Esteban; Riess, Olaf; Nguyen, Huu Phuc

    2013-08-01

    Spinocerebellar Ataxia type 17 (SCA17) is an autosomal dominantly inherited, neurodegenerative disease characterized by ataxia, involuntary movements, and dementia. A novel SCA17 mouse model having a 71 polyglutamine repeat expansion in the TATA-binding protein (TBP) has shown age related motor deficit using a classic motor test, yet concomitant weight increase might be a confounding factor for this measurement. In this study we used an automated home cage system to test several motor readouts for this same model to confirm pathological behavior results and evaluate benefits of automated home cage in behavior phenotyping. Our results confirm motor deficits in the Tbp/Q71 mice and present previously unrecognized behavioral characteristics obtained from the automated home cage, indicating its use for high-throughput screening and testing, e.g. of therapeutic compounds. Copyright © 2013 Elsevier B.V. All rights reserved.

  17. Excitatory inputs to four types of spinocerebellar tract neurons in the cat and the rat thoraco-lumbar spinal cord

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    Shrestha, Sony Shakya; Bannatyne, B Anne; Jankowska, Elzbieta; Hammar, Ingela; Nilsson, Elin; Maxwell, David J

    2012-01-01

    The cerebellum receives information from the hindlimbs through several populations of spinocerebellar tract neurons. Although the role of these neurons has been established in electrophysiological experiments, the relative contribution of afferent fibres and central neurons to their excitatory input has only been estimated approximately so far. Taking advantage of differences in the immunohistochemistry of glutamatergic terminals of peripheral afferents and of central neurons (with vesicular glutamate transporters VGLUT1 or VGLUT2, respectively), we compared sources of excitatory input to four populations of spinocerebellar neurons in the thoraco-lumbar spinal cord: dorsal spinocerebellar tract neurons located in Clarke's column (ccDSCT) and in the dorsal horn (dhDSCT) and ventral spinocerebellar tract (VSCT) neurons including spinal border (SB) neurons. This was done on 22 electrophysiologically identified intracellularly labelled neurons in cats and on 80 neurons labelled by retrograde transport of cholera toxin b subunit injected into the cerebellum of rats. In both species distribution of antibodies against VGLUT1 and VGLUT2 on SB neurons (which have dominating inhibitory input from limb muscles), revealed very few VGLUT1 contacts and remarkably high numbers of VGLUT2 contacts. In VSCT neurons with excitatory afferent input, the number of VGLUT1 contacts was relatively high although VGLUT2 contacts likewise dominated, while the proportions of VGLUT1 and VGLUT2 immunoreactive terminals were the reverse on the two populations of DSCT neurons. These findings provide morphological evidence that SB neurons principally receive excitatory inputs from central neurons and provide the cerebellum with information regarding central neuronal activity. PMID:22371473

  18. Early symptoms in spinocerebellar ataxia type 1, 2, 3, and 6.

    NARCIS (Netherlands)

    Globas, C.; Montcel, S.T. du; Baliko, L.; Boesch, S.; Depondt, C.; DiDonato, S.; Durr, A.; Filla, A.; Klockgether, T.; Mariotti, C.; Melegh, B.; Rakowicz, M.; Ribai, P.; Rola, R.; Schmitz-Hubsch, T.; Szymanski, S.; Timmann, D.; Warrenburg, B.P.C. van de; Bauer, P.; Schols, L.

    2008-01-01

    Onset of genetically determined neurodegenerative diseases is difficult to specify because of their insidious and slowly progressive nature. This is especially true for spinocerebellar ataxia (SCA) because of varying affection of many parts of the nervous system and huge variability of symptoms. We

  19. [Voluntary postural control learning with a use of visual bio-feedback in patients with spinocerebellar degenerations].

    Science.gov (United States)

    Ustinova, K I; Ioffe, M E; Chernikova, L A; Kulikov, M A; Illarioshkin, S N; Markova, E D

    2004-01-01

    The study aimed at evaluation of possibility and features of voluntary postural control learning using biofeedback from a force platform in patients with spinocerebellar ataxias. Thirty-seven patients with different forms of spinocerebellar degenerations and 13 age-matched healthy subjects were trained to shift the center of pressure (CP) during several stabilographic computer games which tested an ability to learn 2 different types of voluntary postural control: general strategy and precise coordination of CP shifting. Despite the disturbances of static posture and ability for voluntary control of CP position, patients with spinocerebellar degenerations can learn to control a vertical posture using biofeedback on stabilogram. In contrast to healthy subjects, improvement of coordination in the training process does not exert a significant influence on the static posture characteristics, in particular on lateral CP oscillations. The results obtained suggest involvement of the cerebellum in both types of postural control that distinguishes them from pathology caused by motor cortex and nigro-striatal system involved only in one type of postural control.

  20. Spinocerebellar ataxia type 6: MRI of three Japanese patients

    International Nuclear Information System (INIS)

    Satoh, J.I.; Tokumoto, H.; Yukitake, M.; Matsui, M.; Kuroda, Y.; Matsuyama, Z.; Kawakami, H.; Nakamura, S.

    1998-01-01

    We describe the MRI findings in three Japanese patients with spinocerebellar ataxia type 6 (SCA6) in which a polymorphic CAG repeat was identified in the gene encoding the α 1A voltage-dependent P/Q-type Ca 2+ channel subunit (CACNL1A4). All showed slowly progressive cerebellar ataxia and mild pyramidal signs. Neuroradiologically, they had moderate cerebellar atrophy, most prominently in the superior vermis, whereas the brain stem appeared to be spared. No abnormal signal intensity was identified. (orig.)

  1. Spinocerebellar ataxia type 3 (Machado-Joseph disease) : severe destruction of the lateral reticular nucleus

    NARCIS (Netherlands)

    Rub, U; de Vos, RAI; Schultz, C; Brunt, ER; Paulson, H; Braak, H

    The lateral reticular nucleus (LRT) of the medulla oblongata is a precerebellar nucleus involved in proprioception and somatomotor automatisms. We investigated this nucleus in five individuals with clinically diagnosed and genetically confirmed spinocerebellar ataxia type 3 (SCA3, Machado-Joseph

  2. Induced pluripotent stem cell - derived neurons for the study of spinocerebellar ataxia type 3

    DEFF Research Database (Denmark)

    Hansen, Susanne Kofoed; Stummann, Tina C.; Madsen, Helena Borland

    2016-01-01

    The neurodegenerative disease spinocerebellar ataxia type 3 (SCA3) is caused by a CAG-repeat expansion in the ATXN3 gene. In this study, induced pluripotent stem cell (iPSC) lines were established from two SCA3 patients. Dermal fibroblasts were reprogrammed using an integration-free method...

  3. MRI of the spinocerebellar degeneration (multiple system atrophy, Holmes type, and Menzel-Joseph type)

    International Nuclear Information System (INIS)

    Mukai, Eiichiro; Makino, Naoki.

    1991-01-01

    We have analyzed MRI in 33 patients with several forms of spinocerebellar degeneration; 17 with multiple system atrophy, 10 with Holmes type, and 6 with Menzel-Joseph type. The MRIs were obtained using a 1.5-T GEMR System. Patients with multiple system atrophy demonstrated: atrophy of the brain stem, particularly basis pontis; decreased signal intensity of the white matter of pons; atrophy of the white matter of cerebellum; atrophy and decreased signal intensity of the putamen, particularly along their lateral and posterior portions; and atrophy of the cerebrum. Patients with Holmes type showed: atrophy of the cerebellum; atrophy of the vermis more than hemispheres; and nuclei of the cerebellum with no decreased intensity on T 2 -weighted sequences. Patients with Menzel-Joseph type demonstrated moderate atrophy of the brain stem and mild atrophy of the white matter of cerebellum. MRI is a useful diagnostic tool in the management of the spinocerebellar degeneration. (author)

  4. Vascular Risk Factors and Clinical Progression in Spinocerebellar Ataxias

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    Raymond Y. Lo

    2015-02-01

    Full Text Available Background: The contributions of vascular risk factors to spinocerebellar ataxia (SCA are not known.Methods: We studied 319 participants with SCA 1, 2, 3, and 6 and repeatedly measured clinical severity using the Scale for Assessment and Rating of Ataxia (SARA for 2 years. Vascular risk factors were summarized by CHA2DS2-VASc scores as the vascular risk factor index. We employed regression models to study the effects of vascular risk factors on ataxia onset and progression after adjusting for age, sex, and pathological CAG repeats. Our secondary analyses took hyperlipidemia into account.Results: Nearly 60% of SCA participants were at low vascular risks with CHA2DS2-VASc = 0, and 31% scored 2 or greater. Higher CHA2DS2-VASc scores were not associated with either earlier onset or faster progression of ataxia. These findings were not altered after accounting for hyperlipidemia. Discussion: Vascular risks are not common in SCAs and are not associated with earlier onset or faster ataxia progression.

  5. Spinocerebellar Ataxia Type 6 Protein Aggregates Cause Deficits in Motor Learning and Cerebellar Plasticity

    NARCIS (Netherlands)

    Mark, Melanie D; Krause, Martin; Boele, Henk-Jan; Kruse, Wolfgang; Pollok, Stefan; Kuner, Thomas; Dalkara, Deniz; Koekkoek, Sebastiaan; De Zeeuw, Chris I; Herlitze, Stefan

    2015-01-01

    Spinocerebellar ataxia type 6 (SCA6) is linked to poly-glutamine (polyQ) within the C terminus (CT) of the pore-forming subunits of P/Q-type Ca(2+) channels (Cav2.1) and is characterized by CT protein aggregates found in cerebellar Purkinje cells (PCs). One hypothesis regarding SCA6 disease is that

  6. Evidence of oxidative stress and mitochondrial dysfunction in spinocerebellar ataxia type 2 (SCA2) patient fibroblasts

    DEFF Research Database (Denmark)

    Cornelius, Nanna; Wardman, Jonathan H; Hargreaves, Iain P

    2017-01-01

    Spinocerebellar ataxia type 2 (SCA2) is a rare neurodegenerative disorder caused by a CAG repeat expansion in the ataxin-2 gene. We show increased oxidative stress, abnormalities in the antioxidant system, changes in complexes involved in oxidative phosphorylation and changes in mitochondrial mor...

  7. Caffeine alleviates progressive motor deficits in a transgenic mouse model of spinocerebellar ataxia.

    Science.gov (United States)

    Gonçalves, Nélio; Simões, Ana T; Prediger, Rui D; Hirai, Hirokazu; Cunha, Rodrigo A; Pereira de Almeida, Luís

    2017-03-01

    Machado-Joseph disease (MJD) is a neurodegenerative spinocerebellar ataxia (SCA) associated with an expanded polyglutamine tract within ataxin-3 for which there is currently no available therapy. We previously showed that caffeine, a nonselective adenosine receptor antagonist, delays the appearance of striatal damage resulting from expression of full-length mutant ataxin-3. Here we investigated the ability of caffeine to alleviate behavioral deficits and cerebellar neuropathology in transgenic mice with a severe ataxia resulting from expression of a truncated fragment of polyglutamine-expanded ataxin-3 in Purkinje cells. Control and transgenic c57Bl6 mice expressing in the mouse cerebella a truncated form of human ataxin-3 with 69 glutamine repeats were allowed to freely drink water or caffeinated water (1g/L). Treatments began at 7 weeks of age, when motor and ataxic phenotype emerges in MJD mice, and lasted up to 20 weeks. Mice were tested in a panel of locomotor behavioral paradigms, namely rotarod, beam balance and walking, pole, and water maze cued-platform version tests, and then sacrificed for cerebellar histology. Caffeine consumption attenuated the progressive loss of general and fine-tuned motor function, balance, and grip strength, in parallel with preservation of cerebellar morphology through decreasing the loss of Purkinje neurons and the thinning of the molecular layer in different folia. Caffeine also rescued the putative striatal-dependent executive and cognitive deficiencies in MJD mice. Our findings provide the first in vivo demonstration that caffeine intake alleviates behavioral disabilities in a severely impaired animal model of SCA. Ann Neurol 2017;81:407-418. © 2016 American Neurological Association.

  8. Operation of a P300-based brain-computer interface by patients with spinocerebellar ataxia

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    Yoji Okahara

    Full Text Available Objective: We investigated the efficacy of a P300-based brain-computer interface (BCI for patients with spinocerebellar ataxia (SCA, which is often accompanied by cerebellar impairment. Methods: Eight patients with SCA and eight age- and gender-matched healthy controls were instructed to input Japanese hiragana characters using the P300-based BCI with green/blue flicker. All patients depended on some assistance in their daily lives (modified Rankin scale: mean 3.5. The chief symptom was cerebellar ataxia; no cognitive deterioration was present. A region-based, two-step P300-based BCI was used. During the P300 task, eight-channel EEG data were recorded, and a linear discriminant analysis distinguished the target from other nontarget regions of the matrix. Results: The mean online accuracy in BCI operation was 82.9% for patients with SCA and 83.2% for controls; no significant difference was detected. Conclusion: The P300-based BCI was operated successfully not only by healthy controls but also by individuals with SCA. Significance: These results suggest that the P300-based BCI may be applicable for patients with SCA. Keywords: BCI, BMI, P300, Visual stimuli, Spinocerebellar ataxia

  9. Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3)

    NARCIS (Netherlands)

    Seidel, K.; Meister, M.; Dugbartey, G. J.; Zijlstra, M. P.; Vinet, J.; Brunt, E. R. P.; van Leeuwen, F. W.; Rueb, U.; Kampinga, H. H.; den Dunnen, W. F. A.

    2012-01-01

    K. Seidel, M. Meister, G. J. Dugbartey, M. P. Zijlstra, J. Vinet, E. R. P. Brunt, F. W. van Leeuwen, U. Rub, H. H. Kampinga and W. F. A. den Dunnen (2012) Neuropathology and Applied Neurobiology38, 548558 Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type

  10. Generation of spinocerebellar ataxia type 3 patient-derived induced pluripotent stem cell line SCA3.B11

    DEFF Research Database (Denmark)

    Hansen, Susanne Kofoed; Borland, Helena; Hasholt, Lis Frydenreich

    2016-01-01

    Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited neurodegenerative disease caused by an expansion of the CAG-repeat in ATXN3. In this study, induced pluripotent stem cells (iPSCs) were generated from SCA3 patient dermal fibroblasts by electroporation with episomal plasmids encoding L...

  11. Generation of spinocerebellar ataxia type 3 patient-derived induced pluripotent stem cell line SCA3.A11

    DEFF Research Database (Denmark)

    Hansen, Susanne Kofoed; Borland, Helena; Hasholt, Lis Frydenreich

    2016-01-01

    Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited neurodegenerative disease caused by a CAG-repeat expanding mutation in ATXN3. We generated induced pluripotent stem cells (iPSCs) from a SCA3 patient by electroporation of dermal fibroblasts with episomal plasmids encoding L-MYC, LIN28...

  12. Spinocerebellar ataxia type 7: Report of an Indian family

    Directory of Open Access Journals (Sweden)

    Gurusidheshwar M Wali

    2013-01-01

    Full Text Available Spinocerebellar ataxia type 7 (SCA7 is a form of autosomal dominant cerebellar ataxia which is associated with pigmentary retinal degeneration. It is known for its world-wide rarity except in the Scandinavian countries. It is very rarely reported from India and the neighbouring Asian countries . The present report describes the neurogenetic findings of a family of SCA7, from the northern part of Karnataka in South India. It documents the wide intrafamilial phenotypic variability, which could be correlated with the CAG repeat counts and phenomenon of anticipation. Genotype phenotype correlation highlighted certain disparities in comparison with the previous studies. The report highlights the need for multiethnic population studies and the role of genetic counseling and prenatal testing in SCA7 patients.

  13. Neuronal vacuolation and spinocerebellar degeneration associated with altered neurotransmission

    Directory of Open Access Journals (Sweden)

    Aggeliki Giannakopoulou

    2017-06-01

    Full Text Available Inherited neurodegenerative disorders are debilitating diseases that occur across different species, such as the domestic dog (Canis lupus familiaris, and many are caused by mutations in the same genes as corresponding human conditions. In the present study, we report an inherited neurodegenerative condition, termed ‘neuronal vacuolation and spinocerebellar degeneration’ (NVSD which affects neonatal or young dogs, mainly Rottweilers, which recently has been linked with the homozygosity for the RAB3GAP1:c.743delC allele. Mutations in human RAB3GAP1 cause Warburg micro syndrome (WARBM, a severe developmental disorder characterized predominantly by abnormalities of the nervous system including axonal peripheral neuropathy. RAB3GAP1 encodes the catalytic subunit of a GTPase activator protein and guanine exchange factor for Rab3 and Rab18 proteins, respectively. Rab proteins are involved in membrane trafficking in the endoplasmic reticulum, autophagy, axonal transport and synaptic transmission. The present study attempts to carry out a detailed histopathological examination of NVSD disease, extending from peripheral nerves to lower brain structures focusing on the neurotransmitter alterations noted in the cerebellum, the major structure affected. NVSD dogs presented with progressive cerebellar ataxia and some clinical manifestations that recapitulate the WARBM phenotype. Neuropathological examination revealed dystrophic axons, neurodegeneration and intracellular vacuolization in specific nuclei. In the cerebellum, severe vacuolation of cerebellar nuclei neurons, atrophy of Purkinje cells, and diminishing of GABAergic and glutamatergic fibres constitute the most striking lesions. The balance of evidence suggests that the neuropathological lesions are a reaction to the altered neurotransmission. The canine phenotype could serve as a model to delineate the disease-causing pathological mechanisms in RAB3GAP1 mutation.

  14. Motor Training in Degenerative Spinocerebellar Disease: Ataxia-Specific Improvements by Intensive Physiotherapy and Exergames

    Science.gov (United States)

    2014-01-01

    The cerebellum is essentially involved in movement control and plays a critical role in motor learning. It has remained controversial whether patients with degenerative cerebellar disease benefit from high-intensity coordinative training. Moreover, it remains unclear by which training methods and mechanisms these patients might improve their motor performance. Here, we review evidence from different high-intensity training studies in patients with degenerative spinocerebellar disease. These studies demonstrate that high-intensity coordinative training might lead to a significant benefit in patients with degenerative ataxia. This training might be based either on physiotherapy or on whole-body controlled videogames (“exergames”). The benefit shown in these studies is equal to regaining one or more years of natural disease progression. In addition, first case studies indicate that even subjects with advanced neurodegeneration might benefit from such training programs. For both types of training, the observed clinical improvements are paralleled by recoveries in ataxia-specific dysfunctions (e.g., multijoint coordination and dynamic stability). Importantly, for both types of training, the retention of the effects seems to depend on the frequency and continuity of training. Based on these studies, we here present preliminary recommendations for clinical practice, and articulate open questions that might guide future studies on neurorehabilitation in degenerative spinocerebellar disease. PMID:24877117

  15. Motor Training in Degenerative Spinocerebellar Disease: Ataxia-Specific Improvements by Intensive Physiotherapy and Exergames

    Directory of Open Access Journals (Sweden)

    Matthis Synofzik

    2014-01-01

    Full Text Available The cerebellum is essentially involved in movement control and plays a critical role in motor learning. It has remained controversial whether patients with degenerative cerebellar disease benefit from high-intensity coordinative training. Moreover, it remains unclear by which training methods and mechanisms these patients might improve their motor performance. Here, we review evidence from different high-intensity training studies in patients with degenerative spinocerebellar disease. These studies demonstrate that high-intensity coordinative training might lead to a significant benefit in patients with degenerative ataxia. This training might be based either on physiotherapy or on whole-body controlled videogames (“exergames”. The benefit shown in these studies is equal to regaining one or more years of natural disease progression. In addition, first case studies indicate that even subjects with advanced neurodegeneration might benefit from such training programs. For both types of training, the observed clinical improvements are paralleled by recoveries in ataxia-specific dysfunctions (e.g., multijoint coordination and dynamic stability. Importantly, for both types of training, the retention of the effects seems to depend on the frequency and continuity of training. Based on these studies, we here present preliminary recommendations for clinical practice, and articulate open questions that might guide future studies on neurorehabilitation in degenerative spinocerebellar disease.

  16. The natural history of spinocerebellar ataxia type 1, 2, 3, and 6: a 2-year follow-up study

    NARCIS (Netherlands)

    Jacobi, H.; Bauer, P.; Giunti, P.; Labrum, R.; Sweeney, M.G.; Charles, P.; Durr, A.; Marelli, C.; Globas, C.; Linnemann, C.; Schols, L.; Rakowicz, M.; Rola, R.; Zdzienicka, E.; Schmitz-Hubsch, T.; Fancellu, R.; Mariotti, C.; Tomasello, C.; Baliko, L.; Melegh, B.; Filla, A.; Rinaldi, C.; Warrenburg, B.P.C. van de; Verstappen, C.C.P.; Szymanski, S.; Berciano, J.; Infante, J.; Timmann, D.; Boesch, S.; Hering, S.; Depondt, C.; Pandolfo, M.; Kang, J.S.; Ratzka, S.; Schulz, J.; Tezenas du Montcel, S.; Klockgether, T.

    2011-01-01

    OBJECTIVE: To obtain quantitative data on the progression of the most common spinocerebellar ataxias (SCAs) and identify factors that influence their progression, we initiated the EUROSCA natural history study, a multicentric longitudinal cohort study of 526 patients with SCA1, SCA2, SCA3, or SCA6.

  17. [Spinocerebellar ataxia type 2 associated to pigmentary retinitis].

    Science.gov (United States)

    Jiménez-Caballero, Pedro Enrique; Serviá, Mónica

    2010-07-01

    Ocular disorders are useful in the characterisation of the different types of spinocerebellar ataxias (SCA); pigmentary retinitis is an alteration that is specifically associated to SCA type 7 and is characterised by night blindness, sensitivity to glare and progressive narrowing of the visual field. A 34-year-old woman with clinical symptoms of progressive ataxia and visual impairment secondary to pigmentary retinitis. The patient had a personal history with an autosomal dominant pattern of a similar disorder in her father and paternal grandmother. In the genetic study she presented a triplet expansion in the SCA type 2 gene. CONCLUSIONS; Although pigmentary retinitis belongs to the SCA type 7 phenotype, our patient presented this retinal disorder, as in other cases of SCA type 2. A genetic study for SCA type 2 must therefore be conducted in patients with a degenerative ataxic clinical picture and who present evidence of pigmentary retinitis.

  18. The human dorsal spinocerebellar tract: myelinated fiber spectrum and fiber density in controls, autosomal dominant spinocerebellar atrophy, Huntington's chorea, radiation myelopathy, and diseases with peripheral sensory nerve involvement

    Energy Technology Data Exchange (ETDEWEB)

    Ringelstein, E.B.; Schroeder, J.M.

    1982-01-01

    The human dorsal spinocerebellar tract (DSCT) was evaluated morphometrically in 14 control cases of different age and sex using semithin sections of epon-embedded cross sections from the C3, T5, and T10 segments of the spinal cord. A bimodal fiber spectrum was revealed with one peak at 2-3 microns, and a second, broader peak at about 6-8 microns. Fiber density at C3 was 11,188 fibers/mm2 and at T5, 11,156 fibers/mm2. Regression analysis relating fiber density to age disclosed a highly significant loss of myelinated fibers at T5 amounting to about 2.5% per decade. A severe reduction of fiber density and a distinct change in the fiber spectrum with predominant loss of large myelinated fibers were noted in a case of autosomal dominant spinocerebellar atrophy with late onset, and, to a lesser degree, in a case of Huntington's chorea. A subtotal loss of fibers with a persistent normal distribution of fiber sizes was observed rostral to a focus of severe radiation myelopathy, indicating Wallerian degeneration of large numbers of fibers, and a reduction of fiber diameters caudal to the lesion, suggesting retrograde fiber change. By contrast, no primary or transneural changes in the DSCT were detected in six cases of long-term alcoholism, carcinomatous sensory neuropathy, and neurofibromatosis in spite of the involvement of numerous nerve roots.

  19. Cellular protein quality control and the evolution of aggregates in spinocerebellar ataxia type 3 (SCA3).

    Science.gov (United States)

    Seidel, K; Meister, M; Dugbartey, G J; Zijlstra, M P; Vinet, J; Brunt, E R P; van Leeuwen, F W; Rüb, U; Kampinga, H H; den Dunnen, W F A

    2012-10-01

    A characteristic of polyglutamine diseases is the increased propensity of disease proteins to aggregate, which is thought to be a major contributing factor to the underlying neurodegeneration. Healthy cells contain mechanisms for handling protein damage, the protein quality control, which must be impaired or inefficient to permit proteotoxicity under pathological conditions. We used a quantitative analysis of immunohistochemistry of the pons of eight patients with the polyglutamine disorder spinocerebellar ataxia type 3. We employed the anti-polyglutamine antibody 1C2, antibodies against p62 that is involved in delivering ubiquitinated protein aggregates to autophagosomes, antibodies against the chaperones HSPA1A and DNAJB1 and the proteasomal stress marker UBB⁺¹. The 1C2 antibody stained neuronal nuclear inclusions (NNIs), diffuse nuclear staining (DNS), granular cytoplasmic staining (GCS) and combinations, with reproducible distribution. P62 always co-localized with 1C2 in NNI. DNS and GCS co-stained with a lower frequency. UBB⁺¹ was present in a subset of neurones with NNI. A subset of UBB⁺¹-containing neurones displayed increased levels of HSPA1A, while DNAJB1 was sequestered into the NNI. Based on our results, we propose a model for the aggregation-associated pathology of spinocerebellar ataxia type 3: GCS and DNS aggregation likely represents early stages of pathology, which progresses towards formation of p62-positive NNI. A fraction of NNI exhibits UBB⁺¹ staining, implying proteasomal overload at a later stage. Subsequently, the stress-inducible HSPA1A is elevated while DNAJB1 is recruited into NNIs. This indicates that the stress response is only induced late when all endogenous protein quality control systems have failed. © 2011 The Authors. Neuropathology and Applied Neurobiology © 2011 British Neuropathological Society.

  20. Spinocerebellar Ataxia Type 2: Clinicogenetic Aspects, Mechanistic Insights, and Management Approaches

    Directory of Open Access Journals (Sweden)

    Luis C. Velázquez-Pérez

    2017-09-01

    Full Text Available Spinocerebellar ataxia type 2 (SCA2 is an autosomal dominant cerebellar ataxia that occurs as a consequence of abnormal CAG expansions in the ATXN2 gene. Progressive clinical features result from the neurodegeneration of cerebellum and extra-cerebellar structures including the pons, the basal ganglia, and the cerebral cortex. Clinical, electrophysiological, and imaging approaches have been used to characterize the natural history of the disease, allowing its classification into four distinct stages, with special emphasis on the prodromal stage, which is characterized by a plethora of motor and non-motor features. Neuropathological investigations of brain tissue from SCA2 patients reveal a widespread involvement of multiple brain systems, mainly cerebellar and brainstem systems. Recent findings linking ataxin-2 intermediate expansions to other neurodegenerative diseases such as amyotrophic lateral sclerosis have provided insights into the ataxin-2-related toxicity mechanism in neurodegenerative diseases and have raised new ethical challenges to molecular predictive diagnosis of SCA2. No effective neuroprotective therapies are currently available for SCA2 patients, but some therapeutic options such as neurorehabilitation and some emerging neuroprotective drugs have shown palliative benefits.

  1. Diffusion tensor imaging for nerve fiber bundles in the brain stem and spinocerebellar degeneration

    International Nuclear Information System (INIS)

    Honma, Tsuguo

    2009-01-01

    Diffusion tensor imaging (DTI) can create an image of the anisotropic nature of diffusion and express it quantitatively. Nerve fibers have a large anisotropic diffusion, and it is possible to obtain images of the nerve fiber bundle. The purpose of this study is to observe the nerve fiber bundles in the brain stem using DTI and study its potential for diagnosing the type of spinocerebellar degeneration (SCD). Fractional anisotropy (FA) maps and 3D-tractography images were obtained for 41 subjects with no brain stem abnormalities. We created an apparent diffusion coefficient (ADC) map and an FA map using DTI for 16 subjects in the disease group (11 with hereditary SCD and 5 with non-hereditary SCD) and 25 in the control group. The diffusion value of the pons and middle cerebellar peduncle was measured using ADC, and the degree of anisotropic diffusion was measured using FA. The pyramidal tract, superior cerebellar peduncle, and inferior cerebellar peduncle were clearly demonstrated for all cases. ADC for the middle cerebellar peduncle in spinocerebellar ataxin (SCA)1 was significantly higher, similar to that for the pons in dentatorubro-pallidoluysian atrophy (DRPLA). In MSA-C, ADC for both the pons and middle cerebellar peduncle was significantly elevated and FA was significantly decreased. There were no significant changes in SCA3. We could observe the nerve fiber bundles in the brain stem using DTI. FA and ADC measurements with DTI can aid in diagnosing the type of SCD. (author)

  2. Rhythmic activity of feline dorsal and ventral spinocerebellar tract neurons during fictive motor actions

    DEFF Research Database (Denmark)

    Fedirchuk, Brent; Stecina, Katinka; Kristensen, Kasper Kyhl

    2013-01-01

    (without phasic afferent feedback). In this study, we compared the activity of DSCT and VSCT neurons during fictive rhythmic motor behaviors. We used decerebrate cat preparations in which fictive motor tasks can be evoked while the animal is paralyzed and there is no rhythmic sensory input from hindlimb......Neurons of the dorsal spinocerebellar tracts (DSCT) have been described to be rhythmically active during walking on a treadmill in decerebrate cats, but this activity ceased following deafferentation of the hindlimb. This observation supported the hypothesis that DSCT neurons primarily relay...

  3. Aqueous Extract of Paeonia lactiflora and Paeoniflorin as Aggregation Reducers Targeting Chaperones in Cell Models of Spinocerebellar Ataxia 3

    Directory of Open Access Journals (Sweden)

    Kuo-Hsuan Chang

    2013-01-01

    Full Text Available Spinocerebellar ataxia (SCA types 1, 2, 3, 6, 7, and 17 as well as Huntington’s disease are a group of neurodegenerative disorders caused by expanded CAG repeats encoding a long polyglutamine (polyQ tract in the respective proteins. Evidence has shown that the accumulation of intranuclear and cytoplasmic misfolded polyQ proteins leads to apoptosis and cell death. Thus suppression of aggregate formation is expected to inhibit a wide range of downstream pathogenic events in polyQ diseases. In this study, we established a high-throughput aggregation screening system using 293 ATXN3/Q75-GFP cells and applied this system to test the aqueous extract of Paeonia lactiflora (P. lactiflora and its constituents. We found that the aggregation can be significantly prohibited by P. lactiflora and its active compound paeoniflorin. Meanwhile, P. lactiflora and paeoniflorin upregulated HSF1 and HSP70 chaperones in the same cell models. Both of them further reduced the aggregation in neuronal differentiated SH-SY5Y ATXN3/Q75-GFP cells. Our results demonstrate how P. lactiflora and paeoniflorin are likely to work on polyQ-aggregation reduction and provide insight into the possible working mechanism of P. lactiflora in SCA3. We anticipate our paper to be a starting point for screening more potential herbs for the treatment of SCA3 and other polyQ diseases.

  4. Germ-line CAG repeat instability causes extreme CAG repeat expansion with infantile-onset spinocerebellar ataxia type 2

    DEFF Research Database (Denmark)

    Vinther-Jensen, Tua; Ek, Jakob; Duno, Morten

    2013-01-01

    The spinocerebellar ataxias (SCA) are a genetically and clinically heterogeneous group of diseases, characterized by dominant inheritance, progressive cerebellar ataxia and diverse extracerebellar symptoms. A subgroup of the ataxias is caused by unstable CAG-repeat expansions in their respective ...... of paternal germ-line repeat sequence instability of the expanded SCA2 locus.European Journal of Human Genetics advance online publication, 10 October 2012; doi:10.1038/ejhg.2012.231....

  5. Generation of an isogenic, gene-corrected control cell line of the spinocerebellar ataxia type 2 patient-derived iPSC line H266

    DEFF Research Database (Denmark)

    Marthaler, Adele Gabriele; Tubsuwan, Alisa; Schmid, Benjamin

    2016-01-01

    Spinocerebellar ataxia type 2 (SCA2) is a neurodegenerative disease primarily affecting the cerebellum. Very little is known about the molecular mechanisms underlying the disease and, to date, no cure or treatment is available. We have successfully generated bona fide induced pluripotent stem cell...

  6. Generation of an isogenic, gene-corrected control cell line of the spinocerebellar ataxia type 2 patient-derived iPSC line H271

    DEFF Research Database (Denmark)

    Marthaler, Adele Gabriele; Schmid, Benjamin; Tubsuwan, Alisa

    2016-01-01

    Spinocerebellar ataxia type 2 (SCA2) is a neurodegenerative disease primarily affecting the cerebellum. Very little is known about the molecular mechanisms underlying the disease and, to date, no cure or treatment is available. We have successfully generated bona fide induced pluripotent stem cell...

  7. Generation of an isogenic, gene-corrected control cell line of the spinocerebellar ataxia type 2 patient-derived iPSC line H196

    DEFF Research Database (Denmark)

    Marthaler, Adele Gabriele; Schmid, Benjamin; Tubsuwan, Alisa

    2016-01-01

    Spinocerebellar ataxia type 2 (SCA2) is a neurodegenerative disease primarily affecting the cerebellum. Very little is known about the molecular mechanisms underlying the disease and, to date, no cure or treatment is available. We have successfully generated bona fide induced pluripotent stem cell...

  8. Recent Advancements in Targeted Delivery of Therapeutic Molecules in Neurodegenerative Disease–-Spinocerebellar Ataxia–-Opportunities and Challenges

    OpenAIRE

    Satya Prakash; Meenakshi Malhotra

    2008-01-01

    Drug discovery and its methodologies have been very effective in terms of treating cancers and immunological disorders but have not been able to stop genetic diseases as most of the drugs target at the protein level. They merely mitigate the symptoms of the disease. Spinocerebellar ataxia is a neurological genetic disorder that is caused by the formation of an abnormal protein. There have been several reports on ataxic drug development but actual clinical treatment is yet to be achieved. Olig...

  9. Increased protein kinase C gamma activity induces Purkinje cell pathology in a mouse model of spinocerebellar ataxia 14.

    Science.gov (United States)

    Ji, Jingmin; Hassler, Melanie L; Shimobayashi, Etsuko; Paka, Nagendher; Streit, Raphael; Kapfhammer, Josef P

    2014-10-01

    Spinocerebellar ataxias (SCAs) are hereditary diseases leading to Purkinje cell degeneration and cerebellar dysfunction. Most forms of SCA are caused by expansion of CAG repeats similar to other polyglutamine disorders such as Huntington's disease. In contrast, in the autosomal dominant SCA-14 the disease is caused by mutations in the protein kinase C gamma (PKCγ) gene which is a well characterized signaling molecule in cerebellar Purkinje cells. The study of SCA-14, therefore, offers the unique opportunity to reveal the molecular and pathological mechanism eventually leading to Purkinje cell dysfunction and degeneration. We have created a mouse model of SCA-14 in which PKCγ protein with a mutation found in SCA-14 is specifically expressed in cerebellar Purkinje cells. We find that in mice expressing the mutated PKCγ protein the morphology of Purkinje cells in cerebellar slice cultures is drastically altered and mimics closely the morphology seen after pharmacological PKC activation. Similar morphological abnormalities were seen in localized areas of the cerebellum of juvenile transgenic mice in vivo. In adult transgenic mice there is evidence for some localized loss of Purkinje cells but there is no overall cerebellar atrophy. Transgenic mice show a mild cerebellar ataxia revealed by testing on the rotarod and on the walking beam. Our findings provide evidence for both an increased PKCγ activity in Purkinje cells in vivo and for pathological changes typical for cerebellar disease thus linking the increased and dysregulated activity of PKCγ tightly to the development of cerebellar disease in SCA-14 and possibly also in other forms of SCA. Copyright © 2014 Elsevier Inc. All rights reserved.

  10. Prevalence of spinocerebellar ataxia 36 in a US population.

    Science.gov (United States)

    Valera, Juliana M; Diaz, Tatyana; Petty, Lauren E; Quintáns, Beatriz; Yáñez, Zuleima; Boerwinkle, Eric; Muzny, Donna; Akhmedov, Dmitry; Berdeaux, Rebecca; Sobrido, Maria J; Gibbs, Richard; Lupski, James R; Geschwind, Daniel H; Perlman, Susan; Below, Jennifer E; Fogel, Brent L

    2017-08-01

    To assess the prevalence and clinical features of individuals affected by spinocerebellar ataxia 36 (SCA36) at a large tertiary referral center in the United States. A total of 577 patients with undiagnosed sporadic or familial cerebellar ataxia comprehensively evaluated at a tertiary referral ataxia center were molecularly evaluated for SCA36. Repeat primed PCR and fragment analysis were used to screen for the presence of a repeat expansion in the NOP56 gene. Fragment analysis of triplet repeat primed PCR products identified a GGCCTG hexanucleotide repeat expansion in intron 1 of NOP56 in 4 index cases. These 4 SCA36-positive families comprised 2 distinct ethnic groups: white (European) (2) and Asian (Japanese [1] and Vietnamese [1]). Individuals affected by SCA36 exhibited typical clinical features with gait ataxia and age at onset ranging between 35 and 50 years. Patients also suffered from ataxic or spastic limbs, altered reflexes, abnormal ocular movement, and cognitive impairment. In a US population, SCA36 was observed to be a rare disorder, accounting for 0.7% (4/577 index cases) of disease in a large undiagnosed ataxia cohort.

  11. Partial Body Weight-Supported Treadmill Training in Spinocerebellar Ataxia.

    Science.gov (United States)

    de Oliveira, Laura Alice Santos; Martins, Camilla Polonini; Horsczaruk, Carlos Henrique Ramos; da Silva, Débora Cristina Lima; Vasconcellos, Luiz Felipe; Lopes, Agnaldo José; Meira Mainenti, Míriam Raquel; Rodrigues, Erika de Carvalho

    2018-01-01

    The motor impairments related to gait and balance have a huge impact on the life of individuals with spinocerebellar ataxia (SCA). Here, the aim was to assess the possibility of retraining gait, improving cardiopulmonary capacity, and challenging balance during gait in SCA using a partial body weight support (BWS) and a treadmill. Also, the effects of this training over functionality and quality of life were investigated. Eight SCA patients were engaged in the first stage of the study that focused on gait training and cardiovascular conditioning. From those, five took part in a second stage of the study centered on dynamic balance training during gait. The first and second stages lasted 8 and 10 weeks, respectively, both comprising sessions of 50 min (2 times per week). The results showed that gait training using partial BWS significantly increased gait performance, treadmill inclination, duration of exercise, and cardiopulmonary capacity in individuals with SCA. After the second stage, balance improvements were also found. Combining gait training and challenging tasks to the postural control system in SCA individuals is viable, well tolerated by patients with SCA, and resulted in changes in capacity for walking and balance.

  12. Far-infrared radiation protects viability in a cell model of Spinocerebellar Ataxia by preventing polyQ protein accumulation and improving mitochondrial function.

    Science.gov (United States)

    Chang, Jui-Chih; Wu, Shey-Lin; Hoel, Fredrik; Cheng, Yu-Shan; Liu, Ko-Hung; Hsieh, Mingli; Hoel, August; Tronstad, Karl Johan; Yan, Kuo-Chia; Hsieh, Ching-Liang; Lin, Wei-Yong; Kuo, Shou-Jen; Su, Shih-Li; Liu, Chin-San

    2016-07-29

    Far infrared radiation (FIR) is currently investigated as a potential therapeutic strategy in various diseases though the mechanism is unknown. Presently, we tested if FIR mediates beneficial effects in a cell model of the neurodegenerative disease spinocerebellar ataxia type 3 (SCA3). SCA3 is caused by a mutation leading to an abnormal polyglutamine expansion (PolyQ) in ataxin-3 protein. The consequent aggregation of mutant ataxin-3 results in disruption of vital cell functions. In this study, neuroblastoma cells (SK-N-SH) was transduced to express either non-pathogenic ataxin-3-26Q or pathogenic ataxin-3-78Q proteins. The cells expressing ataxin-3-78Q demonstrated decreased viability, and increased sensitivity to metabolic stress in the presence rotenone, an inhibitor of mitochondrial respiration. FIR exposure was found to protect against these effects. Moreover, FIR improved mitochondrial respiratory function, which was significantly compromised in ataxin-3-78Q and ataxin-3-26Q expressing cells. This was accompanied by decreased levels of mitochondrial fragmentation in FIR treated cells, as observed by fluorescence microscopy and protein expression analysis. Finally, the expression profile LC3-II, Beclin-1 and p62 suggested that FIR prevent the autophagy inhibiting effects observed in ataxin-3-78Q expressing cells. In summary, our results suggest that FIR have rescuing effects in cells expressing mutated pathogenic ataxin-3, through recovery of mitochondrial function and autophagy.

  13. Missense mutation in CAPN1 is associated with spinocerebellar ataxia in the Parson Russell Terrier dog breed.

    Directory of Open Access Journals (Sweden)

    Oliver P Forman

    Full Text Available Spinocerebellar ataxia (SCA in the Parson Russell Terrier (PRT dog breed is a disease of progressive incoordination of gait and loss of balance. Clinical signs usually become notable between 6 and 12 months of age with affected dogs presenting with symmetric spinocerebellar ataxia particularly evident in the pelvic limbs. The degree of truncal ataxia, pelvic limb hypermetria and impaired balance is progressive, particularly during the initial months of disease. A certain degree of stabilisation as well as intermittent worsening may occur. At the later stages of the disease ambulation often becomes difficult, with owners often electing to euthanise affected dogs on welfare grounds. Using a GWAS approach and target-enriched massively-parallel sequencing, a strongly associated non-synonymous SNP in the CAPN1 gene, encoding the calcium dependent cysteine protease calpain1 (mu-calpain, was identified. The SNP is a missense mutation causing a cysteine to tyrosine substitution at residue 115 of the CAPN1 protein. Cysteine 115 is a highly conserved residue and forms a key part of a catalytic triad of amino acids that are crucial to the enzymatic activity of cysteine proteases. The CAPN1 gene shows high levels of expression in the brain and nervous system and roles for the protein in both neuronal necrosis and maintenance have been suggested. Given the functional implications and high level of conservation observed across species, the CAPN1 variant represents a provocative candidate for the cause of SCA in the PRT and a novel potential cause of ataxia in humans.

  14. Spinocerebellar ataxia type 7 (SCA7) : widespread brain damage in an adult-onset patient with progressive visual impairments in comparison with an adult-onset patient without visual impairments

    NARCIS (Netherlands)

    Rueb, U.; Brunt, E. R.; Seidel, K.; Gierga, K.; Mooy, C. M.; Kettner, M.; Van Broeckhoven, C.; Bechmann, I.; La Spada, A. R.; Schoels, L.; den Dunnen, W.; de Vos, R. A. I.; Deller, T.

    Spinocerebellar ataxia type 7 (SCA7) represents a rare and severe autosomal dominantly inherited ataxic disorder and is among the known CAG-repeat, or polyglutamine, diseases. In contrast to other currently known autosomal dominantly inherited ataxic disorders, SCA7 may manifest itself with

  15. Molecular genetics of a Chinese family with spinocerebellar ataxia

    Directory of Open Access Journals (Sweden)

    Dan-dan WU

    2015-10-01

    Full Text Available Objective To study the genotype of the members of a Chinese family with spinocerebellar ataxia (SCA. Methods The peripheral blood samples of 6 patients and 40 asymptomatic people belonged to the family were collected. Referring to the clinical manifestations of the proband and second-generation sequencing results, the CAG trinucleotide repeats of the pathogenic gene ATXN2 were amplified by polymerase chain reaction (PCR. The repeated times of the trinucleotide in normally and abnormally amplified alleles were defined by agarose gel electrophoresis and PCR products sequencing. Results Autosomal dominant heredity was the cause of the SCA in this family. Six out of 46 in the fourth-generation were SCA2 patients, 7 were the carriers of pathogenic allele. The repeated times of CAG trinucleotide were within the normal range in one of the two alleles of ATXN2, but they were in abnormal range in the another one. The repeated times of CAG trinucleotide were 40-46 in abnormal alleles of patients. Conclusion Autosomal dominant heredity SCA2 has been diagnosed in this family caused by the dynamic nutation of CAG trinucleotide repeats, and 7 pathogenic allele carriers in this family were confirmed by genetic diagnosis. DOI: 10.11855/j.issn.0577-7402.2015.08.07

  16. Spinocerebellar ataxias Ataxias espinocerebelares

    Directory of Open Access Journals (Sweden)

    Hélio A.G. Teive

    2009-12-01

    Full Text Available Spinocerebellar ataxias (SCAs constitute a heterogeneous group of neurodegenerative diseases characterized by progressive cerebellar ataxia in association with some or all of the following conditions: ophthalmoplegia, pyramidal signs, movement disorders, pigmentary retinopathy, peripheral neuropathy, cognitive dysfunction and dementia. OBJECTIVE: To carry out a clinical and genetic review of the main types of SCA. METHOD: The review was based on a search of the PUBMED and OMIM databases. RESULTS: Thirty types of SCAs are currently known, and 16 genes associated with the disease have been identified. The most common types are SCA type 3, or Machado-Joseph disease, SCA type 10 and SCA types 7, 2, 1 and 6. SCAs are genotypically and phenotypically very heterogeneous. A clinical algorithm can be used to distinguish between the different types of SCAs. CONCLUSIONS: Detailed clinical neurological examination of SCA patients can be of great help when assessing them, and the information thus gained can be used in an algorithm to screen patients before molecular tests to investigate the correct etiology of the disease are requested.As ataxias espinocerebelares (AECs compreendem um grupo heterogeneo de enfermidades neurodegenerativas, que se caracterizam pela presença de ataxia cerebelar progressiva, associada de forma variada com oftalmoplegia, sinais piramidais, distúrbios do movimento, retinopatia pigmentar, neuropatia periférica, disfunção cognitiva e demência. OBJETIVO: Realizar uma revisão clínico-genética dos principais tipos de AECs. MÉTODO: A revisão foi realizada através da pesquisa pelo sistema do PUBMED e do OMIM. RESULTADOS: Na atualidade existem cerca de 30 tipos de AECs, com a descoberta de 16 genes. Os tipos mais comuns são a AEC tipo 3, ou doença de Machado-Joseph, a AEC tipo 10, e as AECs tipo 7, 2 1, e 6. As AECs apresentam grande heterogeneidade genotípica e fenotípica. Pode-se utilizar um algoritmo clínico para a

  17. Spinocerebellar ataxia: a critical review of cognitive and socio-cognitive deficits.

    Science.gov (United States)

    Giocondo, Flora; Curcio, Giuseppe

    2018-02-01

    The primary aim of this contribution is to provide a critical discussion on cognitive and sociocognitive implications of spinocerebellar ataxias (SCAs) subtypes. The term SCA refers to a group of neurodegenerative disorders that have been increasingly investigated in the last years, sharing the characteristic of progressive ataxia resulting from degeneration of cerebellum and its connections. In past decades only involvement of cerebellum in behaviour and timing has been investigated, bringing to the belief about its central role in timing of movement and sensation, particularly for short intervals of time. Only very recently the cerebellum has been considered as a potentially important centre for cognitive processing and related spheres of social cognition, so that several studies with SCA patients have been carried out on these topics: as a consequence a section of this review will be dedicated to this important aspect. After a brief discussion on most commonly used methods to assess cognitive and socio-cognitive abilities in SCAs, cognitive and socio-cognitive profiles of principal SCA subtypes have been thoroughly reviewed and critically discussed. Due to the very poor literature in this field the most common SCA variants have been fully included (i.e. SCA1, SCA2, SCA3, SCA6 and SCA7). A comparative summary of the main characteristics of cognitive and social cognition deficit in SCA subtypes has been proposed together with a research agenda for future investigation in this field principally aimed at using measures of cognition and/or social cognition as potential predictors of the extent and progression of disease.

  18. Depression as the Primary Cause of Insomnia and Excessive Daytime Sleepiness in a Family with Multiple Cases of Spinocerebellar Ataxia.

    Science.gov (United States)

    Hsu, Chun-Hsien; Chen, Yen-Lin; Pei, Dee; Yu, Shu-Man; Liu, I-Chao

    2016-07-15

    Spinocerebellar ataxia (SCA) is a hereditary disease characterized by central nervous system-related motor dysfunctions. Sleep disorders and frequent non-motor manifestations are commonly comorbid with SCA. To elucidate this relationship, we present three cases in a family that included multiple SCA type 2 patients with various sleep disorders. Complete physical examination, and genetic and imaging studies were performed. Anti-parkinsonism medications were prescribed after neurological examination. Clonazepam and/or quetiapine were administered for sleep disorders but failed to resolve insomnia and excessive daytime sleepiness (EDS). Based on DSM-5 criteria, all cases were diagnosed with depression. After treatment with serotonin-norepinephrine reuptake inhibitors and noradrenergic and specific serotonergic antidepressants, symptoms of insomnia and EDS, which are strongly associated with depression in SCA type 2 patients, improved significantly. It is crucial to recognize insomnia and EDS in neurodegenerative diseases, not only for earlier diagnosis, but also to improve quality of life. © 2016 American Academy of Sleep Medicine.

  19. Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.

    Science.gov (United States)

    Mascalchi, Mario; Diciotti, Stefano; Giannelli, Marco; Ginestroni, Andrea; Soricelli, Andrea; Nicolai, Emanuele; Aiello, Marco; Tessa, Carlo; Galli, Lucia; Dotti, Maria Teresa; Piacentini, Silvia; Salvatore, Elena; Toschi, Nicola

    2014-01-01

    Spinocerebellar ataxia type 2 (SCA2) is the second most frequent autosomal dominant inherited ataxia worldwide. We investigated the capability of magnetic resonance imaging (MRI) to track in vivo progression of brain atrophy in SCA2 by examining twice 10 SCA2 patients (mean interval 3.6 years) and 16 age- and gender-matched healthy controls (mean interval 3.3 years) on the same 1.5 T MRI scanner. We used T1-weighted images and tensor-based morphometry (TBM) to investigate volume changes and the Inherited Ataxia Clinical Rating Scale to assess the clinical deficit. With respect to controls, SCA2 patients showed significant higher atrophy rates in the midbrain, including substantia nigra, basis pontis, middle cerebellar peduncles and posterior medulla corresponding to the gracilis and cuneatus tracts and nuclei, cerebellar white matter (WM) and cortical gray matter (GM) in the inferior portions of the cerebellar hemisphers. No differences in WM or GM volume loss were observed in the supratentorial compartment. TBM findings did not correlate with modifications of the neurological deficit. In conclusion, MRI volumetry using TBM is capable of demonstrating the progression of pontocerebellar atrophy in SCA2, supporting a possible role of MRI as biomarker in future trials.

  20. Progression of brain atrophy in spinocerebellar ataxia type 2: a longitudinal tensor-based morphometry study.

    Directory of Open Access Journals (Sweden)

    Mario Mascalchi

    Full Text Available Spinocerebellar ataxia type 2 (SCA2 is the second most frequent autosomal dominant inherited ataxia worldwide. We investigated the capability of magnetic resonance imaging (MRI to track in vivo progression of brain atrophy in SCA2 by examining twice 10 SCA2 patients (mean interval 3.6 years and 16 age- and gender-matched healthy controls (mean interval 3.3 years on the same 1.5 T MRI scanner. We used T1-weighted images and tensor-based morphometry (TBM to investigate volume changes and the Inherited Ataxia Clinical Rating Scale to assess the clinical deficit. With respect to controls, SCA2 patients showed significant higher atrophy rates in the midbrain, including substantia nigra, basis pontis, middle cerebellar peduncles and posterior medulla corresponding to the gracilis and cuneatus tracts and nuclei, cerebellar white matter (WM and cortical gray matter (GM in the inferior portions of the cerebellar hemisphers. No differences in WM or GM volume loss were observed in the supratentorial compartment. TBM findings did not correlate with modifications of the neurological deficit. In conclusion, MRI volumetry using TBM is capable of demonstrating the progression of pontocerebellar atrophy in SCA2, supporting a possible role of MRI as biomarker in future trials.

  1. [123I]-IMP SPECT findings in spinocerebellar ataxia type 6

    International Nuclear Information System (INIS)

    Ito, Yasuhiko; Nakabayashi, Haruo; Iguchi, Yasuyuki; Suzuki, Masahiko; Kobayashi, Masayuki; Nakajima, Takashi

    2000-01-01

    To study the dynamics of metabolic function in the cerebellar hemispheres, vermis and brain stem of patients with spinocerebellar ataxia type 6 (SCA6), we used single photon emission computed tomography (SPECT) with N-isopropyl-p-[ 123 I] iodoamphetamine (IMP) to measure regional cerebral blood flow (rCBF) in six Japanese patients with SCA6 and nine normal control subjects. All patients with SCA6 were found to have expanded CAG repeats (from 22 to 24 repeats). The SPECT data were also analyzed semiquantitatively. The rCBF in the cerebellar hemisphere, vermis and brain stem was not significantly lower in patients with SCA6 than in normal controls. However, the ratio of the cerebellar hemisphere to occipital lobe (C/O ratio) was significantly lower in patients. The ratio of the vermis and brain stem to occipital lobe (V/O, P/O ratio) were not significantly lower in patients. The C/O, V/O and P/O ratio were especially sensitive indexes for regional cerebral function in patients with SCA6. Results of this study suggest that the functional decrease in SCA6 may begin in the cerebellar hemispheres. IMP SPECT was useful for evaluating rCBF in patients with SCA6. (author)

  2. Progression of Dysphagia in Spinocerebellar Ataxia Type 6.

    Science.gov (United States)

    Isono, Chiharu; Hirano, Makito; Sakamoto, Hikaru; Ueno, Shuichi; Kusunoki, Susumu; Nakamura, Yusaku

    2017-06-01

    Spinocerebellar ataxia type 6 (SCA6), an autosomal dominant triplet repeat disease, predominantly affects the cerebellum with a late onset and generally good prognosis. Dysphagia is commonly associated with the outcomes of neurodegenerative diseases such as SCA6. Although the characteristics of dysphagia have been rarely reported in SCA6, our previous study indicated that dysphagia is generally milder in SCA6 than in SCA3, another inherited ataxia with multisystem involvement. However, abnormalities in the pharyngeal phase in SCA6 were indistinguishable from those in SCA3, with no explainable reason. To determine the reason, we repeatedly performed videofluoroscopic examinations (VF) in 14 patients with SCA6. The results showed that the gross progression of dysphagia was apparently slow, but four patients had progressive dysphagia at an early disease stage; dysphagia began within 10 years from the onset of ataxia and rapidly progressed. A common clinical feature of the four patients was a significantly older age at the onset of ataxia (74.0 vs. 60.3 years), associated with significantly shorter triplet repeats. This finding surprisingly indicated that patients who had shorter repeats and thereby later onset and potentially better prognoses were at risk for dysphagia-associated problems. Ischemic changes, homozygous mutation, and diabetes mellitus as well as aging might have contributed to the observed progressive dysphagia. We found that conventionally monitored somatosensory evoked potentials at least partly reflected progressive dysphagia. Despite the small study group, our findings suggest that clinicians should carefully monitor dysphagia in patients with SCA6 who are older at disease onset (>60 years).

  3. A Novel TTBK2 De Novo Mutation in a Danish Family with Early-Onset Spinocerebellar Ataxia

    DEFF Research Database (Denmark)

    Lindquist, Suzanne Granhøj; Møller, Lisbeth Birk; Dali, Christine I.

    2017-01-01

    Spinocerebellar ataxia type 11 (SCA11) is rare and has previously been described in four families worldwide. We report a Danish family with onset of symptoms in early childhood and affected family members in two generations. The proband, a Danish female born in 1968, and family members were...... examined. Exome sequencing was performed and a “movement disorders” gene panel consisting of approximately 200 genes was used for filtering, while Sanger sequencing was used for subsequent testing for the mutation in the family. Onset of symptoms in affected family members was in early childhood. A novel...... delineates the phenotypic spectrum of the rare SCA11 disease. In contrast to previously reported cases, onset of symptoms was in early childhood and the mutation was de novo in the proband....

  4. From mild ataxia to huntington disease phenocopy: the multiple faces of spinocerebellar ataxia 17.

    Science.gov (United States)

    Koutsis, Georgios; Panas, Marios; Paraskevas, George P; Bougea, Anastasia M; Kladi, Athina; Karadima, Georgia; Kapaki, Elisabeth

    2014-01-01

    Introduction. Spinocerebellar ataxia 17 (SCA 17) is a rare autosomal dominant cerebellar ataxia (ADCA) caused by a CAG/CAA expansion in the TBP gene, reported from a limited number of countries. It is a very heterogeneous ADCA characterized by ataxia, cognitive decline, psychiatric symptoms, and involuntary movements, with some patients presenting with Huntington disease (HD) phenocopies. The SCA 17 expansion is stable during parent-child transmission and intrafamilial phenotypic homogeneity has been reported. However, significant phenotypic variability within families has also been observed. Report of the Family. We presently report a Greek family with a pathological expansion of 54 repeats at the SCA 17 locus that displayed remarkable phenotypic variability. Among 3 affected members, one presented with HD phenocopy; one with progressive ataxia, dementia, chorea, dystonia, and seizures, and one with mild slowly progressive ataxia with minor cognitive and affective symptoms. Conclusions. This is the first family with SCA 17 identified in Greece and highlights the multiple faces of this rare disorder, even within the same family.

  5. Spinocerebellar ataxia type 6 in eastern India: Some new observations.

    Science.gov (United States)

    Bhattacharyya, Kalyan B; Pulai, Debabrata; Guin, Deb Shankar; Ganguly, Goutam; Joardar, Anindita; Roy, Sarnava; Rai, Saurabh; Biswas, Atanu; Pandit, Alok; Roy, Arijit; Senapati, Asit Kumar

    2016-01-01

    Spinocerebellar ataxias (SCAs) are hereditary, autosomal dominant progressive neurodegenerative disorders showing clinical and genetic heterogeneity. They are usually manifested clinically in the third to fifth decade of life although there is a wide variability in the age of onset. More than 36 different types of SCAs have been reported so far and about half of them are caused by pathological expansion of the trinucleotide, Cytosine Alanine Guanine (CAG) repeat. The global prevalence of SCA is 0.3-2 per 100,000 population, SCA3 being the commonest variety worldwide, accounting for 20-50 per cent of all cases, though SCA 2 is generally considered as the commonest one in India. However, SCA6 has not been addressed adequately from India though it is common in the eastern Asian countries like, Japan, Korea and Thailand. The present study was undertaken to identify the prevalence of SCA6 in the city of Kolkata and the eastern part of India. 83 consecutive patients were recruited for the study of possible SCAs and their clinical features and genotype were investigated. 6 of the 83 subjects turned out positive for SCA6, constituting therefore, 13.33% of the patient pool. SCA6 is prevalent in the eastern part of India, though not as frequent as the other common varieties. Further community based studies are required in order to understand the magnitude of SCA6 in the eastern part, as well as in other regions of India.

  6. Two in one: report of a patient with spinocerebellar ataxia types 2 and 10.

    Science.gov (United States)

    Kapur, Sachin S; Goldman, Jennifer G

    2012-09-01

    To report a rare case of the coexistence of 2 spinocerebellar ataxia (SCA) mutations in a single patient. Case report. University hospital, Movement Disorders Center. A 54-year-old man of Mexican, American Indian, and French descent with an 11-year history of gait and limb ataxia. Findings of clinical examination, magnetic resonance imaging, and video electroencephalographic monitoring. Neurologic history revealed a gradually progressive gait and limb ataxia along with muscle cramps and sensory symptoms in his distal extremities; examination revealed executive dysfunction, dysarthria, ataxia, and sensory neuronopathy. Episodes of loss of awareness were reported, but electroencephalograms were negative. Brain imaging demonstrated severe cerebellar and brainstem atrophy. Genetic evaluation of the case revealed mutations in both the SCA2 and SCA10 genes. Our patient has a unique combination of genetic mutations for 2 different SCAs, types 2 and 10, which to our knowledge, has not been previously reported. His clinical phenotype is largely consistent with SCA2, but his possible seizures and Mexican heritage suggest influences of SCA10.

  7. From Mild Ataxia to Huntington Disease Phenocopy: The Multiple Faces of Spinocerebellar Ataxia 17

    Directory of Open Access Journals (Sweden)

    Georgios Koutsis

    2014-01-01

    Full Text Available Introduction. Spinocerebellar ataxia 17 (SCA 17 is a rare autosomal dominant cerebellar ataxia (ADCA caused by a CAG/CAA expansion in the TBP gene, reported from a limited number of countries. It is a very heterogeneous ADCA characterized by ataxia, cognitive decline, psychiatric symptoms, and involuntary movements, with some patients presenting with Huntington disease (HD phenocopies. The SCA 17 expansion is stable during parent-child transmission and intrafamilial phenotypic homogeneity has been reported. However, significant phenotypic variability within families has also been observed. Report of the Family. We presently report a Greek family with a pathological expansion of 54 repeats at the SCA 17 locus that displayed remarkable phenotypic variability. Among 3 affected members, one presented with HD phenocopy; one with progressive ataxia, dementia, chorea, dystonia, and seizures, and one with mild slowly progressive ataxia with minor cognitive and affective symptoms. Conclusions. This is the first family with SCA 17 identified in Greece and highlights the multiple faces of this rare disorder, even within the same family.

  8. Cerebello-cerebral functional relationship in spinocerebellar degeneration using positron emission tomography

    International Nuclear Information System (INIS)

    Koshi, Yasuhiko; Kitamura, Shin; Sakayori, Osamu; Komaba, Yuichi; Terashi, Akiro

    1995-01-01

    In order to investigate the laterality of cerebellar ataxia and its influence for the cerebral cortex in spinocerebellar degeneration (SCD), regional cerebral blood flow (rCBF) was measured using positron emission tomography (PET) in 10 patients with sporadic olivopontocerebellar atrophy (sOPCA), 7 patients with hereditary SCD (hSCD), and 10 age matched control subjects. The laterality of cerebellar ataxia was evaluated by the total score of the difference between left and right limbs of three limb-coordination tests. The lateralities of rCBF were calculated by asymmetry indices (AIs) of each region of interest in the cerebellum, thalamus, caudate, putamen, cerebral cortices. The laterality of cerebellar ataxia was significantly correlated with AI in the cerebellum in patients with sOPCA. Furthermore, significant negative correlations were observed between AI in the cerebellum and each AI in the thalamus, frontal cortex in patients with sOPCA. However, no correlations were observed between AI in the cerebellum and the other AIs in controls and patients with h SCD. Duration of illness in patients with sOPCA with laterality is shorter than that in patients without laterality. These results suggest that the existence of crossed cerebello-cerebral diaschisis (CCCD) resulting from transneuronal deactivation through cerebello-thalamo-cerebral pathway in patients with the early stage of sOPCA with laterality. (author)

  9. Cerebello-cerebral functional relationship in spinocerebellar degeneration using positron emission tomography

    Energy Technology Data Exchange (ETDEWEB)

    Koshi, Yasuhiko; Kitamura, Shin; Sakayori, Osamu; Komaba, Yuichi; Terashi, Akiro [Nippon Medical School, Tokyo (Japan)

    1995-07-01

    In order to investigate the laterality of cerebellar ataxia and its influence for the cerebral cortex in spinocerebellar degeneration (SCD), regional cerebral blood flow (rCBF) was measured using positron emission tomography (PET) in 10 patients with sporadic olivopontocerebellar atrophy (sOPCA), 7 patients with hereditary SCD (hSCD), and 10 age matched control subjects. The laterality of cerebellar ataxia was evaluated by the total score of the difference between left and right limbs of three limb-coordination tests. The lateralities of rCBF were calculated by asymmetry indices (AIs) of each region of interest in the cerebellum, thalamus, caudate, putamen, cerebral cortices. The laterality of cerebellar ataxia was significantly correlated with AI in the cerebellum in patients with sOPCA. Furthermore, significant negative correlations were observed between AI in the cerebellum and each AI in the thalamus, frontal cortex in patients with sOPCA. However, no correlations were observed between AI in the cerebellum and the other AIs in controls and patients with h SCD. Duration of illness in patients with sOPCA with laterality is shorter than that in patients without laterality. These results suggest that the existence of crossed cerebello-cerebral diaschisis (CCCD) resulting from transneuronal deactivation through cerebello-thalamo-cerebral pathway in patients with the early stage of sOPCA with laterality. (author).

  10. Mutation in the kv3.3 voltage-gated potassium channel causing spinocerebellar ataxia 13 disrupts sound-localization mechanisms.

    Directory of Open Access Journals (Sweden)

    John C Middlebrooks

    Full Text Available Normal sound localization requires precise comparisons of sound timing and pressure levels between the two ears. The primary localization cues are interaural time differences, ITD, and interaural level differences, ILD. Voltage-gated potassium channels, including Kv3.3, are highly expressed in the auditory brainstem and are thought to underlie the exquisite temporal precision and rapid spike rates that characterize brainstem binaural pathways. An autosomal dominant mutation in the gene encoding Kv3.3 has been demonstrated in a large Filipino kindred manifesting as spinocerebellar ataxia type 13 (SCA13. This kindred provides a rare opportunity to test in vivo the importance of a specific channel subunit for human hearing. Here, we demonstrate psychophysically that individuals with the mutant allele exhibit profound deficits in both ITD and ILD sensitivity, despite showing no obvious impairment in pure-tone sensitivity with either ear. Surprisingly, several individuals exhibited the auditory deficits even though they were pre-symptomatic for SCA13. We would expect that impairments of binaural processing as great as those observed in this family would result in prominent deficits in localization of sound sources and in loss of the "spatial release from masking" that aids in understanding speech in the presence of competing sounds.

  11. Spinocerebellar ataxia type 6 in eastern India: Some new observations

    Directory of Open Access Journals (Sweden)

    Kalyan B Bhattacharyya

    2016-01-01

    Full Text Available Introduction: Spinocerebellar ataxias (SCAs are hereditary, autosomal dominant progressive neurodegenerative disorders showing clinical and genetic heterogeneity. They are usually manifested clinically in the third to fifth decade of life although there is a wide variability in the age of onset. More than 36 different types of SCAs have been reported so far and about half of them are caused by pathological expansion of the trinucleotide, Cytosine Alanine Guanine (CAG repeat. The global prevalence of SCA is 0.3-2 per 100,000 population, SCA3 being the commonest variety worldwide, accounting for 20-50 per cent of all cases, though SCA 2 is generally considered as the commonest one in India. However, SCA6 has not been addressed adequately from India though it is common in the eastern Asian countries like, Japan, Korea and Thailand. Objective: The present study was undertaken to identify the prevalence of SCA6 in the city of Kolkata and the eastern part of India. Materials and Methods: 83 consecutive patients were recruited for the study of possible SCAs and their clinical features and genotype were investigated. Results: 6 of the 83 subjects turned out positive for SCA6, constituting therefore, 13.33% of the patient pool. Discussion: SCA6 is prevalent in the eastern part of India, though not as frequent as the other common varieties. Conclusions: Further community based studies are required in order to understand the magnitude of SCA6 in the eastern part, as well as in other regions of India.

  12. Modifications of resting state networks in spinocerebellar ataxia type 2.

    Science.gov (United States)

    Cocozza, Sirio; Saccà, Francesco; Cervo, Amedeo; Marsili, Angela; Russo, Cinzia Valeria; Giorgio, Sara Maria Delle Acque; De Michele, Giuseppe; Filla, Alessandro; Brunetti, Arturo; Quarantelli, Mario

    2015-09-01

    We aimed to investigate the integrity of the Resting State Networks in spinocerebellar ataxia type 2 (SCA2) and the correlations between the modification of these networks and clinical variables. Resting-state functional magnetic resonance imaging (RS-fMRI) data from 19 SCA2 patients and 29 healthy controls were analyzed using an independent component analysis and dual regression, controlling at voxel level for the effect of atrophy by co-varying for gray matter volume. Correlations between the resting state networks alterations and disease duration, age at onset, number of triplets, and clinical score were assessed by Spearman's coefficient, for each cluster which was significantly different in SCA2 patients compared with healthy controls. In SCA2 patients, disruption of the cerebellar components of all major resting state networks was present, with supratentorial involvement only for the default mode network. When controlling at voxel level for gray matter volume, the reduction in functional connectivity in supratentorial regions of the default mode network, and in cerebellar regions within the default mode, executive and right fronto-parietal networks, was still significant. No correlations with clinical variables were found for any of the investigated resting state networks. The SCA2 patients show significant alterations of the resting state networks, only partly explained by the atrophy. The default mode network is the only resting state network that shows also supratentorial changes, which appear unrelated to the cortical gray matter volume. Further studies are needed to assess the clinical significance of these changes. © 2015 International Parkinson and Movement Disorder Society.

  13. Alteration of CBF and CMRO2 and TRH effects on CBF in spinocerebellar degeneration

    International Nuclear Information System (INIS)

    Harada, Kiyoshi; Fukuyama, Hidenao; Miyoshi, Toshihiko; Namura, Yasuhiro; Kameyama, Masakuni

    1988-01-01

    The purpose of this study is to elucidate the effects of thyrotropin-releasing hormone (TRH) on cerebral blood flow (CBF) in patients with spinocerebellar degeneration (SCD) and to evaluate the cerebral circulation and metabolism in patients with SCD. We performed a positron emission tomography study on each of six SCD patients (mean age 47.7 ± 3.6 : 5 cases; OPCA of Dejerene-Thomas type, 1 case; OPCA of Menzel type) and twelve normal volunteers. In SCD patients there were marked reductions in CBF (p < 0.01) and CMRO2 (p < 0.01) in the cerebellum compared with normal volunteers, while in the cerebral cortices and the thalamus, SCD patients showed normal values. There were no significant changes in regional and global CBF after 2 mg TRH intravenous injection in the SCD patients. But comparing CBF before TRH administration with corrected CBF (CBF after TRH · mean global CBF before TRH/mean global CBF after TRH), it is only the CBF of the cerebellum that increased after TRH administration (paired t test, p < 0.02). This elevation of CBF in the cerebellum would be related to some clinical effects of TRH in SCD patients. (author)

  14. Spinocerebellar ataxias in Venezuela: genetic epidemiology and their most likely ethnic descent.

    Science.gov (United States)

    Paradisi, Irene; Ikonomu, Vassiliki; Arias, Sergio

    2016-03-01

    Dominantly inherited ataxias (spinocerebellar ataxias, SCAs) are a genetically heterogeneous group of neurologic diseases characterized by progressive cerebellar and spinal tract degeneration with ataxia and other signs, common to all known subtypes. Several types are relatively frequent worldwide, but in several countries, one specific SCA may show a higher prevalence owing to founder phenomena. In Venezuela, genetic epidemiological features of SCAs have been assessed during the last 30 years; mutations in ATXN1 (SCA1), ATXN2 (SCA2), ATXN3 (SCA3), CACNA1A (SCA6), ATXN7 (SCA7), ATXN8 (SCA8), ATXN10 (SCA10), TBP (SCA17) and ATN1 (dentatorubral pallidoluysian atrophy, DRPLA) loci were searched among 115 independent families. SCA7 was the most frequent subtype (26.6%), followed by SCA3 (25.0%), SCA2 (21.9%), SCA1 (17.2%), SCA10 (4.7%) and DRPLA (3.1%); in 43% of the families, the subtype remained unidentified. SCA7 mutations displayed strong geographic aggregation in two independent founder foci, and SCA1 showed a very remote founder effect for a subset of families. SCA10 families were scattered across the country, but all had an identical in-phase haplotype carried also by Mexican, Brazilian and Sioux patients, supporting a very old common Amerindian origin. Prevalence for dominant SCAs in Venezuela was estimated as 1:25 000 nuclear families, provenances of which are either Caucasoid, African or Amerindian.

  15. Evaluation of supra- and infratentorial brain atrophy by computerized tomography in spinocerebellar degeneration

    International Nuclear Information System (INIS)

    Yamamoto, Hiroko; Asano, Yasuhiko; Watanabe, Takatoshi; Hirao, Yoshitaka; Mizuno, Yasushi; Sobue, Itsuro

    1986-01-01

    Measurement of various parameters of supra- and infratentorial brain atrophy in computerized tomographs of 142 cases of spinocerebellar degeneration (SCD) and 100 age and sex matched controls was carried out in order to investigate whether these parameters would correspond to the subtypes of this disease and differing grades of various clinical manifestations. One supra- and all infratentorial parameters of SCD showed statistically significant atrophy with a risk of P < 0.005. Among the subtypes, OPCA had a more severely atrophied pons than LCCA (P < 0.005), Menzel (P < 0.05) and SSP (P < 0.01). There was a correlation between the distribution of symptoms like gait, speech, ataxia of extremities and ocular movement disorders, and distribution and degree of infratentorial atrophy with statistical significance (P < 0.05 ∼ P < 0.005). The degree of atrophy of the pons and the width of the IV ventricle were directly proportional to the duration of the illness in cases of less than 10 years, but not to those of over 10 years. Follow-up CT scan was done for 24 patients, 12 within 3 years, 12 after the lapse of 3 years. The latter group showed statistically significant atrophy between the 1st and 2nd scans in several parameters, but there was no significance between those of the former group. (author)

  16. Evaluation of supra- and infratentorial brain atrophy by computerized tomography in spinocerebellar degeneration

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, Hiroko; Asano, Yasuhiko; Watanabe, Takatoshi; Hirao, Yoshitaka; Mizuno, Yasushi; Sobue, Itsuro

    1986-08-01

    Measurement of various parameters of supra- and infratentorial brain atrophy in computerized tomographs of 142 cases of spinocerebellar degeneration (SCD) and 100 age and sex matched controls was carried out in order to investigate whether these parameters would correspond to the subtypes of this disease and differing grades of various clinical manifestations. One supra- and all infratentorial parameters of SCD showed statistically significant atrophy with a risk of P < 0.005. Among the subtypes, OPCA had a more severely atrophied pons than LCCA (P < 0.005), Menzel (P < 0.05) and SSP (P < 0.01). There was a correlation between the distribution of symptoms like gait, speech, ataxia of extremities and ocular movement disorders, and distribution and degree of infratentorial atrophy with statistical significance (P < 0.05 -- P < 0.005). The degree of atrophy of the pons and the width of the IV ventricle were directly proportional to the duration of the illness in cases of less than 10 years, but not to those of over 10 years. Follow-up CT scan was done for 24 patients, 12 within 3 years, 12 after the lapse of 3 years. The latter group showed statistically significant atrophy between the 1st and 2nd scans in several parameters, but there was no significance between those of the former group.

  17. Peripheral Neuropathy in Spinocerebellar Ataxia Type 1, 2, 3, and 6.

    Science.gov (United States)

    Linnemann, Christoph; Tezenas du Montcel, Sophie; Rakowicz, Maryla; Schmitz-Hübsch, Tanja; Szymanski, Sandra; Berciano, Jose; van de Warrenburg, Bart P; Pedersen, Karine; Depondt, Chantal; Rola, Rafal; Klockgether, Thomas; García, Antonio; Mutlu, Gurkan; Schöls, Ludger

    2016-04-01

    Spinocerebellar ataxias (SCAs) are characterized by autosomal dominantly inherited progressive ataxia but are clinically heterogeneous due to variable involvement of non-cerebellar parts of the nervous system. Non-cerebellar symptoms contribute significantly to the burden of SCAs, may guide the clinician to the underlying genetic subtype, and might be useful markers to monitor disease. Peripheral neuropathy is frequently observed in SCA, but subtype-specific features and subclinical manifestations have rarely been evaluated. We performed a multicenter nerve conduction study with 162 patients with genetically confirmed SCA1, SCA2, SCA3, and SCA6. The study proved peripheral nerves to be involved in the neurodegenerative process in 82 % of SCA1, 63 % of SCA2, 55 % of SCA3, and 22 % of SCA6 patients. Most patients of all subtypes revealed affection of both sensory and motor fibers. Neuropathy was most frequently of mixed type with axonal and demyelinating characteristics in all SCA subtypes. However, nerve conduction velocities of SCA1 patients were slower compared to other genotypes. SCA6 patients revealed less axonal damage than patients with other subtypes. No influence of CAG repeat length or biometric determinants on peripheral neuropathy could be identified in SCA1, SCA3, and SCA6. In SCA2, earlier onset and more severe ataxia were associated with peripheral neuropathy. We proved peripheral neuropathy to be a frequent site of the neurodegenerative process in all common SCA subtypes. Since damage to peripheral nerves is readily assessable by electrophysiological means, nerve conduction studies should be performed in a longitudinal approach to assess these parameters as potential progression markers.

  18. Recent Advancements in Targeted Delivery of Therapeutic Molecules in Neurodegenerative Disease - Spinocerebellar Ataxia - Opportunities and Challenges

    Directory of Open Access Journals (Sweden)

    Satya Prakash

    2008-01-01

    Full Text Available Drug discovery and its methodologies have been very effective in terms of treating cancers and immunological disorders but have not been able to stop genetic diseases as most of the drugs target at the protein level. They merely mitigate the symptoms of the disease. Spinocerebellar ataxia is a neurological genetic disorder that is caused by the formation of an abnormal protein. There have been several reports on ataxic drug development but actual clinical treatment is yet to be achieved. Oligonucleotide therapy called sequence specific siRNA mediated gene silencing has evolved with promising results. This approach emphasizes on suppressing the expression of the diseased gene at mRNA level. However, there is a limitation in delivery of siRNA to the target site. Several methods have been developed over the last decade to enhance the target specific delivery of DNA, siRNA, protein and small drug molecules for therapeutic purpose with less or no side effects. This review discusses the latest upcoming technologies in the field that focus on a number of nonviral nanocarriers for targeted delivery. In this review, we explore the promise and potential of novel therapeutics with interest on ataxia therapy.

  19. Recent Advancements in Targeted Delivery of Therapeutic Molecules in Neurodegenerative Disease–-Spinocerebellar Ataxia–-Opportunities and Challenges

    Directory of Open Access Journals (Sweden)

    Satya Prakash

    2008-01-01

    Full Text Available Drug discovery and its methodologies have been very effective in terms of treating cancers and immunological disorders but have not been able to stop genetic diseases as most of the drugs target at the protein level. They merely mitigate the symptoms of the disease. Spinocerebellar ataxia is a neurological genetic disorder that is caused by the formation of an abnormal protein. There have been several reports on ataxic drug development but actual clinical treatment is yet to be achieved. Oligonucleotide therapy called sequence specific siRNA mediated gene silencing has evolved with promising results. This approach emphasizes on suppressing the expression of the diseased gene at mRNA level. However, there is a limitation in delivery of siRNA to the target site. Several methods have been developed over the last decade to enhance the target specific delivery of DNA, siRNA, protein and small drug molecules for therapeutic purpose with less or no side effects. This review discusses the latest upcoming technologies in the field that focus on a number of nonviral nanocarriers for targeted delivery. In this review, we explore the promise and potential of novel therapeutics with interest on ataxia therapy.

  20. CT scanning of the brain and lumber CSF monoamine metabolites in spinocerebellar degenerative disorders

    International Nuclear Information System (INIS)

    Sasaki, Hidenao; Kanazawa, Ichiro; Nakanishi, Takao; Kuramoto, Kenmei

    1984-01-01

    Eight patients with parenchymatous cerebellar degeneration (PCD) group (3 with late cortical cerebellar atrophy and 5 with Holmes' hereditary ataxia), 14 with olivo-ponto-cerebellar atrophy (OPCA) group (4 with Shy-Drager syndrome, 6 with OPCA without family history and 4 with Menzel type SCS), 15 with Parkinson's disease and 44 control with other neurological diseases were studied. In all the spinocerebellar degenerative disorder s (SCD) cases, CVI values corresponding to the cerebellar atrophy were definitely reduced. On the other hand, PVI values corresponding to the pontine atrophy were only significantly decreased in OPCA group. However, since there were several cases showing only questionable pontine atrpphy, it seems difficult to clearly differentiate individual OPCA cases from other SCD cases on CT films alone. Concerning monoamine metabolites in CSF, it was noted that a significant reduction of HVA and total MHPG was found in the OPCA group. Among them, the patients with overt autonomic failure showed the lowest HVA level and the cases of Menzel type of SCD showed a slight reduction of HVA but an unexpected elevation of free MHPG values. The cases of Parkinson's disease showed a definite reduction of HVA. On the other hand, the cases of PCD group showed no significant difference against controls. 5-HIAA levels were not significantly different among the SCD subgroups. (J.P.N.)

  1. Repeat interruptions in spinocerebellar ataxia type 10 expansions are strongly associated with epileptic seizures

    Science.gov (United States)

    McFarland, Karen N.; Liu, Jilin; Landrian, Ivette; Zeng, Desmond; Raskin, Salmo; Moscovich, Mariana; Gatto, Emilia M.; Ochoa, Adriana; Teive, Hélio A. G.; Rasmussen, Astrid; Ashizawa, Tetsuo

    2014-01-01

    Spinocerebellar ataxia type 10 (SCA10), an autosomal dominant neurodegenerative disorder, is the result of a non-coding, pentanucleotide repeat expansion within intron 9 of the Ataxin 10 gene. SCA10 patients present with pure cerebellar ataxia; yet, some families also have a high incidence of epilepsy. SCA10 expansions containing penta- and heptanucleotide interruption motifs, termed “ATCCT interruptions,” experience large contractions during germline transmission, particularly in paternal lineages. At the same time, these alleles confer an earlier age at onset which contradicts traditional rules of genetic anticipation in repeat expansions. Previously, ATCCT interruptions have been associated with a higher prevalence of epileptic seizures in one Mexican-American SCA10 family. In a large cohort of SCA10 families, we analyzed whether ATCCT interruptions confers a greater risk for developing seizures in these families. Notably, we find that the presence of repeat interruptions within the SCA10 expansion confers a 6.3-fold increase in the risk of an SCA10 patient developing epilepsy (6.2-fold when considering patients of Mexican ancestry only) and a 13.7-fold increase in having a positive family history of epilepsy (10.5-fold when considering patients of Mexican ancestry only). We conclude that the presence of repeat interruptions in SCA10 repeat expansion indicates a significant risk for the epilepsy phenotype and should be considered during genetic counseling. PMID:24318420

  2. CT scanning of the brain and lumbar CSF monoamine metabolites in spinocerebellar degenerative disorders

    Energy Technology Data Exchange (ETDEWEB)

    Sasaki, Hidenao; Kanazawa, Ichiro; Nakanishi, Takao; Kuramoto, Kenmei [Tsukuba Univ., Sakura, Ibaraki (Japan)

    1984-08-01

    Eight patients with parenchymatous cerebellar degeneration (PCD) group (3 with late cortical cerebellar atrophy and 5 with Holmes' hereditary ataxia), 14 with olivo-ponto-cerebellar atrophy (OPCA) group (4 with Shy-Drager syndrome, 6 with OPCA without family history and 4 with Menzel type SCS), 15 with Parkinson's disease and 44 control with other neurological diseases were studied. In all the spinocerebellar degenerative disorders (SCD) cases, CVI values corresponding to the cerebellar atrophy were definitely reduced. On the other hand, PVI values corresponding to the pontine atrophy were only significantly decreased in OPCA group. However, since there were several cases showing only questionable pontine atrophy, it seems difficult to clearly differentiate individual OPCA cases from other SCD cases on CT films alone. Concerning monoamine metabolites in CSF, it was noted that a significant reduction of HVA and total MHPG was found in the OPCA group. Among them, the patients with overt autonomic failure showed the lowest HVA level and the cases of Menzel type of SCD showed a slight reduction of HVA but an unexpected elevation of free MHPG values. The cases of Parkinson's disease showed a definite reduction of HVA. On the other hand, the cases of PCD group showed no significant difference against controls. 5-HIAA levels were not significantly different among the SCD subgroups.

  3. A novel nuclear DnaJ protein, DNAJC8, can suppress the formation of spinocerebellar ataxia 3 polyglutamine aggregation in a J-domain independent manner

    Energy Technology Data Exchange (ETDEWEB)

    Ito, Norie [Department of Pathology, Sapporo Medical University School of Medicine, South-1, West-17, Chuo-ku, Sapporo 060-8556 (Japan); Department of Neurology, Sapporo Medical University School of Medicine, South-1, West-17, Chuo-ku, Sapporo 060-8556 (Japan); Kamiguchi, Kenjiro; Nakanishi, Katsuya; Sokolovskya, Alice; Hirohashi, Yoshihiko; Tamura, Yasuaki; Murai, Aiko; Yamamoto, Eri; Kanaseki, Takayuki; Tsukahara, Tomohide; Kochin, Vitaly [Department of Pathology, Sapporo Medical University School of Medicine, South-1, West-17, Chuo-ku, Sapporo 060-8556 (Japan); Chiba, Susumu [Department of Neurology, Clinical Brain Research Laboratory, Toyokura Memorial Hall, Sapporo Yamano-ue Hospital (Japan); Shimohama, Shun [Department of Neurology, Sapporo Medical University School of Medicine, South-1, West-17, Chuo-ku, Sapporo 060-8556 (Japan); Sato, Noriyuki [Department of Pathology, Sapporo Medical University School of Medicine, South-1, West-17, Chuo-ku, Sapporo 060-8556 (Japan); Torigoe, Toshihiko, E-mail: torigoe@sapmed.ac.jp [Department of Pathology, Sapporo Medical University School of Medicine, South-1, West-17, Chuo-ku, Sapporo 060-8556 (Japan)

    2016-06-10

    Polyglutamine (polyQ) diseases comprise neurodegenerative disorders caused by expression of expanded polyQ-containing proteins. The cytotoxicity of the expanded polyQ-containing proteins is closely associated with aggregate formation. In this study, we report that a novel J-protein, DNAJ (HSP40) Homolog, Subfamily C, Member 8 (DNAJC8), suppresses the aggregation of polyQ-containing protein in a cellular model of spinocerebellar ataxia type 3 (SCA3), which is also known as Machado-Joseph disease. Overexpression of DNAJC8 in SH-SY5Y neuroblastoma cells significantly reduced the polyQ aggregation and apoptosis, and DNAJC8 was co-localized with the polyQ aggregation in the cell nucleus. Deletion mutants of DNAJC8 revealed that the C-terminal domain of DNAJC8 was essential for the suppression of polyQ aggregation, whereas the J-domain was dispensable. Furthermore, 22-mer oligopeptide derived from C-termilal domain could suppress the polyQ aggregation. These results indicate that DNAJC8 can suppress the polyQ aggregation via a distinct mechanism independent of HSP70-based chaperone machinery and have a unique protective role against the aggregation of expanded polyQ-containing proteins such as pathogenic ataxin-3 proteins.

  4. Rare Disease Patient Registry & Natural History Study - Coordination of Rare Diseases at Sanford

    Science.gov (United States)

    2017-09-28

    -Hirschhorn Syndrome; 4p16.3 Microduplication Syndrome; 4p Deletion Syndrome, Non-Wolf-Hirschhorn Syndrome; Autosomal Recessive Stickler Syndrome; Stickler Syndrome Type 2; Stickler Syndrome Type 1; Stickler Syndrome; Mucolipidosis Type 4; X-linked Spinocerebellar Ataxia Type 4; X-linked Spinocerebellar Ataxia Type 3; X-linked Intellectual Disability - Ataxia - Apraxia; X-linked Progressive Cerebellar Ataxia; X-linked Non Progressive Cerebellar Ataxia; X-linked Cerebellar Ataxia; Vitamin B12 Deficiency Ataxia; Toxic Exposure Ataxia; Unclassified Autosomal Dominant Spinocerebellar Ataxia; Thyroid Antibody Ataxia; Sporadic Adult-onset Ataxia of Unknown Etiology; Spinocerebellar Ataxia With Oculomotor Anomaly; Spinocerebellar Ataxia With Epilepsy; Spinocerebellar Ataxia With Axonal Neuropathy Type 2; Spinocerebellar Ataxia Type 8; Spinocerebellar Ataxia Type 7; Spinocerebellar Ataxia Type 6; Spinocerebellar Ataxia Type 5; Spinocerebellar Ataxia Type 4; Spinocerebellar Ataxia Type 37; Spinocerebellar Ataxia Type 36; Spinocerebellar Ataxia Type 35; Spinocerebellar Ataxia Type 34; Spinocerebellar Ataxia Type 32; Spinocerebellar Ataxia Type 31; Spinocerebellar Ataxia Type 30; Spinocerebellar Ataxia Type 3; Spinocerebellar Ataxia Type 29; Spinocerebellar Ataxia Type 28; Spinocerebellar Ataxia Type 27; Spinocerebellar Ataxia Type 26; Spinocerebellar Ataxia Type 25; Spinocerebellar Ataxia Type 23; Spinocerebellar Ataxia Type 22; Spinocerebellar Ataxia Type 21; Spinocerebellar Ataxia Type 20; Spinocerebellar Ataxia Type 2; Spinocerebellar Ataxia Type 19/22; Spinocerebellar Ataxia Type 18; Spinocerebellar Ataxia Type 17; Spinocerebellar Ataxia Type 16; Spinocerebellar Ataxia Type 15/16; Spinocerebellar Ataxia Type 14; Spinocerebellar Ataxia Type 13; Spinocerebellar Ataxia Type 12; Spinocerebellar Ataxia Type 11; Spinocerebellar Ataxia Type 10; Spinocerebellar Ataxia Type 1 With Axonal Neuropathy; Spinocerebellar Ataxia Type 1; Spinocerebellar Ataxia - Unknown; Spinocerebellar Ataxia - Dysmorphism

  5. Spinocerebellar ataxia type 29 due to mutations in ITPR1: a case series and review of this emerging congenital ataxia.

    Science.gov (United States)

    Zambonin, Jessica L; Bellomo, Allison; Ben-Pazi, Hilla; Everman, David B; Frazer, Lee M; Geraghty, Michael T; Harper, Amy D; Jones, Julie R; Kamien, Benjamin; Kernohan, Kristin; Koenig, Mary Kay; Lines, Matthew; Palmer, Elizabeth Emma; Richardson, Randal; Segel, Reeval; Tarnopolsky, Mark; Vanstone, Jason R; Gibbons, Melissa; Collins, Abigail; Fogel, Brent L; Dudding-Byth, Tracy; Boycott, Kym M

    2017-06-28

    Spinocerebellar ataxia type 29 (SCA29) is an autosomal dominant, non-progressive cerebellar ataxia characterized by infantile-onset hypotonia, gross motor delay and cognitive impairment. Affected individuals exhibit cerebellar dysfunction and often have cerebellar atrophy on neuroimaging. Recently, missense mutations in ITPR1 were determined to be responsible. Clinical information on 21 individuals from 15 unrelated families with ITPR1 mutations was retrospectively collected using standardized questionnaires, including 11 previously unreported singletons and 2 new patients from a previously reported family. We describe the genetic, clinical and neuroimaging features of these patients to further characterize the clinical features of this rare condition and assess for any genotype-phenotype correlation for this disorder. Our cohort consisted of 9 males and 12 females, with ages ranging from 28 months to 49 years. Disease course was non-progressive with infantile-onset hypotonia and delays in motor and speech development. Gait ataxia was present in all individuals and 10 (48%) were not ambulating independently between the ages of 3-12 years of age. Mild-to-moderate cognitive impairment was present in 17 individuals (85%). Cerebellar atrophy developed after initial symptom presentation in 13 individuals (72%) and was not associated with disease progression or worsening functional impairment. We identified 12 different mutations including 6 novel mutations; 10 mutations were missense (with 4 present in >1 individual), 1 a splice site mutation leading to an in-frame insertion and 1 an in-frame deletion. No specific genotype-phenotype correlations were observed within our cohort. Our findings document significant clinical heterogeneity between individuals with SCA29 in a large cohort of molecularly confirmed cases. Based on the retrospective observed clinical features and disease course, we provide recommendations for management. Further research into the natural

  6. Targeting the CACNA1A IRES as a Treatment for Spinocerebellar Ataxia Type 6.

    Science.gov (United States)

    Pastor, Parviz Daniel Hejazi; Du, Xiaofei; Fazal, Sarah; Davies, Andre N; Gomez, Christopher M

    2018-02-01

    We have discovered that the P/Q-type voltage-gated Ca 2+ channel (VGCC) gene, CACNA1A, encodes both the α1A (Cav2.1) subunit and a newly recognized transcription factor, α1ACT, by means of a novel internal ribosomal entry site (IRES) within the α1A C-terminal coding region. α1ACT, when mutated with an expansion of the polyglutamine tract in the C-terminus, gives rise to spinocerebellar ataxia type 6 (SCA6). Because silencing of the entire CACNA1A gene would result in the loss of the essential Cav2.1 channel, the IRES controlling α1ACT expression is an excellent target for selective silencing of α1ACT as a therapeutic intervention for SCA6. We performed a high-throughput screen of FDA-approved small molecules using a dual luciferase reporter system and identified ten hits able to selectively inhibit the IRES. We identified four main candidates that showed selective suppression of α1ACT relative to α1A in HEK cells expressing a native CACNA1A vector. We previously pursued another avenue of molecular intervention through miRNA silencing. We studied three human miRNAs (miRNA-711, -3191-5p, -4786) that would potentially bind to sequences within the CACNA1A IRES region, based on an miRNA prediction program. Only miRNA-3191-5p was found to selectively inhibit the translation of α1ACT in cells. We developed a hyperacute model of SCA6 in mice by injecting a pathogenic form of the IRES-mediated α1ACT (AAV9-α1ACTQ33). Finally, we tested the effectiveness of the miRNA therapy by co-expressing either control miRNA or miRNA-3191-5p and found that miRNA-3191-5p decreased the levels of α1ACTQ33 and prevented the hyperacute disease in mice. These studies provide the proof of principle that a therapy directed at selectively preventing α1ACT expression could be used to treat SCA6.

  7. MOS modeling hierarchy including radiation effects

    International Nuclear Information System (INIS)

    Alexander, D.R.; Turfler, R.M.

    1975-01-01

    A hierarchy of modeling procedures has been developed for MOS transistors, circuit blocks, and integrated circuits which include the effects of total dose radiation and photocurrent response. The models were developed for use with the SCEPTRE circuit analysis program, but the techniques are suitable for other modern computer aided analysis programs. The modeling hierarchy permits the designer or analyst to select the level of modeling complexity consistent with circuit size, parametric information, and accuracy requirements. Improvements have been made in the implementation of important second order effects in the transistor MOS model, in the definition of MOS building block models, and in the development of composite terminal models for MOS integrated circuits

  8. Misclassification of Patients with Spinocerebellar Ataxia as having Psychogenic Postural Instability based on Computerized Dynamic Posturography

    Directory of Open Access Journals (Sweden)

    Susan J Herdman

    2011-04-01

    Full Text Available Specific criteria have been developed based on computerized dynamic posturography (CDP to assist clinicians in identifying patients with psychogenic balance problems1-4. Patients with known Spinocerebellar Ataxia (SCA meet several of the criteria for psychogenic balance problem and risk being misclassified as having imbalance of psychogenic origin. However, our research shows that patients with SCA may be distinguished from patients with psychogenic balance problems in several ways. We compared test performance on CDP and the observation of specific behaviors that are associated with psychogenic balance problems in patients with SCA (n = 43 and patients with known psychogenic balance problems (n = 40. Chi square analysis was used to determine if there were significant differences between the groups for the frequency of each criterion for psychogenic CDP and Observed Behaviors. Level of significance was Bonferroni corrected for multiple comparisons. Sensitivity, specificity, and positive likelihood ratios were calculated for each criterion. Hierarchical cluster analysis was used to examine whether the two patient groups demonstrated similar groupings of criteria. Comparison of the results of these analyses identified two criteria that were significantly more frequent in the Psychogenic group than in the SCA group: Regular Periodicity of sway and Circular Sway. Sensitivity, specificity and positive likelihood ratios identified two additional criteria, Inconsistent Motor Responses and Large lateral Sway that also seem to suggest a psychogenic component to a person’s imbalance. Prospective studies are needed to validate the usefulness of these findings.

  9. Social and cultural elements associated with neurocognitive dysfunctions in Spinocerebellar Ataxia Type 2 patients

    Directory of Open Access Journals (Sweden)

    Roberto Emmanuele Mercadillo

    2015-06-01

    Full Text Available Spinocerebellar Ataxia Type 2 (SCA2 is a rare genetic disorder producing cerebellar degeneration and affecting motor abilities. Neuroimaging studies also show neurodegeneration in subcortical and cortical regions related to emotional and social processes. From social neuroscience it is suggested that motor and social abilities can be influenced by particular cultural dynamics so, culture is fundamental to understand the effect of brain related alterations. Here we present the first analysis about the cultural elements related to the SCA2 disorder in 15 patients previously evaluated with neuroimaging and psychometric instruments, and their nuclear relationships distributed in six geographical and cultural regions in Mexico. Ethnographic records and photographic and video archives about the quotidian participant’s routine were obtained from the patients, their relatives and their caregivers. The information was categorized and interpreted taking into consideration cultural issues and patients’ medical files. Our analyses suggest that most of the participants do not understand the nature of the disease and this misunderstanding favors magic and non-medical explanations. Patients’ testimonies suggest a decrease in pain perception as well as motor alterations that may be related to interoceptive dysfunctions. Relatives’ testimonies indicate patients’ lack of social and emotional interests that may be related to frontal, temporal and cerebellar degeneration. In general, participants use their religious beliefs to deal with the disease and only a few of them trust the health system. Patients and their families are either openly rejected and ignored, tolerated or even helped by their community accordingly to different regional traits. We propose that ethnography can provide social representations to understand the patients’ alterations, to formulate neurobiological hypotheses, to develop neurocognitive interventions, and to improve the

  10. PET Study in a Patient with Spinocerebellar Degeneration before and after Long-Term Administration of Thyrotropin Releasing Hormone

    Directory of Open Access Journals (Sweden)

    H. Tanji

    1996-01-01

    Full Text Available We studied the chronic effect of thyrotropin releasing hormone (TRH in a patient with spinocerebellar degeneration by measuring cerebral metabolic rate for glucose (CMRG1c using 2-[18F]fluoro-2-deoxy-D-glucose (18FDG and positron emission tomography (PET. A 56-year-old female, who had suffered from progressive ataxia for 2 years, was treated by intravenous administration of 2 mg TRH for 3 weeks, and CMRG1c of the brain was measured before and after treatment. CMRG1c was markedly decreased in the cerebellum and there was no significant difference before and after the treatment, i.e. mean CMRG1c values were 4.92 and 4.90 mg/100 g/min, and the ratios of the cerebellum versus the frontal cortex were 0.50 and 0.51, respectively. The degree of disequilibrium of her body examined with stabilography became better by the 19th day and further improved by the 26th day after the start of TRH treatment. Based on the present study we conclude that long-term administration of TRH did not improve CMRG1c in the cerebellum, but evidently improved the sway of gravity center by stabilography. We speculate that the chronic effect of TRH was not necessarily due to an improvement of cerebellar function, because TRH receptors are widely distributed throughout the central nervous system.

  11. Double-blind crossover trial of trimethoprim-sulfamethoxazole in spinocerebellar ataxia type 3/Machado-Joseph disease.

    Science.gov (United States)

    Schulte, T; Mattern, R; Berger, K; Szymanski, S; Klotz, P; Kraus, P H; Przuntek, H; Schöls, L

    2001-09-01

    To evaluate the efficiency of a combination of trimethoprim and sulfamethoxazole in patients with spinocerebellar ataxia type 3/Machado-Joseph disease (SCA3/MJD). Placebo-controlled, double-blind crossover trial in 22 patients with genetically confirmed SCA3/MJD. Study phases of 6 months were separated by a washout period of 4 weeks. Dosages were a combination of trimethoprim, 160 mg, and sulfamethoxazole, 800 mg, twice daily for 2 weeks, followed by a combination of trimethoprim, 80 mg, and sulfamethoxazole, 400 mg, twice daily for 5.5 months. Outpatient department of the Neurological Clinic, Ruhr-University, Bochum, Germany. Ataxia ranking scale, self-assessment score, static posturography, and results of motor performance testing. Effects on the visual system were studied using the achromatic Vision Contrast Test System and the Farnsworth-Munsell 100-hue test for color discrimination. Physical and mental health were documented using the Medical Outcomes Study 36-Item Short-Form Health Survey. Subgroup analyses assessed the influence of age, sex, age at onset, duration of the disease, phenotype, and CAG repeat length on test performance. Twenty of 22 patients completed the study. Dropouts were due to a rash (placebo phase) and an attempted suicide in a family conflict. Trimethoprim-sulfamethoxazole therapy had no significant effect in SCA3/MJD patients in the short-term analysis (2 weeks) or in the long-term interval (6 months). In contrast to previous reports that studied smaller groups of patients, treatment with trimethoprim-sulfamethoxazole did not improve the diverse and complex movement disorders caused by SCA3/MJD. Trimethoprim-sulfamethoxazole had no effect on the visual system and cannot be recommended as a continuous treatment for SCA3/MJD patients.

  12. Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7.

    Science.gov (United States)

    Rüb, Udo; Schöls, Ludger; Paulson, Henry; Auburger, Georg; Kermer, Pawel; Jen, Joanna C; Seidel, Kay; Korf, Horst-Werner; Deller, Thomas

    2013-05-01

    The spinocerebellar ataxias type 1 (SCA1), 2 (SCA2), 3 (SCA3), 6 (SCA6) and 7 (SCA7) are genetically defined autosomal dominantly inherited progressive cerebellar ataxias (ADCAs). They belong to the group of CAG-repeat or polyglutamine diseases and share pathologically expanded and meiotically unstable glutamine-encoding CAG-repeats at distinct gene loci encoding elongated polyglutamine stretches in the disease proteins. In recent years, progress has been made in the understanding of the pathogenesis of these currently incurable diseases: Identification of underlying genetic mechanisms made it possible to classify the different ADCAs and to define their clinical and pathological features. Furthermore, advances in molecular biology yielded new insights into the physiological and pathophysiological role of the gene products of SCA1, SCA2, SCA3, SCA6 and SCA7 (i.e. ataxin-1, ataxin-2, ataxin-3, α-1A subunit of the P/Q type voltage-dependent calcium channel, ataxin-7). In the present review we summarize our current knowledge about the polyglutamine ataxias SCA1, SCA2, SCA3, SCA6 and SCA7 and compare their clinical and electrophysiological features, genetic and molecular biological background, as well as their brain pathologies. Furthermore, we provide an overview of the structure, interactions and functions of the different disease proteins. On the basis of these comprehensive data, similarities, differences and possible disease mechanisms are discussed. Copyright © 2013 Elsevier Ltd. All rights reserved.

  13. The history of spinocerebellar ataxia type 10 in Brazil: travels of a gene A história da ataxia espinocerebelar tipo 10 no Brasil: as viagens de um gene

    Directory of Open Access Journals (Sweden)

    Hélio A.G. Teive

    2007-12-01

    Full Text Available The authors report the history of spinocerebellar ataxia 10 (SCA10, since its first report in a large Portuguese-ancestry Family with autosomal dominant pure cerebellar ataxia, till the final identification of further families without Mexican ancestry. These families present a quite different phenotype from those SCA10 families described in Mexico.Os autores apresentam a história da descoberta da ataxia espinocerebelar tipo 10 (AEC10 no Brasil, desde o primeiro relato em uma família com ancestrais portugueses com ataxia cerebelar pura, autossômica dominante, até a identificação de famílias sem ancestrais mexicanos. Essas famílias apresentam um fenótipo de AEC10, com ataxia cerebelar "pura", distinta daquele descrito nas famílias no México.

  14. A novel missense mutation in CCDC88C activates the JNK pathway and causes a dominant form of spinocerebellar ataxia.

    Science.gov (United States)

    Tsoi, Ho; Yu, Allen C S; Chen, Zhefan S; Ng, Nelson K N; Chan, Anne Y Y; Yuen, Liz Y P; Abrigo, Jill M; Tsang, Suk Ying; Tsui, Stephen K W; Tong, Tony M F; Lo, Ivan F M; Lam, Stephen T S; Mok, Vincent C T; Wong, Lawrence K S; Ngo, Jacky C K; Lau, Kwok-Fai; Chan, Ting-Fung; Chan, H Y Edwin

    2014-09-01

    Spinocerebellar ataxias (SCAs) are a group of clinically and genetically diverse and autosomal-dominant disorders characterised by neurological deficits in the cerebellum. At present, there is no cure for SCAs. Of the different distinct subtypes of autosomal-dominant SCAs identified to date, causative genes for only a fraction of them are currently known. In this study, we investigated the cause of an autosomal-dominant SCA phenotype in a family that exhibits cerebellar ataxia and pontocerebellar atrophy along with a global reduction in brain volume. Whole-exome analysis revealed a missense mutation c.G1391A (p.R464H) in the coding region of the coiled-coil domain containing 88C (CCDC88C) gene in all affected individuals. Functional studies showed that the mutant form of CCDC88C activates the c-Jun N-terminal kinase (JNK) pathway, induces caspase 3 cleavage and triggers apoptosis. This study expands our understanding of the cause of autosomal-dominant SCAs, a group of heterogeneous congenital neurological conditions in humans, and unveils a link between the JNK stress pathway and cerebellar atrophy. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  15. The SCA1 (Spinocerebellar ataxia type 1 and MJD (Machado-Joseph disease CAG repeats in normal individuals: segregation analysis and allele frequencies

    Directory of Open Access Journals (Sweden)

    Cláudia Emília Vieira Wiezel

    2003-01-01

    Full Text Available Spinocerebellar ataxia type 1 (SCA1 and Machado-Joseph disease (MJD/SCA3 are autosomal dominant neurodegenerative diseases caused by expansions of a CAG trinucleotide repeat in the SCA1 and MJD genes. These expanded sequences are unstable upon transmission, leading to an intergeneration increase in the number of repeats (dynamic mutation. The transmission of the CAG repeat was studied in normal mother-father-child trios, referred for paternity testing (SCA1, n = 367; MJD, n = 879. No segregation distortion was detected. The CAG allele frequencies were determined in 330 unrelated individuals (fathers from couples tested for paternity. The allele frequency distributions did not differ from those previously reported for European populations. The estimated values for the statistic parameters indicating diversity at the SCA1 locus did not differ much from those reported previously for other STRs in the Brazilian population, while those for the MJD locus were close to or higher than the maximum values of previous reports. This shows that SCA1 and MJD are highly informative loci for applications in genetic and population studies and for forensic analysis.

  16. Challenges in sleep stage R scoring in patients with autosomal dominant spinocerebellar ataxias (SCA1, SCA2 and SCA3) and oculomotor abnormalities: a whole night polysomnographic evaluation.

    Science.gov (United States)

    Seshagiri, Doniparthi Venkata; Sasidharan, Arun; Kumar, Gulshan; Pal, Pramod Kumar; Jain, Sanjeev; Kutty, Bindu M; Yadav, Ravi

    2018-02-01

    Spinocerebellar ataxias are progressive neurodegenerative disorders characterized by progressive cerebellar features with additional neuro-axis involvement. Oculomotor abnormality is one of the most frequent manifestations. This study was done to assess the polysomnographic abnormalities in patients with Spinocerebellar ataxia (SCA1, SCA2 and SCA3) and also to evaluate whether oculomotor abnormalities interfere with sleep stage R scoring. The study was carried out using 36 genetically positive SCA patients. All patients underwent neurological examination with special focus on oculomotor function (optokinetic nystagmus-OKN and extraocular movement restriction-EOM). The sleep quality was measured with Pittsburgh Sleep Quality Index (PSQI) and Epworth Sleepiness Scale (ESS). Disease severity was assessed with International Cooperative Ataxia Rating Scale (ICARS). All the patients underwent over-night video-polysomnography (VPSG). Out of 36 patients studied, the data of 34 patients [SCA1 (n = 12), SCA2 (n = 13), SCA3 (n = 9)] were used for final analysis. Patients from SCA1, SCA2, and SCA3 category did not show significant differences in age and diseases severity (ICARS). All patients had vertical OKN impairment. Oculomotor impairment was higher in SCA2 patients. Sleep macro-architecture analysis showed absent stage R sleep, predominantly in SCA2 (69%) followed by SCA3 (44%) and SCA1 (8%). Patients showed a strong negative correlation of stage R sleep percentage with disease severity and oculomotor dysfunction. Voluntary saccadic eye movement velocity and rapid eye movements (REMs) in sleep are strongly correlated. The more severe the saccadic velocity impairment, the less likely was it to generate REMs (rapid eye movements) during stage R. Accordingly 69% of SCA2 patients with severe occulomotor impairments showed absent stage R as per the AASM sleep scoring. We presume that the impaired REMs generation in sleep could be due to oculomotor abnormality and has

  17. Mangifera indica L. extract (Vimang improves the aversive memory in spinocerebellar ataxia type 2 transgenic mice.

    Directory of Open Access Journals (Sweden)

    Natasha Maurmann

    2014-06-01

    Full Text Available Context: The spinocerebellar ataxia type 2 (SCA-2 is a progressive neurodegenerative disorder without specific therapy identified, and it is related to the loss of function in the cerebellum, mitochondrial dysfunction, oxidative stress and neurotoxic processes. Scientific evidence indicates that Mangifera indica L. aqueous extract (MiE and its major constituent (mangiferin display antioxidant, anti-inflammatory and neuroprotective actions. Aims: To investigate the MiE and mangiferin effects on behavioral outcomes of neurological function in SCA-2 transgenic mice. Methods: The SCA-2 transgenic mice were daily and orally administered during 12 months with MiE (10, 50, and 100 mg/kg, mangiferin (10 mg/kg or vehicle. It was evaluated locomotion (open-field, aversive memory (inhibitory avoidance and declarative memory (object recognition. To explore possible cellular mechanisms underlying the in vivo effects was also evaluated their effects on nerve grow factor (NGF and tumor necrosis factor-α (TNF-α levels in the human glioblastoma cell line U138-MG supernatant. Results: MiE administration did not affect the object recognition memory, but mangiferin did. The natural extract improved selectively the aversive memory in SCA-2 mice, indicating that MiE can affect behavioral parameters regarding fear-related memory. MiE also induced a significant increase in supernatant levels of NGF and TNF-α in vitro in human U138-MG glioblastoma cells. Conclusions: The results suggest that MiE enhances the aversive memory through a mechanism that might involve an increase in neurotrophin and cytokine levels. These findings constitute the basis for the use of the natural extract in the prevention/treatment of memory deficits in SCA-2.

  18. Modeling neurodegenerative diseases with patient-derived induced pluripotent cells: Possibilities and challenges.

    Science.gov (United States)

    Poon, Anna; Zhang, Yu; Chandrasekaran, Abinaya; Phanthong, Phetcharat; Schmid, Benjamin; Nielsen, Troels T; Freude, Kristine K

    2017-10-25

    The rising prevalence of progressive neurodegenerative diseases coupled with increasing longevity poses an economic burden at individual and societal levels. There is currently no effective cure for the majority of neurodegenerative diseases and disease-affected tissues from patients have been difficult to obtain for research and drug discovery in pre-clinical settings. While the use of animal models has contributed invaluable mechanistic insights and potential therapeutic targets, the translational value of animal models could be further enhanced when combined with in vitro models derived from patient-specific induced pluripotent stem cells (iPSCs) and isogenic controls generated using CRISPR-Cas9 mediated genome editing. The iPSCs are self-renewable and capable of being differentiated into the cell types affected by the diseases. These in vitro models based on patient-derived iPSCs provide the opportunity to model disease development, uncover novel mechanisms and test potential therapeutics. Here we review findings from iPSC-based modeling of selected neurodegenerative diseases, including Alzheimer's disease, frontotemporal dementia and spinocerebellar ataxia. Furthermore, we discuss the possibilities of generating three-dimensional (3D) models using the iPSCs-derived cells and compare their advantages and disadvantages to conventional two-dimensional (2D) models. Copyright © 2017 Elsevier B.V. All rights reserved.

  19. Progressive IRP Models for Power Resources Including EPP

    Directory of Open Access Journals (Sweden)

    Yiping Zhu

    2017-01-01

    Full Text Available In the view of optimizing regional power supply and demand, the paper makes effective planning scheduling of supply and demand side resources including energy efficiency power plant (EPP, to achieve the target of benefit, cost, and environmental constraints. In order to highlight the characteristics of different supply and demand resources in economic, environmental, and carbon constraints, three planning models with progressive constraints are constructed. Results of three models by the same example show that the best solutions to different models are different. The planning model including EPP has obvious advantages considering pollutant and carbon emission constraints, which confirms the advantages of low cost and emissions of EPP. The construction of progressive IRP models for power resources considering EPP has a certain reference value for guiding the planning and layout of EPP within other power resources and achieving cost and environmental objectives.

  20. Including investment risk in large-scale power market models

    DEFF Research Database (Denmark)

    Lemming, Jørgen Kjærgaard; Meibom, P.

    2003-01-01

    Long-term energy market models can be used to examine investments in production technologies, however, with market liberalisation it is crucial that such models include investment risks and investor behaviour. This paper analyses how the effect of investment risk on production technology selection...... can be included in large-scale partial equilibrium models of the power market. The analyses are divided into a part about risk measures appropriate for power market investors and a more technical part about the combination of a risk-adjustment model and a partial-equilibrium model. To illustrate...... the analyses quantitatively, a framework based on an iterative interaction between the equilibrium model and a separate risk-adjustment module was constructed. To illustrate the features of the proposed modelling approach we examined how uncertainty in demand and variable costs affects the optimal choice...

  1. Dantrolene is neuroprotective in Huntington's disease transgenic mouse model

    Directory of Open Access Journals (Sweden)

    Chen Xi

    2011-11-01

    Full Text Available Abstract Background Huntington's disease (HD is a progressive neurodegenerative disorder caused by a polyglutamine expansion in the Huntingtin protein which results in the selective degeneration of striatal medium spiny neurons (MSNs. Our group has previously demonstrated that calcium (Ca2+ signaling is abnormal in MSNs from the yeast artificial chromosome transgenic mouse model of HD (YAC128. Moreover, we demonstrated that deranged intracellular Ca2+ signaling sensitizes YAC128 MSNs to glutamate-induced excitotoxicity when compared to wild type (WT MSNs. In previous studies we also observed abnormal neuronal Ca2+ signaling in neurons from spinocerebellar ataxia 2 (SCA2 and spinocerebellar ataxia 3 (SCA3 mouse models and demonstrated that treatment with dantrolene, a ryanodine receptor antagonist and clinically relevant Ca2+ signaling stabilizer, was neuroprotective in experiments with these mouse models. The aim of the current study was to evaluate potential beneficial effects of dantrolene in experiments with YAC128 HD mouse model. Results The application of caffeine and glutamate resulted in increased Ca2+ release from intracellular stores in YAC128 MSN cultures when compared to WT MSN cultures. Pre-treatment with dantrolene protected YAC128 MSNs from glutamate excitotoxicty, with an effective concentration of 100 nM and above. Feeding dantrolene (5 mg/kg twice a week to YAC128 mice between 2 months and 11.5 months of age resulted in significantly improved performance in the beam-walking and gait-walking assays. Neuropathological analysis revealed that long-term dantrolene feeding to YAC128 mice significantly reduced the loss of NeuN-positive striatal neurons and reduced formation of Httexp nuclear aggregates. Conclusions Our results support the hypothesis that deranged Ca2+ signaling plays an important role in HD pathology. Our data also implicate the RyanRs as a potential therapeutic target for the treatment of HD and demonstrate that Ryan

  2. Dantrolene is neuroprotective in Huntington's disease transgenic mouse model.

    Science.gov (United States)

    Chen, Xi; Wu, Jun; Lvovskaya, Svetlana; Herndon, Emily; Supnet, Charlene; Bezprozvanny, Ilya

    2011-11-25

    Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a polyglutamine expansion in the Huntingtin protein which results in the selective degeneration of striatal medium spiny neurons (MSNs). Our group has previously demonstrated that calcium (Ca2+) signaling is abnormal in MSNs from the yeast artificial chromosome transgenic mouse model of HD (YAC128). Moreover, we demonstrated that deranged intracellular Ca2+ signaling sensitizes YAC128 MSNs to glutamate-induced excitotoxicity when compared to wild type (WT) MSNs. In previous studies we also observed abnormal neuronal Ca2+ signaling in neurons from spinocerebellar ataxia 2 (SCA2) and spinocerebellar ataxia 3 (SCA3) mouse models and demonstrated that treatment with dantrolene, a ryanodine receptor antagonist and clinically relevant Ca2+ signaling stabilizer, was neuroprotective in experiments with these mouse models. The aim of the current study was to evaluate potential beneficial effects of dantrolene in experiments with YAC128 HD mouse model. The application of caffeine and glutamate resulted in increased Ca2+ release from intracellular stores in YAC128 MSN cultures when compared to WT MSN cultures. Pre-treatment with dantrolene protected YAC128 MSNs from glutamate excitotoxicty, with an effective concentration of 100 nM and above. Feeding dantrolene (5 mg/kg) twice a week to YAC128 mice between 2 months and 11.5 months of age resulted in significantly improved performance in the beam-walking and gait-walking assays. Neuropathological analysis revealed that long-term dantrolene feeding to YAC128 mice significantly reduced the loss of NeuN-positive striatal neurons and reduced formation of Httexp nuclear aggregates. Our results support the hypothesis that deranged Ca2+ signaling plays an important role in HD pathology. Our data also implicate the RyanRs as a potential therapeutic target for the treatment of HD and demonstrate that RyanR inhibitors and Ca2+ signaling stabilizers such as

  3. Capillary electrophoresis fragment analysis and clone sequencing in detection of dynamic mutations of spinocerebellar ataxia

    Directory of Open Access Journals (Sweden)

    Yuan-yuan CHEN

    2018-04-01

    Full Text Available Objective To estimate the accuracy and stability of capillary electrophoresis fragment analysis and clone sequencing in detecting dynamic mutations of spinocerebellar ataxia (SCA. Methods Capillary electrophoresis fragment analysis and clone sequencing were used in detecting trinucleotide repeated sequence of 14 SCA patients (3 cases of SCA2, 2 cases of SCA7, 7 cases of SCA8 and 2 cases of SCA17. Results Capillary electrophoresis fragment analysis of 3 SCA2 cases showed the expanded cytosine-adenine-guanine (CAG repeats were 31, 30 and 32, and the copy numbers of 3 clone sequencing for 3 colonies in each case were 37/40/40, 37/38/39 and 38/39/40 respectively. Capillary electrophoresis fragment analysis of 2 SCA7 cases showed the expanded CAG repeats were 57 and 34, and the copy numbers of repeats were 69, 74, 75 in 3 colonies of one case, and was 45 in the other case. For the 7 SCA8 cases with the expanded cytosine-thymine-adenine (CTA/cytosine-thymine-guanine (CTG repeats of 99, 111, 104, 92, 89, 104 and 75, the results of clone sequencing were 97, 116, 104, 90, 90, 102 and 76 respectively. For 2 SCA17 cases with the short/expanded CAG repeats of 37/50 and 36/45, the results of clone sequencing were 51/50/52 and 45/44 for 3 and 2 colonies. Conclusions Although the higher mobility of polymerase chain reaction (PCR products containing dynamic mutation in the capillary electrophoresis fragment analysis might cause the deviation for analysis of copy numbers, the deviation was predictable and the results were repeatable. The clone sequencing results showed obvious instability, especially for SCA2 and SCA7 genes, which might owing to their simple CAG repeats. Consequently, clone sequencing is not suited for detection of dynamic mutation, not to mention the quantitative criteria of dynamic mutation sequencing. DOI: 10.3969/j.issn.1672-6731.2018.03.008

  4. Unsteady panel method for complex configurations including wake modeling

    CSIR Research Space (South Africa)

    Van Zyl, Lourens H

    2008-01-01

    Full Text Available implementations of the DLM are however not very versatile in terms of geometries that can be modeled. The ZONA6 code offers a versatile surface panel body model including a separated wake model, but uses a pressure panel method for lifting surfaces. This paper...

  5. Stochastic modelling of two-phase flows including phase change

    International Nuclear Information System (INIS)

    Hurisse, O.; Minier, J.P.

    2011-01-01

    Stochastic modelling has already been developed and applied for single-phase flows and incompressible two-phase flows. In this article, we propose an extension of this modelling approach to two-phase flows including phase change (e.g. for steam-water flows). Two aspects are emphasised: a stochastic model accounting for phase transition and a modelling constraint which arises from volume conservation. To illustrate the whole approach, some remarks are eventually proposed for two-fluid models. (authors)

  6. Population genetics and new insight into range of CAG repeats of spinocerebellar ataxia type 3 in the Han Chinese population.

    Directory of Open Access Journals (Sweden)

    Shi-Rui Gan

    Full Text Available Spinocerebellar ataxia type 3 (SCA3, also called Machado-Joseph disease (MJD, is one of the most common SCAs worldwide and caused by a CAG repeat expansion located in ATXN3 gene. Based on the CAG repeat numbers, alleles of ATXN3 can be divided into normal alleles (ANs, intermediate alleles (AIs and expanded alleles (AEs. It was controversial whether the frequency of large normal alleles (large ANs is related to the prevalence of SCA3 or not. And there were huge chaos in the comprehension of the specific numbers of the range of CAG repeats which is fundamental for genetic analysis of SCA3. To illustrate these issues, we made a novel CAG repeat ladder to detect CAG repeats of ATXN3 in 1003 unrelated Chinese normal individuals and studied haplotypes defined by three single nucleotide polymorphisms (SNPs closed to ATXN3. We found that the number of CAG repeats ranged from 13 to 49, among them, 14 was the most common number. Positive skew, the highest frequency of large ANs and 4 AIs which had never been reported before were found. Also, AEs and large ANs shared the same haplotypes defined by the SNPs. Based on these data and other related studies, we presumed that de novo mutations of ATXN3 emerging from large ANs are at least one survival mechanisms of mutational ATXN3 and we can redefine the range of CAG repeats as: ANs≤44, 45 ≤AIs ≤49 and AEs≥50.

  7. Modeling Electric Double-Layers Including Chemical Reaction Effects

    DEFF Research Database (Denmark)

    Paz-Garcia, Juan Manuel; Johannesson, Björn; Ottosen, Lisbeth M.

    2014-01-01

    A physicochemical and numerical model for the transient formation of an electric double-layer between an electrolyte and a chemically-active flat surface is presented, based on a finite elements integration of the nonlinear Nernst-Planck-Poisson model including chemical reactions. The model works...... for symmetric and asymmetric multi-species electrolytes and is not limited to a range of surface potentials. Numerical simulations are presented, for the case of a CaCO3 electrolyte solution in contact with a surface with rate-controlled protonation/deprotonation reactions. The surface charge and potential...... are determined by the surface reactions, and therefore they depends on the bulk solution composition and concentration...

  8. MODEL OF THE TOKAMAK EDGE DENSITY PEDESTAL INCLUDING DIFFUSIVE NEUTRALS

    International Nuclear Information System (INIS)

    BURRELL, K.H.

    2003-01-01

    OAK-B135 Several previous analytic models of the tokamak edge density pedestal have been based on diffusive transport of plasma plus free-streaming of neutrals. This latter neutral model includes only the effect of ionization and neglects charge exchange. The present work models the edge density pedestal using diffusive transport for both the plasma and the neutrals. In contrast to the free-streaming model, a diffusion model for the neutrals includes the effect of both charge exchange and ionization and is valid when charge exchange is the dominant interaction. Surprisingly, the functional forms for the electron and neutral density profiles from the present calculation are identical to the results of the previous analytic models. There are some differences in the detailed definition of various parameters in the solution. For experimentally relevant cases where ionization and charge exchange rate are comparable, both models predict approximately the same width for the edge density pedestal

  9. BALANCED SCORECARDS EVALUATION MODEL THAT INCLUDES ELEMENTS OF ENVIRONMENTAL MANAGEMENT SYSTEM USING AHP MODEL

    Directory of Open Access Journals (Sweden)

    Jelena Jovanović

    2010-03-01

    Full Text Available The research is oriented on improvement of environmental management system (EMS using BSC (Balanced Scorecard model that presents strategic model of measurem ents and improvement of organisational performance. The research will present approach of objectives and environmental management me trics involvement (proposed by literature review in conventional BSC in "Ad Barska plovi dba" organisation. Further we will test creation of ECO-BSC model based on business activities of non-profit organisations in order to improve envir onmental management system in parallel with other systems of management. Using this approach we may obtain 4 models of BSC that includ es elements of environmen tal management system for AD "Barska plovidba". Taking into acc ount that implementation and evaluation need long period of time in AD "Barska plovidba", the final choice will be based on 14598 (Information technology - Software product evaluation and ISO 9126 (Software engineering - Product quality using AHP method. Those standards are usually used for evaluation of quality software product and computer programs that serve in organisation as support and factors for development. So, AHP model will be bas ed on evolution criteria based on suggestion of ISO 9126 standards and types of evaluation from two evaluation teams. Members of team & will be experts in BSC and environmental management system that are not em ployed in AD "Barska Plovidba" organisation. The members of team 2 will be managers of AD "Barska Plovidba" organisation (including manage rs from environmental department. Merging results based on previously cr eated two AHP models, one can obtain the most appropriate BSC that includes elements of environmental management system. The chosen model will present at the same time suggestion for approach choice including ecological metrics in conventional BSC model for firm that has at least one ECO strategic orientation.

  10. Double-gate junctionless transistor model including short-channel effects

    International Nuclear Information System (INIS)

    Paz, B C; Pavanello, M A; Ávila-Herrera, F; Cerdeira, A

    2015-01-01

    This work presents a physically based model for double-gate junctionless transistors (JLTs), continuous in all operation regimes. To describe short-channel transistors, short-channel effects (SCEs), such as increase of the channel potential due to drain bias, carrier velocity saturation and mobility degradation due to vertical and longitudinal electric fields, are included in a previous model developed for long-channel double-gate JLTs. To validate the model, an analysis is made by using three-dimensional numerical simulations performed in a Sentaurus Device Simulator from Synopsys. Different doping concentrations, channel widths and channel lengths are considered in this work. Besides that, the series resistance influence is numerically included and validated for a wide range of source and drain extensions. In order to check if the SCEs are appropriately described, besides drain current, transconductance and output conductance characteristics, the following parameters are analyzed to demonstrate the good agreement between model and simulation and the SCEs occurrence in this technology: threshold voltage (V TH ), subthreshold slope (S) and drain induced barrier lowering. (paper)

  11. Model for safety reports including descriptive examples

    International Nuclear Information System (INIS)

    1995-12-01

    Several safety reports will be produced in the process of planning and constructing the system for disposal of high-level radioactive waste in Sweden. The present report gives a model, with detailed examples, of how these reports should be organized and what steps they should include. In the near future safety reports will deal with the encapsulation plant and the repository. Later reports will treat operation of the handling systems and the repository

  12. Ubiquitous expression of CUG or CAG trinucleotide repeat RNA causes common morphological defects in a Drosophila model of RNA-mediated pathology.

    Directory of Open Access Journals (Sweden)

    Kynan T Lawlor

    Full Text Available Expanded DNA repeat sequences are known to cause over 20 diseases, including Huntington's disease, several types of spinocerebellar ataxia and myotonic dystrophy type 1 and 2. A shared genetic basis, and overlapping clinical features for some of these diseases, indicate that common pathways may contribute to pathology. Multiple mechanisms, mediated by both expanded homopolymeric proteins and expanded repeat RNA, have been identified by the use of model systems, that may account for shared pathology. The use of such animal models enables identification of distinct pathways and their 'molecular hallmarks' that can be used to determine the contribution of each pathway in human pathology. Here we characterise a tergite disruption phenotype in adult flies, caused by ubiquitous expression of either untranslated CUG or CAG expanded repeat RNA. Using the tergite phenotype as a quantitative trait we define a new genetic system in which to examine 'hairpin' repeat RNA-mediated cellular perturbation. Further experiments use this system to examine whether pathways involving Muscleblind sequestration or Dicer processing, which have been shown to mediate repeat RNA-mediated pathology in other model systems, contribute to cellular perturbation in this model.

  13. Modelling a linear PM motor including magnetic saturation

    NARCIS (Netherlands)

    Polinder, H.; Slootweg, J.G.; Compter, J.C.; Hoeijmakers, M.J.

    2002-01-01

    The use of linear permanent-magnet (PM) actuators increases in a wide variety of applications because of the high force density, robustness and accuracy. The paper describes the modelling of a linear PM motor applied in, for example, wafer steppers, including magnetic saturation. This is important

  14. Olkiluoto surface hydrological modelling: Update 2012 including salt transport modelling

    International Nuclear Information System (INIS)

    Karvonen, T.

    2013-11-01

    Posiva Oy is responsible for implementing a final disposal program for spent nuclear fuel of its owners Teollisuuden Voima Oyj and Fortum Power and Heat Oy. The spent nuclear fuel is planned to be disposed at a depth of about 400-450 meters in the crystalline bedrock at the Olkiluoto site. Leakages located at or close to spent fuel repository may give rise to the upconing of deep highly saline groundwater and this is a concern with regard to the performance of the tunnel backfill material after the closure of the tunnels. Therefore a salt transport sub-model was added to the Olkiluoto surface hydrological model (SHYD). The other improvements include update of the particle tracking algorithm and possibility to estimate the influence of open drillholes in a case where overpressure in inflatable packers decreases causing a hydraulic short-circuit between hydrogeological zones HZ19 and HZ20 along the drillhole. Four new hydrogeological zones HZ056, HZ146, BFZ100 and HZ039 were added to the model. In addition, zones HZ20A and HZ20B intersect with each other in the new structure model, which influences salinity upconing caused by leakages in shafts. The aim of the modelling of long-term influence of ONKALO, shafts and repository tunnels provide computational results that can be used to suggest limits for allowed leakages. The model input data included all the existing leakages into ONKALO (35-38 l/min) and shafts in the present day conditions. The influence of shafts was computed using eight different values for total shaft leakage: 5, 11, 20, 30, 40, 50, 60 and 70 l/min. The selection of the leakage criteria for shafts was influenced by the fact that upconing of saline water increases TDS-values close to the repository areas although HZ20B does not intersect any deposition tunnels. The total limit for all leakages was suggested to be 120 l/min. The limit for HZ20 zones was proposed to be 40 l/min: about 5 l/min the present day leakages to access tunnel, 25 l/min from

  15. Olkiluoto surface hydrological modelling: Update 2012 including salt transport modelling

    Energy Technology Data Exchange (ETDEWEB)

    Karvonen, T. [WaterHope, Helsinki (Finland)

    2013-11-15

    Posiva Oy is responsible for implementing a final disposal program for spent nuclear fuel of its owners Teollisuuden Voima Oyj and Fortum Power and Heat Oy. The spent nuclear fuel is planned to be disposed at a depth of about 400-450 meters in the crystalline bedrock at the Olkiluoto site. Leakages located at or close to spent fuel repository may give rise to the upconing of deep highly saline groundwater and this is a concern with regard to the performance of the tunnel backfill material after the closure of the tunnels. Therefore a salt transport sub-model was added to the Olkiluoto surface hydrological model (SHYD). The other improvements include update of the particle tracking algorithm and possibility to estimate the influence of open drillholes in a case where overpressure in inflatable packers decreases causing a hydraulic short-circuit between hydrogeological zones HZ19 and HZ20 along the drillhole. Four new hydrogeological zones HZ056, HZ146, BFZ100 and HZ039 were added to the model. In addition, zones HZ20A and HZ20B intersect with each other in the new structure model, which influences salinity upconing caused by leakages in shafts. The aim of the modelling of long-term influence of ONKALO, shafts and repository tunnels provide computational results that can be used to suggest limits for allowed leakages. The model input data included all the existing leakages into ONKALO (35-38 l/min) and shafts in the present day conditions. The influence of shafts was computed using eight different values for total shaft leakage: 5, 11, 20, 30, 40, 50, 60 and 70 l/min. The selection of the leakage criteria for shafts was influenced by the fact that upconing of saline water increases TDS-values close to the repository areas although HZ20B does not intersect any deposition tunnels. The total limit for all leakages was suggested to be 120 l/min. The limit for HZ20 zones was proposed to be 40 l/min: about 5 l/min the present day leakages to access tunnel, 25 l/min from

  16. Study of a diffusion flamelet model, with preferential diffusion effects included

    NARCIS (Netherlands)

    Delhaye, S.; Somers, L.M.T.; Bongers, H.; Oijen, van J.A.; Goey, de L.P.H.; Dias, V.

    2005-01-01

    The non-premixed flamelet model of Peters [1] (model1), which does not include preferential diffusion effects is investigated. Two similar models are presented, but without the assumption of unity Lewis numbers. One of these models was derived by Peters & Pitsch [2] (model2), while the other one was

  17. SEEPAGE MODEL FOR PA INCLUDING DRIFT COLLAPSE

    International Nuclear Information System (INIS)

    C. Tsang

    2004-01-01

    The purpose of this report is to document the predictions and analyses performed using the seepage model for performance assessment (SMPA) for both the Topopah Spring middle nonlithophysal (Tptpmn) and lower lithophysal (Tptpll) lithostratigraphic units at Yucca Mountain, Nevada. Look-up tables of seepage flow rates into a drift (and their uncertainty) are generated by performing numerical simulations with the seepage model for many combinations of the three most important seepage-relevant parameters: the fracture permeability, the capillary-strength parameter 1/a, and the percolation flux. The percolation flux values chosen take into account flow focusing effects, which are evaluated based on a flow-focusing model. Moreover, multiple realizations of the underlying stochastic permeability field are conducted. Selected sensitivity studies are performed, including the effects of an alternative drift geometry representing a partially collapsed drift from an independent drift-degradation analysis (BSC 2004 [DIRS 166107]). The intended purpose of the seepage model is to provide results of drift-scale seepage rates under a series of parameters and scenarios in support of the Total System Performance Assessment for License Application (TSPA-LA). The SMPA is intended for the evaluation of drift-scale seepage rates under the full range of parameter values for three parameters found to be key (fracture permeability, the van Genuchten 1/a parameter, and percolation flux) and drift degradation shape scenarios in support of the TSPA-LA during the period of compliance for postclosure performance [Technical Work Plan for: Performance Assessment Unsaturated Zone (BSC 2002 [DIRS 160819], Section I-4-2-1)]. The flow-focusing model in the Topopah Spring welded (TSw) unit is intended to provide an estimate of flow focusing factors (FFFs) that (1) bridge the gap between the mountain-scale and drift-scale models, and (2) account for variability in local percolation flux due to

  18. Atmosphere-soil-vegetation model including CO2 exchange processes: SOLVEG2

    International Nuclear Information System (INIS)

    Nagai, Haruyasu

    2004-11-01

    A new atmosphere-soil-vegetation model named SOLVEG2 (SOLVEG version 2) was developed to study the heat, water, and CO 2 exchanges between the atmosphere and land-surface. The model consists of one-dimensional multilayer sub-models for the atmosphere, soil, and vegetation. It also includes sophisticated processes for solar and long-wave radiation transmission in vegetation canopy and CO 2 exchanges among the atmosphere, soil, and vegetation. Although the model usually simulates only vertical variation of variables in the surface-layer atmosphere, soil, and vegetation canopy by using meteorological data as top boundary conditions, it can be used by coupling with a three-dimensional atmosphere model. In this paper, details of SOLVEG2, which includes the function of coupling with atmosphere model MM5, are described. (author)

  19. Exclusive queueing model including the choice of service windows

    Science.gov (United States)

    Tanaka, Masahiro; Yanagisawa, Daichi; Nishinari, Katsuhiro

    2018-01-01

    In a queueing system involving multiple service windows, choice behavior is a significant concern. This paper incorporates the choice of service windows into a queueing model with a floor represented by discrete cells. We contrived a logit-based choice algorithm for agents considering the numbers of agents and the distances to all service windows. Simulations were conducted with various parameters of agent choice preference for these two elements and for different floor configurations, including the floor length and the number of service windows. We investigated the model from the viewpoint of transit times and entrance block rates. The influences of the parameters on these factors were surveyed in detail and we determined that there are optimum floor lengths that minimize the transit times. In addition, we observed that the transit times were determined almost entirely by the entrance block rates. The results of the presented model are relevant to understanding queueing systems including the choice of service windows and can be employed to optimize facility design and floor management.

  20. RNA FISH for detecting expanded repeats in human diseases.

    Science.gov (United States)

    Urbanek, Martyna O; Krzyzosiak, Wlodzimierz J

    2016-04-01

    RNA fluorescence in situ hybridization (FISH) is a widely used technique for detecting transcripts in fixed cells and tissues. Many variants of RNA FISH have been proposed to increase signal strength, resolution and target specificity. The current variants of this technique facilitate the detection of the subcellular localization of transcripts at a single molecule level. Among the applications of RNA FISH are studies on nuclear RNA foci in diseases resulting from the expansion of tri-, tetra-, penta- and hexanucleotide repeats present in different single genes. The partial or complete retention of mutant transcripts forming RNA aggregates within the nucleoplasm has been shown in multiple cellular disease models and in the tissues of patients affected with these atypical mutations. Relevant diseases include, among others, myotonic dystrophy type 1 (DM1) with CUG repeats, Huntington's disease (HD) and spinocerebellar ataxia type 3 (SCA3) with CAG repeats, fragile X-associated tremor/ataxia syndrome (FXTAS) with CGG repeats, myotonic dystrophy type 2 (DM2) with CCUG repeats, amyotrophic lateral sclerosis/frontotemporal dementia (ALS/FTD) with GGGGCC repeats and spinocerebellar ataxia type 32 (SCA32) with GGCCUG. In this article, we summarize the results obtained with FISH to examine RNA nuclear inclusions. We provide a detailed protocol for detecting RNAs containing expanded CAG and CUG repeats in different cellular models, including fibroblasts, lymphoblasts, induced pluripotent stem cells and murine and human neuronal progenitors. We also present the results of the first single-molecule FISH application in a cellular model of polyglutamine disease. Copyright © 2015 Elsevier Inc. All rights reserved.

  1. Hsp104 suppresses polyglutamine-induced degeneration post onset in a drosophila MJD/SCA3 model.

    Directory of Open Access Journals (Sweden)

    Mimi Cushman-Nick

    Full Text Available There are no effective therapeutics that antagonize or reverse the protein-misfolding events underpinning polyglutamine (PolyQ disorders, including Spinocerebellar Ataxia Type-3 (SCA3. Here, we augment the proteostasis network of Drosophila SCA3 models with Hsp104, a powerful protein disaggregase from yeast, which is bafflingly absent from metazoa. Hsp104 suppressed eye degeneration caused by a C-terminal ataxin-3 (MJD fragment containing the pathogenic expanded PolyQ tract, but unexpectedly enhanced aggregation and toxicity of full-length pathogenic MJD. Hsp104 suppressed toxicity of MJD variants lacking a portion of the N-terminal deubiquitylase domain and full-length MJD variants unable to engage polyubiquitin, indicating that MJD-ubiquitin interactions hinder protective Hsp104 modalities. Importantly, in staging experiments, Hsp104 suppressed toxicity of a C-terminal MJD fragment when expressed after the onset of PolyQ-induced degeneration, whereas Hsp70 was ineffective. Thus, we establish the first disaggregase or chaperone treatment administered after the onset of pathogenic protein-induced degeneration that mitigates disease progression.

  2. Key Characteristics of Combined Accident including TLOFW accident for PSA Modeling

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Bo Gyung; Kang, Hyun Gook [KAIST, Daejeon (Korea, Republic of); Yoon, Ho Joon [Khalifa University of Science, Technology and Research, Abu Dhabi (United Arab Emirates)

    2015-05-15

    The conventional PSA techniques cannot adequately evaluate all events. The conventional PSA models usually focus on single internal events such as DBAs, the external hazards such as fire, seismic. However, the Fukushima accident of Japan in 2011 reveals that very rare event is necessary to be considered in the PSA model to prevent the radioactive release to environment caused by poor treatment based on lack of the information, and to improve the emergency operation procedure. Especially, the results from PSA can be used to decision making for regulators. Moreover, designers can consider the weakness of plant safety based on the quantified results and understand accident sequence based on human actions and system availability. This study is for PSA modeling of combined accidents including total loss of feedwater (TLOFW) accident. The TLOFW accident is a representative accident involving the failure of cooling through secondary side. If the amount of heat transfer is not enough due to the failure of secondary side, the heat will be accumulated to the primary side by continuous core decay heat. Transients with loss of feedwater include total loss of feedwater accident, loss of condenser vacuum accident, and closure of all MSIVs. When residual heat removal by the secondary side is terminated, the safety injection into the RCS with direct primary depressurization would provide alternative heat removal. This operation is called feed and bleed (F and B) operation. Combined accidents including TLOFW accident are very rare event and partially considered in conventional PSA model. Since the necessity of F and B operation is related to plant conditions, the PSA modeling for combined accidents including TLOFW accident is necessary to identify the design and operational vulnerabilities.The PSA is significant to assess the risk of NPPs, and to identify the design and operational vulnerabilities. Even though the combined accident is very rare event, the consequence of combined

  3. Heat shock proteins as potential targets for protective strategies in neurodegeneration

    NARCIS (Netherlands)

    Kampinga, Harm H.; Bergink, Steven

    Protein aggregates are hallmarks of nearly all age-related neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, and several polyglutamine diseases such as Huntington's disease and different forms of spinocerebellar ataxias (SCA; SCA1-3, SCA6,

  4. Deregulation of the actin cytoskeleton and macropinocytosis in response to phorbol ester by the mutant protein kinase C gamma that causes spinocerebellar ataxia type 14

    Directory of Open Access Journals (Sweden)

    Kazuhiro eYamamoto

    2014-04-01

    Full Text Available Several missense mutations in the protein kinase Cγ (γPKC gene have been found to cause spinocerebellar ataxia type 14 (SCA14, an autosomal dominant neurodegenerative disease. γPKC is a neuron-specific member of the classical PKCs and is activated and translocated to subcellular regions as a result of various stimuli, including diacylglycerol synthesis, increased intracellular Ca2+ and phorbol esters. We investigated whether SCA14 mutations affect the γPKC-related functions by stimulating HeLa cells with TPA (12-O-tetradecanoylpholbol 13-acetate, a type of phorbol ester. Wild-type (WT γPKC-GFP was translocated to the plasma membrane within 10 min of TPA stimulation, followed by its perinuclear translocation and cell shrinkage, in a PKC kinase activity- and microtubule-dependent manner. On the other hand, although SCA14 mutant γPKC-GFP exhibited a similar translocation to the plasma membrane, the subsequent perinuclear translocation and cell shrinkage were significantly impaired in response to TPA. Translocated WT γPKC colocalized with F-actin and formed large vesicular structures in the perinuclear region. The uptake of FITC-dextran, a marker of macropinocytosis, was promoted by TPA stimulation in cells expressing WT γPKC, and FITC-dextran was surrounded by γPKC-positive vesicles. Moreover, TPA induced the phosphorylation of MARCKS, which is a membrane-substrate of PKC, resulting in the translocation of phosphorylated MARCKS to the perinuclear region, suggesting that TPA induces macropinocytosis via γPKC activation. However, TPA failed to activate macropinocytosis and trigger the translocation of phosphorylated MARCKS in cells expressing the SCA14 mutant γPKC. These findings suggest that γPKC is involved in the regulation of the actin cytoskeleton and macropinocytosis in HeLa cells, while SCA14 mutant γPKC fails to regulate these processes due to its reduced kinase activity at the plasma membrane. This property might be involved in

  5. RELAP5-3D Code Includes ATHENA Features and Models

    International Nuclear Information System (INIS)

    Riemke, Richard A.; Davis, Cliff B.; Schultz, Richard R.

    2006-01-01

    Version 2.3 of the RELAP5-3D computer program includes all features and models previously available only in the ATHENA version of the code. These include the addition of new working fluids (i.e., ammonia, blood, carbon dioxide, glycerol, helium, hydrogen, lead-bismuth, lithium, lithium-lead, nitrogen, potassium, sodium, and sodium-potassium) and a magnetohydrodynamic model that expands the capability of the code to model many more thermal-hydraulic systems. In addition to the new working fluids along with the standard working fluid water, one or more noncondensable gases (e.g., air, argon, carbon dioxide, carbon monoxide, helium, hydrogen, krypton, nitrogen, oxygen, SF 6 , xenon) can be specified as part of the vapor/gas phase of the working fluid. These noncondensable gases were in previous versions of RELAP5-3D. Recently four molten salts have been added as working fluids to RELAP5-3D Version 2.4, which has had limited release. These molten salts will be in RELAP5-3D Version 2.5, which will have a general release like RELAP5-3D Version 2.3. Applications that use these new features and models are discussed in this paper. (authors)

  6. Mass spectrometry analyses of normal and polyglutamine expanded ataxin-3 reveal novel interaction partners involved in mitochondrial function

    DEFF Research Database (Denmark)

    Kristensen, Line V.; Oppermann, Felix S.; Rauen, Matthias J.

    2018-01-01

    Deubiquitinating enzymes (DUBs) play important roles in a variety of cellular processes, including regulation of protein homeostasis. The DUB ataxin-3 is an enzyme implicated in protein quality control mechanisms. In the neurodegenerative disease spinocerebellar ataxia type 3 (SCA3), ataxin-3...

  7. Improving weather predictability by including land-surface model parameter uncertainty

    Science.gov (United States)

    Orth, Rene; Dutra, Emanuel; Pappenberger, Florian

    2016-04-01

    The land surface forms an important component of Earth system models and interacts nonlinearly with other parts such as ocean and atmosphere. To capture the complex and heterogenous hydrology of the land surface, land surface models include a large number of parameters impacting the coupling to other components of the Earth system model. Focusing on ECMWF's land-surface model HTESSEL we present in this study a comprehensive parameter sensitivity evaluation using multiple observational datasets in Europe. We select 6 poorly constrained effective parameters (surface runoff effective depth, skin conductivity, minimum stomatal resistance, maximum interception, soil moisture stress function shape, total soil depth) and explore their sensitivity to model outputs such as soil moisture, evapotranspiration and runoff using uncoupled simulations and coupled seasonal forecasts. Additionally we investigate the possibility to construct ensembles from the multiple land surface parameters. In the uncoupled runs we find that minimum stomatal resistance and total soil depth have the most influence on model performance. Forecast skill scores are moreover sensitive to the same parameters as HTESSEL performance in the uncoupled analysis. We demonstrate the robustness of our findings by comparing multiple best performing parameter sets and multiple randomly chosen parameter sets. We find better temperature and precipitation forecast skill with the best-performing parameter perturbations demonstrating representativeness of model performance across uncoupled (and hence less computationally demanding) and coupled settings. Finally, we construct ensemble forecasts from ensemble members derived with different best-performing parameterizations of HTESSEL. This incorporation of parameter uncertainty in the ensemble generation yields an increase in forecast skill, even beyond the skill of the default system. Orth, R., E. Dutra, and F. Pappenberger, 2016: Improving weather predictability by

  8. Collisional-radiative model including recombination processes for W27+ ion★

    Science.gov (United States)

    Murakami, Izumi; Sasaki, Akira; Kato, Daiji; Koike, Fumihiro

    2017-10-01

    We have constructed a collisional-radiative (CR) model for W27+ ions including 226 configurations with n ≤ 9 and ł ≤ 5 for spectroscopic diagnostics. We newly include recombination processes in the model and this is the first result of extreme ultraviolet spectrum calculated for recombining plasma component. Calculated spectra in 40-70 Å range in ionizing and recombining plasma components show similar 3 strong lines and 1 line weak in recombining plasma component at 45-50 Å and many weak lines at 50-65 Å for both components. Recombination processes do not contribute much to the spectrum at around 60 Å for W27+ ion. Dielectronic satellite lines are also minor contribution to the spectrum of recombining plasma component. Dielectronic recombination (DR) rate coefficient from W28+ to W27+ ions is also calculated with the same atomic data in the CR model. We found that larger set of energy levels including many autoionizing states gave larger DR rate coefficients but our rate agree within factor 6 with other works at electron temperature around 1 keV in which W27+ and W28+ ions are usually observed in plasmas. Contribution to the Topical Issue "Atomic and Molecular Data and their Applications", edited by Gordon W.F. Drake, Jung-Sik Yoon, Daiji Kato, and Grzegorz Karwasz.

  9. Interferon β induces clearance of mutant ataxin 7 and improves locomotion in SCA7 knock-in mice.

    Science.gov (United States)

    Chort, Alice; Alves, Sandro; Marinello, Martina; Dufresnois, Béatrice; Dornbierer, Jean-Gabriel; Tesson, Christelle; Latouche, Morwena; Baker, Darren P; Barkats, Martine; El Hachimi, Khalid H; Ruberg, Merle; Janer, Alexandre; Stevanin, Giovanni; Brice, Alexis; Sittler, Annie

    2013-06-01

    We showed previously, in a cell model of spinocerebellar ataxia 7, that interferon beta induces the expression of PML protein and the formation of PML protein nuclear bodies that degrade mutant ataxin 7, suggesting that the cytokine, used to treat multiple sclerosis, might have therapeutic value in spinocerebellar ataxia 7. We now show that interferon beta also induces PML-dependent clearance of ataxin 7 in a preclinical model, SCA7(266Q/5Q) knock-in mice, and improves motor function. Interestingly, the presence of mutant ataxin 7 in the mice induces itself the expression of endogenous interferon beta and its receptor. Immunohistological studies in brains from two patients with spinocerebellar ataxia 7 confirmed that these modifications are also caused by the disease in humans. Interferon beta, administered intraperitoneally three times a week in the knock-in mice, was internalized with its receptor in Purkinje and other cells and translocated to the nucleus. The treatment induced PML protein expression and the formation of PML protein nuclear bodies and decreased mutant ataxin 7 in neuronal intranuclear inclusions, the hallmark of the disease. No reactive gliosis or other signs of toxicity were observed in the brain or internal organs. The performance of the SCA7(266Q/5Q) knock-in mice was significantly improved on two behavioural tests sensitive to cerebellar function: the Locotronic® Test of locomotor function and the Beam Walking Test of balance, motor coordination and fine movements, which are affected in patients with spinocerebellar ataxia 7. In addition to motor dysfunction, SCA7(266Q/5Q) mice present abnormalities in the retina as in patients: ataxin 7-positive neuronal intranuclear inclusions that were reduced by interferon beta treatment. Finally, since neuronal death does not occur in the cerebellum of SCA7(266Q/5Q) mice, we showed in primary cell cultures expressing mutant ataxin 7 that interferon beta treatment improves Purkinje cell survival.

  10. Extending Primitive Spatial Data Models to Include Semantics

    Science.gov (United States)

    Reitsma, F.; Batcheller, J.

    2009-04-01

    Our traditional geospatial data model involves associating some measurable quality, such as temperature, or observable feature, such as a tree, with a point or region in space and time. When capturing data we implicitly subscribe to some kind of conceptualisation. If we can make this explicit in an ontology and associate it with the captured data, we can leverage formal semantics to reason with the concepts represented in our spatial data sets. To do so, we extend our fundamental representation of geospatial data in a data model by including a URI in our basic data model that links it to our ontology defining our conceptualisation, We thus extend Goodchild et al's geo-atom [1] with the addition of a URI: (x, Z, z(x), URI) . This provides us with pixel or feature level knowledge and the ability to create layers of data from a set of pixels or features that might be drawn from a database based on their semantics. Using open source tools, we present a prototype that involves simple reasoning as a proof of concept. References [1] M.F. Goodchild, M. Yuan, and T.J. Cova. Towards a general theory of geographic representation in gis. International Journal of Geographical Information Science, 21(3):239-260, 2007.

  11. Conceptualizing a Dynamic Fall Risk Model Including Intrinsic Risks and Exposures.

    Science.gov (United States)

    Klenk, Jochen; Becker, Clemens; Palumbo, Pierpaolo; Schwickert, Lars; Rapp, Kilan; Helbostad, Jorunn L; Todd, Chris; Lord, Stephen R; Kerse, Ngaire

    2017-11-01

    Falls are a major cause of injury and disability in older people, leading to serious health and social consequences including fractures, poor quality of life, loss of independence, and institutionalization. To design and provide adequate prevention measures, accurate understanding and identification of person's individual fall risk is important. However, to date, the performance of fall risk models is weak compared with models estimating, for example, cardiovascular risk. This deficiency may result from 2 factors. First, current models consider risk factors to be stable for each person and not change over time, an assumption that does not reflect real-life experience. Second, current models do not consider the interplay of individual exposure including type of activity (eg, walking, undertaking transfers) and environmental risks (eg, lighting, floor conditions) in which activity is performed. Therefore, we posit a dynamic fall risk model consisting of intrinsic risk factors that vary over time and exposure (activity in context). eHealth sensor technology (eg, smartphones) begins to enable the continuous measurement of both the above factors. We illustrate our model with examples of real-world falls from the FARSEEING database. This dynamic framework for fall risk adds important aspects that may improve understanding of fall mechanisms, fall risk models, and the development of fall prevention interventions. Copyright © 2017 AMDA – The Society for Post-Acute and Long-Term Care Medicine. Published by Elsevier Inc. All rights reserved.

  12. Grand unified models including extra Z bosons

    International Nuclear Information System (INIS)

    Li Tiezhong

    1989-01-01

    The grand unified theories (GUT) of the simple Lie groups including extra Z bosons are discussed. Under authors's hypothesis there are only SU 5+m SO 6+4n and E 6 groups. The general discussion of SU 5+m is given, then the SU 6 and SU 7 are considered. In SU 6 the 15+6 * +6 * fermion representations are used, which are not same as others in fermion content, Yukawa coupling and broken scales. A conception of clans of particles, which are not families, is suggested. These clans consist of extra Z bosons and the corresponding fermions of the scale. The all of fermions in the clans are down quarks except for the standard model which consists of Z bosons and 15 fermions, therefore, the spectrum of the hadrons which are composed of these down quarks are different from hadrons at present

  13. Modeling of cylindrical surrounding gate MOSFETs including the fringing field effects

    International Nuclear Information System (INIS)

    Gupta, Santosh K.; Baishya, Srimanta

    2013-01-01

    A physically based analytical model for surface potential and threshold voltage including the fringing gate capacitances in cylindrical surround gate (CSG) MOSFETs has been developed. Based on this a subthreshold drain current model has also been derived. This model first computes the charge induced in the drain/source region due to the fringing capacitances and considers an effective charge distribution in the cylindrically extended source/drain region for the development of a simple and compact model. The fringing gate capacitances taken into account are outer fringe capacitance, inner fringe capacitance, overlap capacitance, and sidewall capacitance. The model has been verified with the data extracted from 3D TCAD simulations of CSG MOSFETs and was found to be working satisfactorily. (semiconductor devices)

  14. A roller chain drive model including contact with guide-bars

    DEFF Research Database (Denmark)

    Pedersen, Sine Leergaard; Hansen, John Michael; Ambrósio, J. A. C.

    2004-01-01

    A model of a roller chain drive is developed and applied to the simulation and analysis of roller chain drives of large marine diesel engines. The model includes the impact with guide-bars that are the motion delimiter components on the chain strands between the sprockets. The main components...... and the sprocket centre, i.e. a constraint is added when such distance is less than the pitch radius. The unilateral kinematic constraint is removed when its associated constraint reaction force, applied on the roller, is in the direction of the root of the sprocket teeth. In order to improve the numerical...

  15. A constitutive model for the forces of a magnetic bearing including eddy currents

    Science.gov (United States)

    Taylor, D. L.; Hebbale, K. V.

    1993-01-01

    A multiple magnet bearing can be developed from N individual electromagnets. The constitutive relationships for a single magnet in such a bearing is presented. Analytical expressions are developed for a magnet with poles arranged circumferencially. Maxwell's field equations are used so the model easily includes the effects of induced eddy currents due to the rotation of the journal. Eddy currents must be included in any dynamic model because they are the only speed dependent parameter and may lead to a critical speed for the bearing. The model is applicable to bearings using attraction or repulsion.

  16. Including policy and management in socio-hydrology models: initial conceptualizations

    Science.gov (United States)

    Hermans, Leon; Korbee, Dorien

    2017-04-01

    Socio-hydrology studies the interactions in coupled human-water systems. So far, the use of dynamic models that capture the direct feedback between societal and hydrological systems has been dominant. What has not yet been included with any particular emphasis, is the policy or management layer, which is a central element in for instance integrated water resources management (IWRM) or adaptive delta management (ADM). Studying the direct interactions between human-water systems generates knowledges that eventually helps influence these interactions in ways that may ensure better outcomes - for society and for the health and sustainability of water systems. This influence sometimes occurs through spontaneous emergence, uncoordinated by societal agents - private sector, citizens, consumers, water users. However, the term 'management' in IWRM and ADM also implies an additional coordinated attempt through various public actors. This contribution is a call to include the policy and management dimension more prominently into the research focus of the socio-hydrology field, and offers first conceptual variables that should be considered in attempts to include this policy or management layer in socio-hydrology models. This is done by drawing on existing frameworks to study policy processes throughout both planning and implementation phases. These include frameworks such as the advocacy coalition framework, collective learning and policy arrangements, which all emphasis longer-term dynamics and feedbacks between actor coalitions in strategic planning and implementation processes. A case about longter-term dynamics in the management of the Haringvliet in the Netherlands is used to illustrate the paper.

  17. Frequency of the different mutations causing spinocerebellar ataxia (SCA1, SCA2, MJD/SCA3 and DRPLA in a large group of Brazilian patients Freqüência das mutações que causam ataxia espinocerebelar (SCA1, SCA2, MJD/SCA3 e DRPLA em um grupo numeroso de pacientes Brasileiros

    Directory of Open Access Journals (Sweden)

    Iscia Lopes-Cendesi

    1997-09-01

    Full Text Available Spinocerebellar ataxia type 1 (SCA1, spinocerebellar ataxia type 2 (SCA2 and Machado-Joseph disease or spinocerebellar ataxia type 3 (MJD/SCA3 are three distinctive forms of autosomal dominant spinocerebellar ataxia (SCA caused by expansions of an unstable CAG repeat localized in the coding region of the causative genes. Another related disease, dentatorubropallidoluysian atrophy (DRPLA is also caused by an unstable triplet repeat and can present as SCA in late onset patients. We investigated the frequency of the SCA1, SCA2, MJD/SCA3 and DRPLA mutations in 328 Brazilian patients with SCA, belonging to 90 unrelated families with various patterns of inheritance and originating in different geographic regions of Brazil. We found mutations in 35 families (39%, 32 of them with a clear autosomal dominant inheritance. The frequency of the SCA1 mutation was 3% of all patients; and 6 % in the dominantly inherited SCAs. We identified the SCA2 mutation in 6% of all families and in 9% of the families with autosomal dominant inheritance. The MJD/SCA3 mutation was detected in 30 % of all patients; and in the 44% of the dominantly inherited cases. We found no DRPLA mutation. In addition, we observed variability in the frequency of the different mutations according to geographic origin of the patients, which is probably related to the distinct colonization of different parts of Brazil. These results suggest that SCA may be occasionally caused by the SCA1 and SCA2 mutations in the Brazilian population, and that the MJD/SCA3 mutation is the most common cause of dominantly inherited SCA in Brazil.Ataxia espinocerebelar tipo 1 (SCA1, ataxia espinocerebelar tipo 2 (SCA2 e doença de Machado-Joseph ou ataxia espinocerebelar tipo 3 (MJD/SCA3 são três formas de ataxia espinocerebelar (SCA que apresentam herança genética autossômica dominante. Nessas três doenças foi encontrada uma expansão instável de trinucleotídeo CAG localizada na região codificadora dos

  18. A hydrodynamic model for granular material flows including segregation effects

    Science.gov (United States)

    Gilberg, Dominik; Klar, Axel; Steiner, Konrad

    2017-06-01

    The simulation of granular flows including segregation effects in large industrial processes using particle methods is accurate, but very time-consuming. To overcome the long computation times a macroscopic model is a natural choice. Therefore, we couple a mixture theory based segregation model to a hydrodynamic model of Navier-Stokes-type, describing the flow behavior of the granular material. The granular flow model is a hybrid model derived from kinetic theory and a soil mechanical approach to cover the regime of fast dilute flow, as well as slow dense flow, where the density of the granular material is close to the maximum packing density. Originally, the segregation model has been formulated by Thornton and Gray for idealized avalanches. It is modified and adapted to be in the preferred form for the coupling. In the final coupled model the segregation process depends on the local state of the granular system. On the other hand, the granular system changes as differently mixed regions of the granular material differ i.e. in the packing density. For the modeling process the focus lies on dry granular material flows of two particle types differing only in size but can be easily extended to arbitrary granular mixtures of different particle size and density. To solve the coupled system a finite volume approach is used. To test the model the rotational mixing of small and large particles in a tumbler is simulated.

  19. Mathematical Model of Thyristor Inverter Including a Series-parallel Resonant Circuit

    OpenAIRE

    Miroslaw Luft; Elzbieta Szychta

    2008-01-01

    The article presents a mathematical model of thyristor inverter including a series-parallel resonant circuit with theaid of state variable method. Maple procedures are used to compute current and voltage waveforms in the inverter.

  20. Mathematical model of thyristor inverter including a series-parallel resonant circuit

    OpenAIRE

    Luft, M.; Szychta, E.

    2008-01-01

    The article presents a mathematical model of thyristor inverter including a series-parallel resonant circuit with the aid of state variable method. Maple procedures are used to compute current and voltage waveforms in the inverter.

  1. Novel polyglutamine model uncouples proteotoxicity from aging.

    Science.gov (United States)

    Christie, Nakeirah T M; Lee, Amy L; Fay, Hannah G; Gray, Amelia A; Kikis, Elise A

    2014-01-01

    Polyglutamine expansions in certain proteins are the genetic determinants for nine distinct progressive neurodegenerative disorders and resultant age-related dementia. In these cases, neurodegeneration is due to the aggregation propensity and resultant toxic properties of the polyglutamine-containing proteins. We are interested in elucidating the underlying mechanisms of toxicity of the protein ataxin-3, in which a polyglutamine expansion is the genetic determinant for Machado-Joseph Disease (MJD), also referred to as spinocerebellar ataxia 3 (SCA3). To this end, we have developed a novel model for ataxin-3 protein aggregation, by expressing a disease-related polyglutamine-containing fragment of ataxin-3 in the genetically tractable body wall muscle cells of the model system C. elegans. Here, we demonstrate that this ataxin-3 fragment aggregates in a polyQ length-dependent manner in C. elegans muscle cells and that this aggregation is associated with cellular dysfunction. However, surprisingly, this aggregation and resultant toxicity was not influenced by aging. This is in contrast to polyglutamine peptides alone whose aggregation/toxicity is highly dependent on age. Thus, the data presented here not only describe a new polyglutamine model, but also suggest that protein context likely influences the cellular interactions of the polyglutamine-containing protein and thereby modulates its toxic properties.

  2. Novel polyglutamine model uncouples proteotoxicity from aging.

    Directory of Open Access Journals (Sweden)

    Nakeirah T M Christie

    Full Text Available Polyglutamine expansions in certain proteins are the genetic determinants for nine distinct progressive neurodegenerative disorders and resultant age-related dementia. In these cases, neurodegeneration is due to the aggregation propensity and resultant toxic properties of the polyglutamine-containing proteins. We are interested in elucidating the underlying mechanisms of toxicity of the protein ataxin-3, in which a polyglutamine expansion is the genetic determinant for Machado-Joseph Disease (MJD, also referred to as spinocerebellar ataxia 3 (SCA3. To this end, we have developed a novel model for ataxin-3 protein aggregation, by expressing a disease-related polyglutamine-containing fragment of ataxin-3 in the genetically tractable body wall muscle cells of the model system C. elegans. Here, we demonstrate that this ataxin-3 fragment aggregates in a polyQ length-dependent manner in C. elegans muscle cells and that this aggregation is associated with cellular dysfunction. However, surprisingly, this aggregation and resultant toxicity was not influenced by aging. This is in contrast to polyglutamine peptides alone whose aggregation/toxicity is highly dependent on age. Thus, the data presented here not only describe a new polyglutamine model, but also suggest that protein context likely influences the cellular interactions of the polyglutamine-containing protein and thereby modulates its toxic properties.

  3. Identification of the Drosophila Ortholog of HSPB8 IMPLICATION OF HSPB8 LOSS OF FUNCTION IN PROTEIN FOLDING DISEASES

    NARCIS (Netherlands)

    Carra, Serena; Boncoraglio, Alessandra; Kanon, Bart; Brunsting, Jeanette F.; Minoia, Melania; Rana, Anil; Vos, Michel J.; Seidel, Kay; Sibon, Ody C. M.; Kampinga, Harm H.

    2010-01-01

    Protein aggregation is a hallmark of many neuronal disorders, including the polyglutamine disorder spinocerebellar ataxia 3 and peripheral neuropathies associated with the K141E and K141N mutations in the small heat shock protein HSPB8. In cells, HSPB8 cooperates with BAG3 to stimulate autophagy in

  4. Accurate SHAPE-directed RNA secondary structure modeling, including pseudoknots.

    Science.gov (United States)

    Hajdin, Christine E; Bellaousov, Stanislav; Huggins, Wayne; Leonard, Christopher W; Mathews, David H; Weeks, Kevin M

    2013-04-02

    A pseudoknot forms in an RNA when nucleotides in a loop pair with a region outside the helices that close the loop. Pseudoknots occur relatively rarely in RNA but are highly overrepresented in functionally critical motifs in large catalytic RNAs, in riboswitches, and in regulatory elements of viruses. Pseudoknots are usually excluded from RNA structure prediction algorithms. When included, these pairings are difficult to model accurately, especially in large RNAs, because allowing this structure dramatically increases the number of possible incorrect folds and because it is difficult to search the fold space for an optimal structure. We have developed a concise secondary structure modeling approach that combines SHAPE (selective 2'-hydroxyl acylation analyzed by primer extension) experimental chemical probing information and a simple, but robust, energy model for the entropic cost of single pseudoknot formation. Structures are predicted with iterative refinement, using a dynamic programming algorithm. This melded experimental and thermodynamic energy function predicted the secondary structures and the pseudoknots for a set of 21 challenging RNAs of known structure ranging in size from 34 to 530 nt. On average, 93% of known base pairs were predicted, and all pseudoknots in well-folded RNAs were identified.

  5. Progress Towards an LES Wall Model Including Unresolved Roughness

    Science.gov (United States)

    Craft, Kyle; Redman, Andrew; Aikens, Kurt

    2015-11-01

    Wall models used in large eddy simulations (LES) are often based on theories for hydraulically smooth walls. While this is reasonable for many applications, there are also many where the impact of surface roughness is important. A previously developed wall model has been used primarily for jet engine aeroacoustics. However, jet simulations have not accurately captured thick initial shear layers found in some experimental data. This may partly be due to nozzle wall roughness used in the experiments to promote turbulent boundary layers. As a result, the wall model is extended to include the effects of unresolved wall roughness through appropriate alterations to the log-law. The methodology is tested for incompressible flat plate boundary layers with different surface roughness. Correct trends are noted for the impact of surface roughness on the velocity profile. However, velocity deficit profiles and the Reynolds stresses do not collapse as well as expected. Possible reasons for the discrepancies as well as future work will be presented. This work used the Extreme Science and Engineering Discovery Environment (XSEDE), which is supported by National Science Foundation grant number ACI-1053575. Computational resources on TACC Stampede were provided under XSEDE allocation ENG150001.

  6. Kinetic models of gene expression including non-coding RNAs

    Energy Technology Data Exchange (ETDEWEB)

    Zhdanov, Vladimir P., E-mail: zhdanov@catalysis.r

    2011-03-15

    In cells, genes are transcribed into mRNAs, and the latter are translated into proteins. Due to the feedbacks between these processes, the kinetics of gene expression may be complex even in the simplest genetic networks. The corresponding models have already been reviewed in the literature. A new avenue in this field is related to the recognition that the conventional scenario of gene expression is fully applicable only to prokaryotes whose genomes consist of tightly packed protein-coding sequences. In eukaryotic cells, in contrast, such sequences are relatively rare, and the rest of the genome includes numerous transcript units representing non-coding RNAs (ncRNAs). During the past decade, it has become clear that such RNAs play a crucial role in gene expression and accordingly influence a multitude of cellular processes both in the normal state and during diseases. The numerous biological functions of ncRNAs are based primarily on their abilities to silence genes via pairing with a target mRNA and subsequently preventing its translation or facilitating degradation of the mRNA-ncRNA complex. Many other abilities of ncRNAs have been discovered as well. Our review is focused on the available kinetic models describing the mRNA, ncRNA and protein interplay. In particular, we systematically present the simplest models without kinetic feedbacks, models containing feedbacks and predicting bistability and oscillations in simple genetic networks, and models describing the effect of ncRNAs on complex genetic networks. Mathematically, the presentation is based primarily on temporal mean-field kinetic equations. The stochastic and spatio-temporal effects are also briefly discussed.

  7. Mathematical Model of Thyristor Inverter Including a Series-parallel Resonant Circuit

    Directory of Open Access Journals (Sweden)

    Miroslaw Luft

    2008-01-01

    Full Text Available The article presents a mathematical model of thyristor inverter including a series-parallel resonant circuit with theaid of state variable method. Maple procedures are used to compute current and voltage waveforms in the inverter.

  8. Including sugar cane in the agro-ecosystem model ORCHIDEE-STICS

    Science.gov (United States)

    Valade, A.; Vuichard, N.; Ciais, P.; Viovy, N.

    2010-12-01

    With 4 million ha currently grown for ethanol in Brazil only, approximately half the global bioethanol production in 2005 (Smeets 2008), and a devoted land area expected to expand globally in the years to come, sugar cane is at the heart of the biofuel debate. Indeed, ethanol made from biomass is currently the most widespread option for alternative transportation fuels. It was originally promoted as a carbon neutral energy resource that could bring energy independence to countries and local opportunities to farmers, until attention was drawn to its environmental and socio-economical drawbacks. It is still not clear to which extent it is a solution or a contributor to climate change mitigation. Dynamic Global Vegetation models can help address these issues and quantify the potential impacts of biofuels on ecosystems at scales ranging from on-site to global. The global agro-ecosystem model ORCHIDEE describes water, carbon and energy exchanges at the soil-atmosphere interface for a limited number of natural and agricultural vegetation types. In order to integrate agricultural management to the simulations and to capture more accurately the specificity of crops' phenology, ORCHIDEE has been coupled with the agronomical model STICS. The resulting crop-oriented vegetation model ORCHIDEE-STICS has been used so far to simulate temperate crops such as wheat, corn and soybean. As a generic ecosystem model, each grid cell can include several vegetation types with their own phenology and management practices, making it suitable to spatial simulations. Here, ORCHIDEE-STICS is altered to include sugar cane as a new agricultural Plant functional Type, implemented and parametrized using the STICS approach. An on-site calibration and validation is then performed based on biomass and flux chamber measurements in several sites in Australia and variables such as LAI, dry weight, heat fluxes and respiration are used to evaluate the ability of the model to simulate the specific

  9. Clarifying the use of aggregated exposures in multilevel models: self-included vs. self-excluded measures.

    Directory of Open Access Journals (Sweden)

    Etsuji Suzuki

    Full Text Available Multilevel analyses are ideally suited to assess the effects of ecological (higher level and individual (lower level exposure variables simultaneously. In applying such analyses to measures of ecologies in epidemiological studies, individual variables are usually aggregated into the higher level unit. Typically, the aggregated measure includes responses of every individual belonging to that group (i.e. it constitutes a self-included measure. More recently, researchers have developed an aggregate measure which excludes the response of the individual to whom the aggregate measure is linked (i.e. a self-excluded measure. In this study, we clarify the substantive and technical properties of these two measures when they are used as exposures in multilevel models.Although the differences between the two aggregated measures are mathematically subtle, distinguishing between them is important in terms of the specific scientific questions to be addressed. We then show how these measures can be used in two distinct types of multilevel models-self-included model and self-excluded model-and interpret the parameters in each model by imposing hypothetical interventions. The concept is tested on empirical data of workplace social capital and employees' systolic blood pressure.Researchers assume group-level interventions when using a self-included model, and individual-level interventions when using a self-excluded model. Analytical re-parameterizations of these two models highlight their differences in parameter interpretation. Cluster-mean centered self-included models enable researchers to decompose the collective effect into its within- and between-group components. The benefit of cluster-mean centering procedure is further discussed in terms of hypothetical interventions.When investigating the potential roles of aggregated variables, researchers should carefully explore which type of model-self-included or self-excluded-is suitable for a given situation

  10. Promoter Variation and Expression Levels of Inflammatory Genes IL1A, IL1B, IL6 and TNF in Blood of Spinocerebellar Ataxia Type 3 (SCA3) Patients.

    Science.gov (United States)

    Raposo, Mafalda; Bettencourt, Conceição; Ramos, Amanda; Kazachkova, Nadiya; Vasconcelos, João; Kay, Teresa; Bruges-Armas, Jácome; Lima, Manuela

    2017-03-01

    Age at onset in spinocerebellar ataxia type 3 (SCA3/MJD) is incompletely explained by the size of the CAG tract at the ATXN3 gene, implying the existence of genetic modifiers. A role of inflammation in SCA3 has been postulated, involving altered cytokines levels; promoter variants leading to alterations in cytokines expression could influence onset. Using blood from 86 SCA3 patients and 106 controls, this work aimed to analyse promoter variation of four cytokines (IL1A, IL1B, IL6 and TNF) and to investigate the association between variants detected and their transcript levels, evaluated by quantitative PCR. Moreover, the effect of APOE isoforms, known to modulate cytokines, was investigated. Correlations between cytokine variants and onset were tested; the cumulative modifier effects of cytokines and APOE were analysed. Patients carrying the IL6*C allele had a significant earlier onset (4 years in average) than patients carrying the G allele, in agreement with lower mRNA levels produced by IL6*C carriers. The presence of APOE*ɛ2 allele seems to anticipate onset in average 10 years in patients carrying the IL6*C allele; a larger number of patients will be needed to confirm this result. These results highlight the pertinence of conducting further research on the role of cytokines as SCA3 modulators, pointing to the presence of shared mechanisms involving IL6 and APOE.

  11. Simple suggestions for including vertical physics in oil spill models

    International Nuclear Information System (INIS)

    D'Asaro, Eric; University of Washington, Seatle, WA

    2001-01-01

    Current models of oil spills include no vertical physics. They neglect the effect of vertical water motions on the transport and concentration of floating oil. Some simple ways to introduce vertical physics are suggested here. The major suggestion is to routinely measure the density stratification of the upper ocean during oil spills in order to develop a database on the effect of stratification. (Author)

  12. Hydromechanical modeling of clay rock including fracture damage

    Science.gov (United States)

    Asahina, D.; Houseworth, J. E.; Birkholzer, J. T.

    2012-12-01

    Argillaceous rock typically acts as a flow barrier, but under certain conditions significant and potentially conductive fractures may be present. Fracture formation is well-known to occur in the vicinity of underground excavations in a region known as the excavation disturbed zone. Such problems are of particular importance for low-permeability, mechanically weak rock such as clays and shales because fractures can be relatively transient as a result of fracture self-sealing processes. Perhaps not as well appreciated is the fact that natural fractures can form in argillaceous rock as a result of hydraulic overpressure caused by phenomena such as disequlibrium compaction, changes in tectonic stress, and mineral dehydration. Overpressure conditions can cause hydraulic fracturing if the fluid pressure leads to tensile effective stresses that exceed the tensile strength of the material. Quantitative modeling of this type of process requires coupling between hydrogeologic processes and geomechanical processes including fracture initiation and propagation. Here we present a computational method for three-dimensional, hydromechanical coupled processes including fracture damage. Fractures are represented as discrete features in a fracture network that interact with a porous rock matrix. Fracture configurations are mapped onto an unstructured, three-dimensonal, Voronoi grid, which is based on a random set of spatial points. Discrete fracture networks (DFN) are represented by the connections of the edges of a Voronoi cells. This methodology has the advantage that fractures can be more easily introduced in response to coupled hydro-mechanical processes and generally eliminates several potential issues associated with the geometry of DFN and numerical gridding. A geomechanical and fracture-damage model is developed here using the Rigid-Body-Spring-Network (RBSN) numerical method. The hydrogelogic and geomechanical models share the same geometrical information from a 3D Voronoi

  13. Direct-phase-variable model of a synchronous reluctance motor including all slot and winding harmonics

    International Nuclear Information System (INIS)

    Obe, Emeka S.; Binder, A.

    2011-01-01

    A detailed model in direct-phase variables of a synchronous reluctance motor operating at mains voltage and frequency is presented. The model includes the stator and rotor slot openings, the actual winding layout and the reluctance rotor geometry. Hence, all mmf and permeance harmonics are taken into account. It is seen that non-negligible harmonics introduced by slots are present in the inductances computed by the winding function procedure. These harmonics are usually ignored in d-q models. The machine performance is simulated in the stator reference frame to depict the difference between this new direct-phase model including all harmonics and the conventional rotor reference frame d-q model. Saturation is included by using a polynomial fitting the variation of d-axis inductance with stator current obtained by finite-element software FEMAG DC (registered) . The detailed phase-variable model can yield torque pulsations comparable to those obtained from finite elements while the d-q model cannot.

  14. Solving the puzzle of neurological diseases: an interview with Huda Zoghbi

    Directory of Open Access Journals (Sweden)

    Huda Y. Zoghbi

    2017-05-01

    Full Text Available Huda Zoghbi's achievements in the field of neurology are internationally acclaimed. She is best known for elucidating the genetic basis of two complex neurological disorders, spinocerebellar ataxia type 1 and Rett syndrome, and has been honored with many prizes, including The Shaw Prize in Life Science and Medicine in 2016 and the 2017 Breakthrough Prize for Life Sciences. A diligent and creative research scientist at the bench, a respected lab mentor and founding Director of the Jan and Dan Duncan Neurological Research Institute at Texas Children's Hospital, her inspiration has always been the burning need to help patients faced with devastating neurological problems. Her pursuit of the mechanisms mediating spinocerebellar ataxia and Rett syndrome has been dogged, requiring 30 years of focused effort. As highlighted in this interview, her work is now paying dividends by starting to reveal potential therapeutic targets for these intractable and complex disorders.

  15. Integrated model of port oil piping transportation system safety including operating environment threats

    Directory of Open Access Journals (Sweden)

    Kołowrocki Krzysztof

    2017-06-01

    Full Text Available The paper presents an integrated general model of complex technical system, linking its multistate safety model and the model of its operation process including operating environment threats and considering variable at different operation states its safety structures and its components safety parameters. Under the assumption that the system has exponential safety function, the safety characteristics of the port oil piping transportation system are determined.

  16. Integrated model of port oil piping transportation system safety including operating environment threats

    OpenAIRE

    Kołowrocki, Krzysztof; Kuligowska, Ewa; Soszyńska-Budny, Joanna

    2017-01-01

    The paper presents an integrated general model of complex technical system, linking its multistate safety model and the model of its operation process including operating environment threats and considering variable at different operation states its safety structures and its components safety parameters. Under the assumption that the system has exponential safety function, the safety characteristics of the port oil piping transportation system are determined.

  17. Reduced cardiac {sup 123}I-metaiodobenzylguanidine uptake in patients with spinocerebellar ataxia type 2: a comparative study with Parkinson's disease

    Energy Technology Data Exchange (ETDEWEB)

    De Rosa, Anna; De Leva, Maria Fulvia; Maddaluno, Gennaro; Filla, Alessandro; De Michele, Giuseppe [University Federico II, Department of Neurosciences and Reproductive and Odontostomatologic Sciences, Naples (Italy); Pappata, Sabina; Pellegrino, Teresa [National Council of Research, Institute of Biostructure and Bioimaging, Naples (Italy); Fiumara, Giovanni [Institute of Diagnostic and Nuclear Development, SDN Foundation, Naples (Italy); Carotenuto, Raffaella; Cuocolo, Alberto [University Federico II, Department of Advanced Biomedical Sciences, Naples (Italy); Petretta, Mario [University Federico II, Department of Translational Medical Sciences, Naples (Italy)

    2013-12-15

    Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disorder characterized by cerebellar ataxia, supranuclear ophthalmoplegia, and peripheral neuropathy. Autonomic nervous system dysfunction is often present. This study evaluated the cardiac sympathetic function in patients with SCA2 using {sup 123}I-metaiodobenzylguanidine (MIBG) in comparison with patients with Parkinson's disease (PD) and control subjects. Nine patients with SCA2, nine patients with PD, and nine control subjects underwent {sup 123}I-MIBG imaging studies from which early and late heart-to-mediastinum (H/M) ratios and myocardial washout rates were calculated. Early (F = 12.3, p < 0.0001) and late (F = 16.8, p < 0.0001) H/M ratios were significantly different among groups. In controls, early and late H/M ratios (2.2 {+-} 0.12 and 2.1 {+-} 0.20) were significantly higher than in patients with SCA2 (1.9 {+-} 0.23 and 1.8 {+-} 0.20, both p < 0.05) and with patients with PD (1.7 {+-} 0.29 and 1.4 {+-} 0.35, both p < 0.001). There was also a significant difference in washout rates among groups (F = 11.7, p < 0.0001). In controls the washout rate (19.9 {+-} 9.6 %) was significantly lower (p < 0.005) than in patients with PD (51.0 {+-} 23.7 %), but not different from that in SCA2 patients (19.5 {+-} 9.4 %). In SCA2 patients, in a multivariable linear regression analysis only the Scale for the Assessment and Rating of Ataxia score was independently associated with early H/M ratio ({beta} = -0.12, p < 0.05). {sup 123}I-MIBG myocardial scintigraphy demonstrated an impairment of cardiac sympathetic function in patients with SCA2, which was less marked than in PD patients. These results suggest that {sup 123}I-MIBG cardiac imaging could become a useful tool for analysing the pathophysiology of SCA2. (orig.)

  18. Enhanced UWB Radio Channel Model for Short-Range Communication Scenarios Including User Dynamics

    DEFF Research Database (Denmark)

    Kovacs, Istvan Zsolt; Nguyen, Tuan Hung; Eggers, Patrick Claus F.

    2005-01-01

    channel model represents an enhancement of the existing IEEE 802.15.3a/4a PAN channel model, where antenna and user-proximity effects are not included. Our investigations showed that significant variations of the received wideband power and time-delay signal clustering are possible due the human body...

  19. Aggregated Demand Modelling Including Distributed Generation, Storage and Demand Response

    OpenAIRE

    Marzooghi, Hesamoddin; Hill, David J.; Verbic, Gregor

    2014-01-01

    It is anticipated that penetration of renewable energy sources (RESs) in power systems will increase further in the next decades mainly due to environmental issues. In the long term of several decades, which we refer to in terms of the future grid (FG), balancing between supply and demand will become dependent on demand actions including demand response (DR) and energy storage. So far, FG feasibility studies have not considered these new demand-side developments for modelling future demand. I...

  20. S5-4: Formal Modeling of Affordance in Human-Included Systems

    Directory of Open Access Journals (Sweden)

    Namhun Kim

    2012-10-01

    Full Text Available In spite of it being necessary for humans to consider modeling, analysis, and control of human-included systems, it has been considered a challenging problem because of the critical role of humans in complex systems and of humans' capability of executing unanticipated actions–both beneficial and detrimental ones. Thus, to provide systematic approaches to modeling human actions as a part of system behaviors, a formal modeling framework for human-involved systems in which humans play a controlling role based on their perceptual information is presented. The theory of affordance provides definitions of human actions and their associated properties; Finite State Automata (FSA based modeling is capable of mapping nondeterministic humans into computable components in the system representation. In this talk, we investigate the role of perception in human actions in the system operation and examine the representation of perceptual elements in affordance-based modeling formalism. The proposed framework is expected to capture the natural ways in which humans participate in the system as part of its operation. A human-machine cooperative manufacturing system control example and a human agent simulation example will be introduced for the illustrative purposes at the end of the presentation.

  1. Modeling of the Direct Current Generator Including the Magnetic Saturation and Temperature Effects

    Directory of Open Access Journals (Sweden)

    Alfonso J. Mercado-Samur

    2013-11-01

    Full Text Available In this paper the inclusion of temperature effect on the field resistance on the direct current generator model DC1A, which is valid to stability studies is proposed. First, the linear generator model is presented, after the effect of magnetic saturation and the change in the resistance value due to temperature produced by the field current are included. The comparison of experimental results and model simulations to validate the model is used. A direct current generator model which is a better representation of the generator is obtained. Visual comparison between simulations and experimental results shows the success of the proposed model, because it presents the lowest error of the compared models. The accuracy of the proposed model is observed via Modified Normalized Sum of Squared Errors index equal to 3.8979%.

  2. BioModels: expanding horizons to include more modelling approaches and formats.

    Science.gov (United States)

    Glont, Mihai; Nguyen, Tung V N; Graesslin, Martin; Hälke, Robert; Ali, Raza; Schramm, Jochen; Wimalaratne, Sarala M; Kothamachu, Varun B; Rodriguez, Nicolas; Swat, Maciej J; Eils, Jurgen; Eils, Roland; Laibe, Camille; Malik-Sheriff, Rahuman S; Chelliah, Vijayalakshmi; Le Novère, Nicolas; Hermjakob, Henning

    2018-01-04

    BioModels serves as a central repository of mathematical models representing biological processes. It offers a platform to make mathematical models easily shareable across the systems modelling community, thereby supporting model reuse. To facilitate hosting a broader range of model formats derived from diverse modelling approaches and tools, a new infrastructure for BioModels has been developed that is available at http://www.ebi.ac.uk/biomodels. This new system allows submitting and sharing of a wide range of models with improved support for formats other than SBML. It also offers a version-control backed environment in which authors and curators can work collaboratively to curate models. This article summarises the features available in the current system and discusses the potential benefit they offer to the users over the previous system. In summary, the new portal broadens the scope of models accepted in BioModels and supports collaborative model curation which is crucial for model reproducibility and sharing. © The Author(s) 2017. Published by Oxford University Press on behalf of Nucleic Acids Research.

  3. A thermal conductivity model for nanofluids including effect of the temperature-dependent interfacial layer

    International Nuclear Information System (INIS)

    Sitprasert, Chatcharin; Dechaumphai, Pramote; Juntasaro, Varangrat

    2009-01-01

    The interfacial layer of nanoparticles has been recently shown to have an effect on the thermal conductivity of nanofluids. There is, however, still no thermal conductivity model that includes the effects of temperature and nanoparticle size variations on the thickness and consequently on the thermal conductivity of the interfacial layer. In the present work, the stationary model developed by Leong et al. (J Nanopart Res 8:245-254, 2006) is initially modified to include the thermal dispersion effect due to the Brownian motion of nanoparticles. This model is called the 'Leong et al.'s dynamic model'. However, the Leong et al.'s dynamic model over-predicts the thermal conductivity of nanofluids in the case of the flowing fluid. This suggests that the enhancement in the thermal conductivity of the flowing nanofluids due to the increase in temperature does not come from the thermal dispersion effect. It is more likely that the enhancement in heat transfer of the flowing nanofluids comes from the temperature-dependent interfacial layer effect. Therefore, the Leong et al.'s stationary model is again modified to include the effect of temperature variation on the thermal conductivity of the interfacial layer for different sizes of nanoparticles. This present model is then evaluated and compared with the other thermal conductivity models for the turbulent convective heat transfer in nanofluids along a uniformly heated tube. The results show that the present model is more general than the other models in the sense that it can predict both the temperature and the volume fraction dependence of the thermal conductivity of nanofluids for both non-flowing and flowing fluids. Also, it is found to be more accurate than the other models due to the inclusion of the effect of the temperature-dependent interfacial layer. In conclusion, the present model can accurately predict the changes in thermal conductivity of nanofluids due to the changes in volume fraction and temperature for

  4. Correlation of anatomy and function in medulla oblongata infarction.

    Science.gov (United States)

    Eggers, C; Fink, G R; Möller-Hartmann, W; Nowak, D A

    2009-02-01

    A presentation of all aspects of the dorsolateral medulla oblongata syndrome is clinically very rare to find. In most cases patients present with fragmentary symptoms, e.g. ipsilateral axial lateropulsion, nystagmus, dysarthria, dysphagia or hemiataxia. However, the clinical presentation and lesion anatomy at the level of the medulla oblongata is still unsatisfactory. The aim of this study was to correlate the functional deficit with structural MRI-data. We included thirteen patients (eight male, five female, mean age 65.5) with medulla oblongata infarction with clinically predominant ipsilateral axial lateropulsion and correlated clinical with structural deficits. Magnetic resonance imaging lesion mapping demonstrated ipsilateral axial lateropulsion to result from lesions of the spinocerebellar tract, the inferior cerebellar peduncle or the inferior vestibular nucleus. Nystagmus was associated with lesions of the inferior vestibular nucleus, dissociated sensory loss with the spinothalamic tract and hemiataxia with the spinocerebellar tract. Correlating dysfunction and lesion anatomy is a promising approach to enhance our knowledge on medulla oblongata topography.

  5. Modeling of Temperature-Dependent Noise in Silicon Nanowire FETs including Self-Heating Effects

    Directory of Open Access Journals (Sweden)

    P. Anandan

    2014-01-01

    Full Text Available Silicon nanowires are leading the CMOS era towards the downsizing limit and its nature will be effectively suppress the short channel effects. Accurate modeling of thermal noise in nanowires is crucial for RF applications of nano-CMOS emerging technologies. In this work, a perfect temperature-dependent model for silicon nanowires including the self-heating effects has been derived and its effects on device parameters have been observed. The power spectral density as a function of thermal resistance shows significant improvement as the channel length decreases. The effects of thermal noise including self-heating of the device are explored. Moreover, significant reduction in noise with respect to channel thermal resistance, gate length, and biasing is analyzed.

  6. Seepage Model for PA Including Drift Collapse

    International Nuclear Information System (INIS)

    Li, G.; Tsang, C.

    2000-01-01

    The purpose of this Analysis/Model Report (AMR) is to document the predictions and analysis performed using the Seepage Model for Performance Assessment (PA) and the Disturbed Drift Seepage Submodel for both the Topopah Spring middle nonlithophysal and lower lithophysal lithostratigraphic units at Yucca Mountain. These results will be used by PA to develop the probability distribution of water seepage into waste-emplacement drifts at Yucca Mountain, Nevada, as part of the evaluation of the long term performance of the potential repository. This AMR is in accordance with the ''Technical Work Plan for Unsaturated Zone (UZ) Flow and Transport Process Model Report'' (CRWMS M andO 2000 [153447]). This purpose is accomplished by performing numerical simulations with stochastic representations of hydrological properties, using the Seepage Model for PA, and evaluating the effects of an alternative drift geometry representing a partially collapsed drift using the Disturbed Drift Seepage Submodel. Seepage of water into waste-emplacement drifts is considered one of the principal factors having the greatest impact of long-term safety of the repository system (CRWMS M andO 2000 [153225], Table 4-1). This AMR supports the analysis and simulation that are used by PA to develop the probability distribution of water seepage into drift, and is therefore a model of primary (Level 1) importance (AP-3.15Q, ''Managing Technical Product Inputs''). The intended purpose of the Seepage Model for PA is to support: (1) PA; (2) Abstraction of Drift-Scale Seepage; and (3) Unsaturated Zone (UZ) Flow and Transport Process Model Report (PMR). Seepage into drifts is evaluated by applying numerical models with stochastic representations of hydrological properties and performing flow simulations with multiple realizations of the permeability field around the drift. The Seepage Model for PA uses the distribution of permeabilities derived from air injection testing in niches and in the cross drift to

  7. Seepage Model for PA Including Dift Collapse

    Energy Technology Data Exchange (ETDEWEB)

    G. Li; C. Tsang

    2000-12-20

    The purpose of this Analysis/Model Report (AMR) is to document the predictions and analysis performed using the Seepage Model for Performance Assessment (PA) and the Disturbed Drift Seepage Submodel for both the Topopah Spring middle nonlithophysal and lower lithophysal lithostratigraphic units at Yucca Mountain. These results will be used by PA to develop the probability distribution of water seepage into waste-emplacement drifts at Yucca Mountain, Nevada, as part of the evaluation of the long term performance of the potential repository. This AMR is in accordance with the ''Technical Work Plan for Unsaturated Zone (UZ) Flow and Transport Process Model Report'' (CRWMS M&O 2000 [153447]). This purpose is accomplished by performing numerical simulations with stochastic representations of hydrological properties, using the Seepage Model for PA, and evaluating the effects of an alternative drift geometry representing a partially collapsed drift using the Disturbed Drift Seepage Submodel. Seepage of water into waste-emplacement drifts is considered one of the principal factors having the greatest impact of long-term safety of the repository system (CRWMS M&O 2000 [153225], Table 4-1). This AMR supports the analysis and simulation that are used by PA to develop the probability distribution of water seepage into drift, and is therefore a model of primary (Level 1) importance (AP-3.15Q, ''Managing Technical Product Inputs''). The intended purpose of the Seepage Model for PA is to support: (1) PA; (2) Abstraction of Drift-Scale Seepage; and (3) Unsaturated Zone (UZ) Flow and Transport Process Model Report (PMR). Seepage into drifts is evaluated by applying numerical models with stochastic representations of hydrological properties and performing flow simulations with multiple realizations of the permeability field around the drift. The Seepage Model for PA uses the distribution of permeabilities derived from air injection testing in

  8. Particle-based modeling of heterogeneous chemical kinetics including mass transfer.

    Science.gov (United States)

    Sengar, A; Kuipers, J A M; van Santen, Rutger A; Padding, J T

    2017-08-01

    Connecting the macroscopic world of continuous fields to the microscopic world of discrete molecular events is important for understanding several phenomena occurring at physical boundaries of systems. An important example is heterogeneous catalysis, where reactions take place at active surfaces, but the effective reaction rates are determined by transport limitations in the bulk fluid and reaction limitations on the catalyst surface. In this work we study the macro-micro connection in a model heterogeneous catalytic reactor by means of stochastic rotation dynamics. The model is able to resolve the convective and diffusive interplay between participating species, while including adsorption, desorption, and reaction processes on the catalytic surface. Here we apply the simulation methodology to a simple straight microchannel with a catalytic strip. Dimensionless Damkohler numbers are used to comment on the spatial concentration profiles of reactants and products near the catalyst strip and in the bulk. We end the discussion with an outlook on more complicated geometries and increasingly complex reactions.

  9. Particle-based modeling of heterogeneous chemical kinetics including mass transfer

    Science.gov (United States)

    Sengar, A.; Kuipers, J. A. M.; van Santen, Rutger A.; Padding, J. T.

    2017-08-01

    Connecting the macroscopic world of continuous fields to the microscopic world of discrete molecular events is important for understanding several phenomena occurring at physical boundaries of systems. An important example is heterogeneous catalysis, where reactions take place at active surfaces, but the effective reaction rates are determined by transport limitations in the bulk fluid and reaction limitations on the catalyst surface. In this work we study the macro-micro connection in a model heterogeneous catalytic reactor by means of stochastic rotation dynamics. The model is able to resolve the convective and diffusive interplay between participating species, while including adsorption, desorption, and reaction processes on the catalytic surface. Here we apply the simulation methodology to a simple straight microchannel with a catalytic strip. Dimensionless Damkohler numbers are used to comment on the spatial concentration profiles of reactants and products near the catalyst strip and in the bulk. We end the discussion with an outlook on more complicated geometries and increasingly complex reactions.

  10. Increased sexual arousal in patients with movement disorders.

    Science.gov (United States)

    Teive, Hélio A G; Moro, Adriana; Moscovich, Mariana; Munhoz, Renato P

    2016-04-01

    Increased of sexual arousal (ISA) has been described in different neurological diseases. The purpose of this study was present a case series of ISA in patients with movement disorders. Fifteen patients with different forms of movement disorders (Parkinson's disease, Huntington's disease, Tourette's syndrome, spinocerebellar ataxia type 3), were evaluated in the Movement Disorders Unit of the Federal University of Paraná. Among Parkinson's disease patients there were seven cases with different forms of ISA due to dopaminergic agonist use, levodopa abuse, and deep brain stimulation (DBS). In the group with hyperkinetic disorders, two patients with Huntington's disease, two with Tourette's syndrome, and four with spinocerebellar ataxia type 3 presented with ISA. ISA in this group of patients had different etiologies, predominantly related to dopaminergic treatment or DBS in Parkinson's disease, part of the background clinical picture in Huntington's disease and Tourette's syndrome, and probably associated with cultural aspects in patients with spinocerebellar ataxia type 3.

  11. Observational constraint on the interacting dark energy models including the Sandage-Loeb test

    Science.gov (United States)

    Zhang, Ming-Jian; Liu, Wen-Biao

    2014-05-01

    Two types of interacting dark energy models are investigated using the type Ia supernova (SNIa), observational data (OHD), cosmic microwave background shift parameter, and the secular Sandage-Loeb (SL) test. In the investigation, we have used two sets of parameter priors including WMAP-9 and Planck 2013. They have shown some interesting differences. We find that the inclusion of SL test can obviously provide a more stringent constraint on the parameters in both models. For the constant coupling model, the interaction term has been improved to be only a half of the original scale on corresponding errors. Comparing with only SNIa and OHD, we find that the inclusion of the SL test almost reduces the best-fit interaction to zero, which indicates that the higher-redshift observation including the SL test is necessary to track the evolution of the interaction. For the varying coupling model, data with the inclusion of the SL test show that the parameter at C.L. in Planck priors is , where the constant is characteristic for the severity of the coincidence problem. This indicates that the coincidence problem will be less severe. We then reconstruct the interaction , and we find that the best-fit interaction is also negative, similar to the constant coupling model. However, for a high redshift, the interaction generally vanishes at infinity. We also find that the phantom-like dark energy with is favored over the CDM model.

  12. MEMLS3&a: Microwave Emission Model of Layered Snowpacks adapted to include backscattering

    Directory of Open Access Journals (Sweden)

    M. Proksch

    2015-08-01

    Full Text Available The Microwave Emission Model of Layered Snowpacks (MEMLS was originally developed for microwave emissions of snowpacks in the frequency range 5–100 GHz. It is based on six-flux theory to describe radiative transfer in snow including absorption, multiple volume scattering, radiation trapping due to internal reflection and a combination of coherent and incoherent superposition of reflections between horizontal layer interfaces. Here we introduce MEMLS3&a, an extension of MEMLS, which includes a backscatter model for active microwave remote sensing of snow. The reflectivity is decomposed into diffuse and specular components. Slight undulations of the snow surface are taken into account. The treatment of like- and cross-polarization is accomplished by an empirical splitting parameter q. MEMLS3&a (as well as MEMLS is set up in a way that snow input parameters can be derived by objective measurement methods which avoid fitting procedures of the scattering efficiency of snow, required by several other models. For the validation of the model we have used a combination of active and passive measurements from the NoSREx (Nordic Snow Radar Experiment campaign in Sodankylä, Finland. We find a reasonable agreement between the measurements and simulations, subject to uncertainties in hitherto unmeasured input parameters of the backscatter model. The model is written in Matlab and the code is publicly available for download through the following website: http://www.iapmw.unibe.ch/research/projects/snowtools/memls.html.

  13. Dipole model analysis of highest precision HERA data, including very low Q"2's

    International Nuclear Information System (INIS)

    Luszczak, A.; Kowalski, H.

    2016-12-01

    We analyse, within a dipole model, the final, inclusive HERA DIS cross section data in the low χ region, using fully correlated errors. We show, that these highest precision data are very well described within the dipole model framework starting from Q"2 values of 3.5 GeV"2 to the highest values of Q"2=250 GeV"2. To analyze the saturation effects we evaluated the data including also the very low 0.35< Q"2 GeV"2 region. The fits including this region show a preference of the saturation ansatz.

  14. A speech production model including the nasal Cavity

    DEFF Research Database (Denmark)

    Olesen, Morten

    In order to obtain articulatory analysis of speech production the model is improved. the standard model, as used in LPC analysis, to a large extent only models the acoustic properties of speech signal as opposed to articulatory modelling of the speech production. In spite of this the LPC model...... is by far the most widely used model in speech technology....

  15. Including an ocean carbon cycle model into iLOVECLIM (v1.0)

    NARCIS (Netherlands)

    Bouttes, N.; Roche, D.M.V.A.P.; Mariotti, V.; Bopp, L.

    2015-01-01

    The atmospheric carbon dioxide concentration plays a crucial role in the radiative balance and as such has a strong influence on the evolution of climate. Because of the numerous interactions between climate and the carbon cycle, it is necessary to include a model of the carbon cycle within a

  16. A range of complex probabilistic models for RNA secondary structure prediction that includes the nearest-neighbor model and more.

    Science.gov (United States)

    Rivas, Elena; Lang, Raymond; Eddy, Sean R

    2012-02-01

    The standard approach for single-sequence RNA secondary structure prediction uses a nearest-neighbor thermodynamic model with several thousand experimentally determined energy parameters. An attractive alternative is to use statistical approaches with parameters estimated from growing databases of structural RNAs. Good results have been reported for discriminative statistical methods using complex nearest-neighbor models, including CONTRAfold, Simfold, and ContextFold. Little work has been reported on generative probabilistic models (stochastic context-free grammars [SCFGs]) of comparable complexity, although probabilistic models are generally easier to train and to use. To explore a range of probabilistic models of increasing complexity, and to directly compare probabilistic, thermodynamic, and discriminative approaches, we created TORNADO, a computational tool that can parse a wide spectrum of RNA grammar architectures (including the standard nearest-neighbor model and more) using a generalized super-grammar that can be parameterized with probabilities, energies, or arbitrary scores. By using TORNADO, we find that probabilistic nearest-neighbor models perform comparably to (but not significantly better than) discriminative methods. We find that complex statistical models are prone to overfitting RNA structure and that evaluations should use structurally nonhomologous training and test data sets. Overfitting has affected at least one published method (ContextFold). The most important barrier to improving statistical approaches for RNA secondary structure prediction is the lack of diversity of well-curated single-sequence RNA secondary structures in current RNA databases.

  17. Refitting density dependent relativistic model parameters including Center-of-Mass corrections

    International Nuclear Information System (INIS)

    Avancini, Sidney S.; Marinelli, Jose R.; Carlson, Brett Vern

    2011-01-01

    Full text: Relativistic mean field models have become a standard approach for precise nuclear structure calculations. After the seminal work of Serot and Walecka, which introduced a model Lagrangian density where the nucleons interact through the exchange of scalar and vector mesons, several models were obtained through its generalization, including other meson degrees of freedom, non-linear meson interactions, meson-meson interactions, etc. More recently density dependent coupling constants were incorporated into the Walecka-like models, which are then extensively used. In particular, for these models a connection with the density functional theory can be established. Due to the inherent difficulties presented by field theoretical models, only the mean field approximation is used for the solution of these models. In order to calculate finite nuclei properties in the mean field approximation, a reference set has to be fixed and therefore the translational symmetry is violated. It is well known that in such case spurious effects due to the center-of-mass (COM) motion are present, which are more pronounced for light nuclei. In a previous work we have proposed a technique based on the Pierls-Yoccoz projection operator applied to the mean-field relativistic solution, in order to project out spurious COM contributions. In this work we obtain a new fitting for the density dependent parameters of a density dependent hadronic model, taking into account the COM corrections. Our fitting is obtained taking into account the charge radii and binding energies for He 4 , O 16 , Ca 40 , Ca 48 , Ni 56 , Ni 68 , Sn 100 , Sn 132 and Pb 208 . We show that the nuclear observables calculated using our fit are of a quality comparable to others that can be found in the literature, with the advantage that now a translational invariant many-body wave function is at our disposal. (author)

  18. A numerical model including PID control of a multizone crystal growth furnace

    Science.gov (United States)

    Panzarella, Charles H.; Kassemi, Mohammad

    1992-01-01

    This paper presents a 2D axisymmetric combined conduction and radiation model of a multizone crystal growth furnace. The model is based on a programmable multizone furnace (PMZF) designed and built at NASA Lewis Research Center for growing high quality semiconductor crystals. A novel feature of this model is a control algorithm which automatically adjusts the power in any number of independently controlled heaters to establish the desired crystal temperatures in the furnace model. The control algorithm eliminates the need for numerous trial and error runs previously required to obtain the same results. The finite element code, FIDAP, used to develop the furnace model, was modified to directly incorporate the control algorithm. This algorithm, which presently uses PID control, and the associated heat transfer model are briefly discussed. Together, they have been used to predict the heater power distributions for a variety of furnace configurations and desired temperature profiles. Examples are included to demonstrate the effectiveness of the PID controlled model in establishing isothermal, Bridgman, and other complicated temperature profies in the sample. Finally, an example is given to show how the algorithm can be used to change the desired profile with time according to a prescribed temperature-time evolution.

  19. Safe distance car-following model including backward-looking and its stability analysis

    Science.gov (United States)

    Yang, Da; Jin, Peter Jing; Pu, Yun; Ran, Bin

    2013-03-01

    The focus of this paper is the car-following behavior including backward-looking, simply called the bi-directional looking car-following behavior. This study is motivated by the potential changes of the physical properties of traffic flow caused by the fast developing intelligent transportation system (ITS), especially the new connected vehicle technology. Existing studies on this topic focused on general motors (GM) models and optimal velocity (OV) models. The safe distance car-following model, Gipps' model, which is more widely used in practice have not drawn too much attention in the bi-directional looking context. This paper explores the property of the bi-directional looking extension of Gipps' safe distance model. The stability condition of the proposed model is derived using the linear stability theory and is verified using numerical simulations. The impacts of the driver and vehicle characteristics appeared in the proposed model on the traffic flow stability are also investigated. It is found that taking into account the backward-looking effect in car-following has three types of effect on traffic flow: stabilizing, destabilizing and producing non-physical phenomenon. This conclusion is more sophisticated than the study results based on the OV bi-directional looking car-following models. Moreover, the drivers who have the smaller reaction time or the larger additional delay and think the other vehicles have larger maximum decelerations can stabilize traffic flow.

  20. SPheno 3.1: extensions including flavour, CP-phases and models beyond the MSSM

    Science.gov (United States)

    Porod, W.; Staub, F.

    2012-11-01

    We describe recent extensions of the program SPhenoincluding flavour aspects, CP-phases, R-parity violation and low energy observables. In case of flavour mixing all masses of supersymmetric particles are calculated including the complete flavour structure and all possible CP-phases at the 1-loop level. We give details on implemented seesaw models, low energy observables and the corresponding extension of the SUSY Les Houches Accord. Moreover, we comment on the possibilities to include MSSM extensions in SPheno. Catalogue identifier: ADRV_v2_0 Program summary URL:http://cpc.cs.qub.ac.uk/summaries/ADRV_v2_0.html Program obtainable from: CPC Program Library, Queen’s University, Belfast, N. Ireland Licensing provisions: Standard CPC licence, http://cpc.cs.qub.ac.uk/licence/licence.html No. of lines in distributed program, including test data, etc.: 154062 No. of bytes in distributed program, including test data, etc.: 1336037 Distribution format: tar.gz Programming language: Fortran95. Computer: PC running under Linux, should run in every Unix environment. Operating system: Linux, Unix. Classification: 11.6. Catalogue identifier of previous version: ADRV_v1_0 Journal reference of previous version: Comput. Phys. Comm. 153(2003)275 Does the new version supersede the previous version?: Yes Nature of problem: The first issue is the determination of the masses and couplings of supersymmetric particles in various supersymmetric models, the R-parity conserved MSSM with generation mixing and including CP-violating phases, various seesaw extensions of the MSSM and the MSSM with bilinear R-parity breaking. Low energy data on Standard Model fermion masses, gauge couplings and electroweak gauge boson masses serve as constraints. Radiative corrections from supersymmetric particles to these inputs must be calculated. Theoretical constraints on the soft SUSY breaking parameters from a high scale theory are imposed and the parameters at the electroweak scale are obtained from the

  1. Enhanced battery model including temperature effects

    NARCIS (Netherlands)

    Rosca, B.; Wilkins, S.

    2013-01-01

    Within electric and hybrid vehicles, batteries are used to provide/buffer the energy required for driving. However, battery performance varies throughout the temperature range specific to automotive applications, and as such, models that describe this behaviour are required. This paper presents a

  2. Models of epidemics: when contact repetition and clustering should be included

    Directory of Open Access Journals (Sweden)

    Scholz Roland W

    2009-06-01

    Full Text Available Abstract Background The spread of infectious disease is determined by biological factors, e.g. the duration of the infectious period, and social factors, e.g. the arrangement of potentially contagious contacts. Repetitiveness and clustering of contacts are known to be relevant factors influencing the transmission of droplet or contact transmitted diseases. However, we do not yet completely know under what conditions repetitiveness and clustering should be included for realistically modelling disease spread. Methods We compare two different types of individual-based models: One assumes random mixing without repetition of contacts, whereas the other assumes that the same contacts repeat day-by-day. The latter exists in two variants, with and without clustering. We systematically test and compare how the total size of an outbreak differs between these model types depending on the key parameters transmission probability, number of contacts per day, duration of the infectious period, different levels of clustering and varying proportions of repetitive contacts. Results The simulation runs under different parameter constellations provide the following results: The difference between both model types is highest for low numbers of contacts per day and low transmission probabilities. The number of contacts and the transmission probability have a higher influence on this difference than the duration of the infectious period. Even when only minor parts of the daily contacts are repetitive and clustered can there be relevant differences compared to a purely random mixing model. Conclusion We show that random mixing models provide acceptable estimates of the total outbreak size if the number of contacts per day is high or if the per-contact transmission probability is high, as seen in typical childhood diseases such as measles. In the case of very short infectious periods, for instance, as in Norovirus, models assuming repeating contacts will also behave

  3. Dynamic model of a micro-tubular solid oxide fuel cell stack including an integrated cooling system

    Science.gov (United States)

    Hering, Martin; Brouwer, Jacob; Winkler, Wolfgang

    2017-02-01

    A novel dynamic micro-tubular solid oxide fuel cell (MT-SOFC) and stack model including an integrated cooling system is developed using a quasi three-dimensional, spatially resolved, transient thermodynamic, physical and electrochemical model that accounts for the complex geometrical relations between the cells and cooling-tubes. The modeling approach includes a simplified tubular geometry and stack design including an integrated cooling structure, detailed pressure drop and gas property calculations, the electrical and physical constraints of the stack design that determine the current, as well as control strategies for the temperature. Moreover, an advanced heat transfer balance with detailed radiative heat transfer between the cells and the integrated cooling-tubes, convective heat transfer between the gas flows and the surrounding structures and conductive heat transfer between the solid structures inside of the stack, is included. The detailed model can be used as a design basis for the novel MT-SOFC stack assembly including an integrated cooling system, as well as for the development of a dynamic system control strategy. The evaluated best-case design achieves very high electrical efficiency between around 75 and 55% in the entire power density range between 50 and 550 mW /cm2 due to the novel stack design comprising an integrated cooling structure.

  4. Finite element modeling of contaminant transport in soils including the effect of chemical reactions.

    Science.gov (United States)

    Javadi, A A; Al-Najjar, M M

    2007-05-17

    The movement of chemicals through soils to the groundwater is a major cause of degradation of water resources. In many cases, serious human and stock health implications are associated with this form of pollution. Recent studies have shown that the current models and methods are not able to adequately describe the leaching of nutrients through soils, often underestimating the risk of groundwater contamination by surface-applied chemicals, and overestimating the concentration of resident solutes. Furthermore, the effect of chemical reactions on the fate and transport of contaminants is not included in many of the existing numerical models for contaminant transport. In this paper a numerical model is presented for simulation of the flow of water and air and contaminant transport through unsaturated soils with the main focus being on the effects of chemical reactions. The governing equations of miscible contaminant transport including advection, dispersion-diffusion and adsorption effects together with the effect of chemical reactions are presented. The mathematical framework and the numerical implementation of the model are described in detail. The model is validated by application to a number of test cases from the literature and is then applied to the simulation of a physical model test involving transport of contaminants in a block of soil with particular reference to the effects of chemical reactions. Comparison of the results of the numerical model with the experimental results shows that the model is capable of predicting the effects of chemical reactions with very high accuracy. The importance of consideration of the effects of chemical reactions is highlighted.

  5. Including Effects of Water Stress on Dead Organic Matter Decay to a Forest Carbon Model

    Science.gov (United States)

    Kim, H.; Lee, J.; Han, S. H.; Kim, S.; Son, Y.

    2017-12-01

    Decay of dead organic matter is a key process of carbon (C) cycling in forest ecosystems. The change in decay rate depends on temperature sensitivity and moisture conditions. The Forest Biomass and Dead organic matter Carbon (FBDC) model includes a decay sub-model considering temperature sensitivity, yet does not consider moisture conditions as drivers of the decay rate change. This study aimed to improve the FBDC model by including a water stress function to the decay sub-model. Also, soil C sequestration under climate change with the FBDC model including the water stress function was simulated. The water stress functions were determined with data from decomposition study on Quercus variabilis forests and Pinus densiflora forests of Korea, and adjustment parameters of the functions were determined for both species. The water stress functions were based on the ratio of precipitation to potential evapotranspiration. Including the water stress function increased the explained variances of the decay rate by 19% for the Q. variabilis forests and 7% for the P. densiflora forests, respectively. The increase of the explained variances resulted from large difference in temperature range and precipitation range across the decomposition study plots. During the period of experiment, the mean annual temperature range was less than 3°C, while the annual precipitation ranged from 720mm to 1466mm. Application of the water stress functions to the FBDC model constrained increasing trend of temperature sensitivity under climate change, and thus increased the model-estimated soil C sequestration (Mg C ha-1) by 6.6 for the Q. variabilis forests and by 3.1 for the P. densiflora forests, respectively. The addition of water stress functions increased reliability of the decay rate estimation and could contribute to reducing the bias in estimating soil C sequestration under varying moisture condition. Acknowledgement: This study was supported by Korea Forest Service (2017044B10-1719-BB01)

  6. A unitarized meson model including color Coulomb interaction

    International Nuclear Information System (INIS)

    Metzger, Kees.

    1990-01-01

    Ch. 1 gives a general introduction into the problem field of the thesis. It discusses in how far the internal structure of mesons is understood theoretically and which models exist. It discusses from a phenomenological point of view the problem of confinement indicates how quark models of mesons may provide insight in this phenomenon. In ch. 2 the formal theory of scattering in a system with confinement is given. It is shown how a coupled channel (CC) description and the work of other authors fit into this general framework. Explicit examples and arguments are given to support the CC treatment of such a system. In ch. 3 the full coupled-channel model as is employed in this thesis is presented. On the basis of arguments from the former chapters and the observed regularities in the experimental data, the choices underlying the model are supported. In this model confinement is described with a mass-dependent harmonic-oscillator potential and the presence of open (meson-meson) channels plays an essential role. In ch. 4 the unitarized model is applied to light scalar meson resonances. In this regime the contribution of the open channels is considerable. It is demonstrated that the model parameters as used for the description of the pseudo-scalar and vector mesons, unchanged can be used for the description of these mesons. Ch. 5 treats the color-Coulomb interaction. There the effect of the Coulomb interaction is studied in simple models without decay. The results of incorporating the color-Coulomb interaction into the full CC model are given in ch.6. Ch. 7 discusses the results of the previous chapters and the present status of the model. (author). 182 refs.; 16 figs.; 33 tabs

  7. Single-Phase Bundle Flows Including Macroscopic Turbulence Model

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Seung Jun; Yoon, Han Young [KAERI, Daejeon (Korea, Republic of); Yoon, Seok Jong; Cho, Hyoung Kyu [Seoul National University, Seoul (Korea, Republic of)

    2016-05-15

    To deal with various thermal hydraulic phenomena due to rapid change of fluid properties when an accident happens, securing mechanistic approaches as much as possible may reduce the uncertainty arising from improper applications of the experimental models. In this study, the turbulence mixing model, which is well defined in the subchannel analysis code such as VIPRE, COBRA, and MATRA by experiments, is replaced by a macroscopic k-e turbulence model, which represents the aspect of mathematical derivation. The performance of CUPID with macroscopic turbulence model is validated against several bundle experiments: CNEN 4x4 and PNL 7x7 rod bundle tests. In this study, the macroscopic k-e model has been validated for the application to subchannel analysis. It has been implemented in the CUPID code and validated against CNEN 4x4 and PNL 7x7 rod bundle tests. The results showed that the macroscopic k-e turbulence model can estimate the experiments properly.

  8. MicroRNAs in Experimental Models of Movement Disorders

    Directory of Open Access Journals (Sweden)

    Soon-Tae Lee

    2011-10-01

    Full Text Available MicroRNAs (miRNAs are small RNAs comprised of 20–25 nucleotides that regulates gene expression by inducing translational repression or degradation of target mRNA. The importance of miRNAs as a mediator of disease pathogenesis and therapeutic targets is rapidly emerging in neuroscience, as well as oncology, immunology, and cardiovascular diseases. In Parkinson’s disease and related disorders, multiple studies have identified the implications of specific miRNAs and the polymorphisms of miRNA target genes during the disease pathogenesis. With a focus on Parkinson’s disease, spinocerebellar ataxia, hereditary spastic paraplegia, and Huntington’s disease, this review summarizes and interprets the observations, and proposes future research topics in this field.

  9. A High-Rate, Single-Crystal Model including Phase Transformations, Plastic Slip, and Twinning

    Energy Technology Data Exchange (ETDEWEB)

    Addessio, Francis L. [Los Alamos National Lab. (LANL), Los Alamos, NM (United States). Theoretical Division; Bronkhorst, Curt Allan [Los Alamos National Lab. (LANL), Los Alamos, NM (United States). Theoretical Division; Bolme, Cynthia Anne [Los Alamos National Lab. (LANL), Los Alamos, NM (United States). Explosive Science and Shock Physics Division; Brown, Donald William [Los Alamos National Lab. (LANL), Los Alamos, NM (United States). Materials Science and Technology Division; Cerreta, Ellen Kathleen [Los Alamos National Lab. (LANL), Los Alamos, NM (United States). Materials Science and Technology Division; Lebensohn, Ricardo A. [Los Alamos National Lab. (LANL), Los Alamos, NM (United States). Materials Science and Technology Division; Lookman, Turab [Los Alamos National Lab. (LANL), Los Alamos, NM (United States). Theoretical Division; Luscher, Darby Jon [Los Alamos National Lab. (LANL), Los Alamos, NM (United States). Theoretical Division; Mayeur, Jason Rhea [Los Alamos National Lab. (LANL), Los Alamos, NM (United States). Theoretical Division; Morrow, Benjamin M. [Los Alamos National Lab. (LANL), Los Alamos, NM (United States). Materials Science and Technology Division; Rigg, Paulo A. [Washington State Univ., Pullman, WA (United States). Dept. of Physics. Inst. for Shock Physics

    2016-08-09

    An anisotropic, rate-­dependent, single-­crystal approach for modeling materials under the conditions of high strain rates and pressures is provided. The model includes the effects of large deformations, nonlinear elasticity, phase transformations, and plastic slip and twinning. It is envisioned that the model may be used to examine these coupled effects on the local deformation of materials that are subjected to ballistic impact or explosive loading. The model is formulated using a multiplicative decomposition of the deformation gradient. A plate impact experiment on a multi-­crystal sample of titanium was conducted. The particle velocities at the back surface of three crystal orientations relative to the direction of impact were measured. Molecular dynamics simulations were conducted to investigate the details of the high-­rate deformation and pursue issues related to the phase transformation for titanium. Simulations using the single crystal model were conducted and compared to the high-­rate experimental data for the impact loaded single crystals. The model was found to capture the features of the experiments.

  10. Increased sexual arousal in patients with movement disorders

    Directory of Open Access Journals (Sweden)

    Hélio A. G. Teive

    2016-04-01

    Full Text Available ABSTRACT Increased of sexual arousal (ISA has been described in different neurological diseases. The purpose of this study was present a case series of ISA in patients with movement disorders. Method Fifteen patients with different forms of movement disorders (Parkinson’s disease, Huntington’s disease, Tourette´s syndrome, spinocerebellar ataxia type 3, were evaluated in the Movement Disorders Unit of the Federal University of Paraná. Results Among Parkinson’s disease patients there were seven cases with different forms of ISA due to dopaminergic agonist use, levodopa abuse, and deep brain stimulation (DBS. In the group with hyperkinetic disorders, two patients with Huntington’s disease, two with Tourette’s syndrome, and four with spinocerebellar ataxia type 3 presented with ISA. Conclusions ISA in this group of patients had different etiologies, predominantly related to dopaminergic treatment or DBS in Parkinson’s disease, part of the background clinical picture in Huntington’s disease and Tourette’s syndrome, and probably associated with cultural aspects in patients with spinocerebellar ataxia type 3.

  11. Including model uncertainty in risk-informed decision making

    International Nuclear Information System (INIS)

    Reinert, Joshua M.; Apostolakis, George E.

    2006-01-01

    Model uncertainties can have a significant impact on decisions regarding licensing basis changes. We present a methodology to identify basic events in the risk assessment that have the potential to change the decision and are known to have significant model uncertainties. Because we work with basic event probabilities, this methodology is not appropriate for analyzing uncertainties that cause a structural change to the model, such as success criteria. We use the risk achievement worth (RAW) importance measure with respect to both the core damage frequency (CDF) and the change in core damage frequency (ΔCDF) to identify potentially important basic events. We cross-check these with generically important model uncertainties. Then, sensitivity analysis is performed on the basic event probabilities, which are used as a proxy for the model parameters, to determine how much error in these probabilities would need to be present in order to impact the decision. A previously submitted licensing basis change is used as a case study. Analysis using the SAPHIRE program identifies 20 basic events as important, four of which have model uncertainties that have been identified in the literature as generally important. The decision is fairly insensitive to uncertainties in these basic events. In three of these cases, one would need to show that model uncertainties would lead to basic event probabilities that would be between two and four orders of magnitude larger than modeled in the risk assessment before they would become important to the decision. More detailed analysis would be required to determine whether these higher probabilities are reasonable. Methods to perform this analysis from the literature are reviewed and an example is demonstrated using the case study

  12. Children and adolescents' internal models of food-sharing behavior include complex evaluations of contextual factors.

    Science.gov (United States)

    Markovits, Henry; Benenson, Joyce F; Kramer, Donald L

    2003-01-01

    This study examined internal representations of food sharing in 589 children and adolescents (8-19 years of age). Questionnaires, depicting a variety of contexts in which one person was asked to share a resource with another, were used to examine participants' expectations of food-sharing behavior. Factors that were varied included the value of the resource, the relation between the two depicted actors, the quality of this relation, and gender. Results indicate that internal models of food-sharing behavior showed systematic patterns of variation, demonstrating that individuals have complex contextually based internal models at all ages, including the youngest. Examination of developmental changes in use of individual patterns is consistent with the idea that internal models reflect age-specific patterns of interactions while undergoing a process of progressive consolidation.

  13. The Watts-Strogatz network model developed by including degree distribution: theory and computer simulation

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Y W [Surface Physics Laboratory and Department of Physics, Fudan University, Shanghai 200433 (China); Zhang, L F [Surface Physics Laboratory and Department of Physics, Fudan University, Shanghai 200433 (China); Huang, J P [Surface Physics Laboratory and Department of Physics, Fudan University, Shanghai 200433 (China)

    2007-07-20

    By using theoretical analysis and computer simulations, we develop the Watts-Strogatz network model by including degree distribution, in an attempt to improve the comparison between characteristic path lengths and clustering coefficients predicted by the original Watts-Strogatz network model and those of the real networks with the small-world property. Good agreement between the predictions of the theoretical analysis and those of the computer simulations has been shown. It is found that the developed Watts-Strogatz network model can fit the real small-world networks more satisfactorily. Some other interesting results are also reported by adjusting the parameters in a model degree-distribution function. The developed Watts-Strogatz network model is expected to help in the future analysis of various social problems as well as financial markets with the small-world property.

  14. The Watts-Strogatz network model developed by including degree distribution: theory and computer simulation

    International Nuclear Information System (INIS)

    Chen, Y W; Zhang, L F; Huang, J P

    2007-01-01

    By using theoretical analysis and computer simulations, we develop the Watts-Strogatz network model by including degree distribution, in an attempt to improve the comparison between characteristic path lengths and clustering coefficients predicted by the original Watts-Strogatz network model and those of the real networks with the small-world property. Good agreement between the predictions of the theoretical analysis and those of the computer simulations has been shown. It is found that the developed Watts-Strogatz network model can fit the real small-world networks more satisfactorily. Some other interesting results are also reported by adjusting the parameters in a model degree-distribution function. The developed Watts-Strogatz network model is expected to help in the future analysis of various social problems as well as financial markets with the small-world property

  15. The Benefits of Including Clinical Factors in Rectal Normal Tissue Complication Probability Modeling After Radiotherapy for Prostate Cancer

    International Nuclear Information System (INIS)

    Defraene, Gilles; Van den Bergh, Laura; Al-Mamgani, Abrahim; Haustermans, Karin; Heemsbergen, Wilma; Van den Heuvel, Frank; Lebesque, Joos V.

    2012-01-01

    Purpose: To study the impact of clinical predisposing factors on rectal normal tissue complication probability modeling using the updated results of the Dutch prostate dose-escalation trial. Methods and Materials: Toxicity data of 512 patients (conformally treated to 68 Gy [n = 284] and 78 Gy [n = 228]) with complete follow-up at 3 years after radiotherapy were studied. Scored end points were rectal bleeding, high stool frequency, and fecal incontinence. Two traditional dose-based models (Lyman-Kutcher-Burman (LKB) and Relative Seriality (RS) and a logistic model were fitted using a maximum likelihood approach. Furthermore, these model fits were improved by including the most significant clinical factors. The area under the receiver operating characteristic curve (AUC) was used to compare the discriminating ability of all fits. Results: Including clinical factors significantly increased the predictive power of the models for all end points. In the optimal LKB, RS, and logistic models for rectal bleeding and fecal incontinence, the first significant (p = 0.011–0.013) clinical factor was “previous abdominal surgery.” As second significant (p = 0.012–0.016) factor, “cardiac history” was included in all three rectal bleeding fits, whereas including “diabetes” was significant (p = 0.039–0.048) in fecal incontinence modeling but only in the LKB and logistic models. High stool frequency fits only benefitted significantly (p = 0.003–0.006) from the inclusion of the baseline toxicity score. For all models rectal bleeding fits had the highest AUC (0.77) where it was 0.63 and 0.68 for high stool frequency and fecal incontinence, respectively. LKB and logistic model fits resulted in similar values for the volume parameter. The steepness parameter was somewhat higher in the logistic model, also resulting in a slightly lower D 50 . Anal wall DVHs were used for fecal incontinence, whereas anorectal wall dose best described the other two endpoints. Conclusions

  16. The Benefits of Including Clinical Factors in Rectal Normal Tissue Complication Probability Modeling After Radiotherapy for Prostate Cancer

    Energy Technology Data Exchange (ETDEWEB)

    Defraene, Gilles, E-mail: gilles.defraene@uzleuven.be [Radiation Oncology Department, University Hospitals Leuven, Leuven (Belgium); Van den Bergh, Laura [Radiation Oncology Department, University Hospitals Leuven, Leuven (Belgium); Al-Mamgani, Abrahim [Department of Radiation Oncology, Erasmus Medical Center - Daniel den Hoed Cancer Center, Rotterdam (Netherlands); Haustermans, Karin [Radiation Oncology Department, University Hospitals Leuven, Leuven (Belgium); Heemsbergen, Wilma [Netherlands Cancer Institute - Antoni van Leeuwenhoek Hospital, Amsterdam (Netherlands); Van den Heuvel, Frank [Radiation Oncology Department, University Hospitals Leuven, Leuven (Belgium); Lebesque, Joos V. [Netherlands Cancer Institute - Antoni van Leeuwenhoek Hospital, Amsterdam (Netherlands)

    2012-03-01

    Purpose: To study the impact of clinical predisposing factors on rectal normal tissue complication probability modeling using the updated results of the Dutch prostate dose-escalation trial. Methods and Materials: Toxicity data of 512 patients (conformally treated to 68 Gy [n = 284] and 78 Gy [n = 228]) with complete follow-up at 3 years after radiotherapy were studied. Scored end points were rectal bleeding, high stool frequency, and fecal incontinence. Two traditional dose-based models (Lyman-Kutcher-Burman (LKB) and Relative Seriality (RS) and a logistic model were fitted using a maximum likelihood approach. Furthermore, these model fits were improved by including the most significant clinical factors. The area under the receiver operating characteristic curve (AUC) was used to compare the discriminating ability of all fits. Results: Including clinical factors significantly increased the predictive power of the models for all end points. In the optimal LKB, RS, and logistic models for rectal bleeding and fecal incontinence, the first significant (p = 0.011-0.013) clinical factor was 'previous abdominal surgery.' As second significant (p = 0.012-0.016) factor, 'cardiac history' was included in all three rectal bleeding fits, whereas including 'diabetes' was significant (p = 0.039-0.048) in fecal incontinence modeling but only in the LKB and logistic models. High stool frequency fits only benefitted significantly (p = 0.003-0.006) from the inclusion of the baseline toxicity score. For all models rectal bleeding fits had the highest AUC (0.77) where it was 0.63 and 0.68 for high stool frequency and fecal incontinence, respectively. LKB and logistic model fits resulted in similar values for the volume parameter. The steepness parameter was somewhat higher in the logistic model, also resulting in a slightly lower D{sub 50}. Anal wall DVHs were used for fecal incontinence, whereas anorectal wall dose best described the other two endpoints

  17. An imprecise Dirichlet model for Bayesian analysis of failure data including right-censored observations

    International Nuclear Information System (INIS)

    Coolen, F.P.A.

    1997-01-01

    This paper is intended to make researchers in reliability theory aware of a recently introduced Bayesian model with imprecise prior distributions for statistical inference on failure data, that can also be considered as a robust Bayesian model. The model consists of a multinomial distribution with Dirichlet priors, making the approach basically nonparametric. New results for the model are presented, related to right-censored observations, where estimation based on this model is closely related to the product-limit estimator, which is an important statistical method to deal with reliability or survival data including right-censored observations. As for the product-limit estimator, the model considered in this paper aims at not using any information other than that provided by observed data, but our model fits into the robust Bayesian context which has the advantage that all inferences can be based on probabilities or expectations, or bounds for probabilities or expectations. The model uses a finite partition of the time-axis, and as such it is also related to life-tables

  18. Analysis of electronic models for solar cells including energy resolved defect densities

    Energy Technology Data Exchange (ETDEWEB)

    Glitzky, Annegret

    2010-07-01

    We introduce an electronic model for solar cells including energy resolved defect densities. The resulting drift-diffusion model corresponds to a generalized van Roosbroeck system with additional source terms coupled with ODEs containing space and energy as parameters for all defect densities. The system has to be considered in heterostructures and with mixed boundary conditions from device simulation. We give a weak formulation of the problem. If the boundary data and the sources are compatible with thermodynamic equilibrium the free energy along solutions decays monotonously. In other cases it may be increasing, but we estimate its growth. We establish boundedness and uniqueness results and prove the existence of a weak solution. This is done by considering a regularized problem, showing its solvability and the boundedness of its solutions independent of the regularization level. (orig.)

  19. Mathematical Model of Two Phase Flow in Natural Draft Wet-Cooling Tower Including Flue Gas Injection

    Directory of Open Access Journals (Sweden)

    Hyhlík Tomáš

    2016-01-01

    Full Text Available The previously developed model of natural draft wet-cooling tower flow, heat and mass transfer is extended to be able to take into account the flow of supersaturated moist air. The two phase flow model is based on void fraction of gas phase which is included in the governing equations. Homogeneous equilibrium model, where the two phases are well mixed and have the same velocity, is used. The effect of flue gas injection is included into the developed mathematical model by using source terms in governing equations and by using momentum flux coefficient and kinetic energy flux coefficient. Heat and mass transfer in the fill zone is described by the system of ordinary differential equations, where the mass transfer is represented by measured fill Merkel number and heat transfer is calculated using prescribed Lewis factor.

  20. Cerebellar ataxia of early onset

    International Nuclear Information System (INIS)

    Yamashita, Sumimasa; Miyake, Shota; Yamada, Michiko; Iwamoto, Hiroko; Yamada, Kazuhiko.

    1989-01-01

    Eight cases of childhood cerebellar ataxia were reported. All these cases showed chronic cerebellar ataxia with early onset, and the other diseases of cerebellum such as infections, neoplasms and storage diseases were excluded by clinical symptoms and laboratory findings including blood counts, blood chemistry, lactate, pyruvate, ceruloplasmine, urinalysis, serum immunoglobulins, amino acid analysis in blood and urine, CSF analysis, leukocyte lysosomal enzymes, MCV, EMG, EEG and brain X-CT. Two pairs of siblings were included in this study. The clinical diagnosis were cerebellar type (5), spinocerebellar type (1), one Marinesco-Sjoegren syndrome and undetermined type (1). The age of onset was 1 to 5 years. The chief complaint was motor developmental delay in 6 cases; among them 5 patients could walk alone at the ages of 2 to 3 years'. Mental retardation was observed in 7 cases and epilepsy in 2. TRH was effective in 5 cases. The MRI study revealed that the area of medial sagittal slice of the cerebellum was reduced significantly in all cases and also that of pons was reduced in 5 cases. Different from typical adult onset spinocerebellar degenerations, most of the present cases have achieved slow developmental milestones and the clinical course was not progressive. Genetic factors are suspected in the pathogenesis of this disease in some cases. (author)

  1. Fluid-structure interaction including volumetric coupling with homogenised subdomains for modeling respiratory mechanics.

    Science.gov (United States)

    Yoshihara, Lena; Roth, Christian J; Wall, Wolfgang A

    2017-04-01

    In this article, a novel approach is presented for combining standard fluid-structure interaction with additional volumetric constraints to model fluid flow into and from homogenised solid domains. The proposed algorithm is particularly interesting for investigations in the field of respiratory mechanics as it enables the mutual coupling of airflow in the conducting part and local tissue deformation in the respiratory part of the lung by means of a volume constraint. In combination with a classical monolithic fluid-structure interaction approach, a comprehensive model of the human lung can be established that will be useful to gain new insights into respiratory mechanics in health and disease. To illustrate the validity and versatility of the novel approach, three numerical examples including a patient-specific lung model are presented. The proposed algorithm proves its capability of computing clinically relevant airflow distribution and tissue strain data at a level of detail that is not yet achievable, neither with current imaging techniques nor with existing computational models. Copyright © 2016 John Wiley & Sons, Ltd. Copyright © 2016 John Wiley & Sons, Ltd.

  2. Global stability for infectious disease models that include immigration of infected individuals and delay in the incidence

    Directory of Open Access Journals (Sweden)

    Chelsea Uggenti

    2018-03-01

    Full Text Available We begin with a detailed study of a delayed SI model of disease transmission with immigration into both classes. The incidence function allows for a nonlinear dependence on the infected population, including mass action and saturating incidence as special cases. Due to the immigration of infectives, there is no disease-free equilibrium and hence no basic reproduction number. We show there is a unique endemic equilibrium and that this equilibrium is globally asymptotically stable for all parameter values. The results include vector-style delay and latency-style delay. Next, we show that previous global stability results for an SEI model and an SVI model that include immigration of infectives and non-linear incidence but not delay can be extended to systems with vector-style delay and latency-style delay.

  3. Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia.

    Science.gov (United States)

    Piguet, Françoise; de Montigny, Charline; Vaucamps, Nadège; Reutenauer, Laurence; Eisenmann, Aurélie; Puccio, Hélène

    2018-05-28

    Friedreich ataxia (FA) is a rare mitochondrial disease characterized by sensory and spinocerebellar ataxia, hypertrophic cardiomyopathy, and diabetes, for which there is no treatment. FA is caused by reduced levels of frataxin (FXN), an essential mitochondrial protein involved in the biosynthesis of iron-sulfur (Fe-S) clusters. Despite significant progress in recent years, to date, there are no good models to explore and test therapeutic approaches to stop or reverse the ganglionopathy and the sensory neuropathy associated to frataxin deficiency. Here, we report a new conditional mouse model with complete frataxin deletion in parvalbumin-positive cells that recapitulate the sensory ataxia and neuropathy associated to FA, albeit with a more rapid and severe course. Interestingly, although fully dysfunctional, proprioceptive neurons can survive for many weeks without frataxin. Furthermore, we demonstrate that post-symptomatic delivery of frataxin-expressing AAV allows for rapid and complete rescue of the sensory neuropathy associated with frataxin deficiency, thus establishing the pre-clinical proof of concept for the potential of gene therapy in treating FA neuropathy. Copyright © 2018 The American Society of Gene and Cell Therapy. Published by Elsevier Inc. All rights reserved.

  4. Modeling of the dynamics of wind to power conversion including high wind speed behavior

    DEFF Research Database (Denmark)

    Litong-Palima, Marisciel; Bjerge, Martin Huus; Cutululis, Nicolaos Antonio

    2016-01-01

    This paper proposes and validates an efficient, generic and computationally simple dynamic model for the conversion of the wind speed at hub height into the electrical power by a wind turbine. This proposed wind turbine model was developed as a first step to simulate wind power time series...... for power system studies. This paper focuses on describing and validating the single wind turbine model, and is therefore neither describing wind speed modeling nor aggregation of contributions from a whole wind farm or a power system area. The state-of-the-art is to use static power curves for the purpose...... of power system studies, but the idea of the proposed wind turbine model is to include the main dynamic effects in order to have a better representation of the fluctuations in the output power and of the fast power ramping especially because of high wind speed shutdowns of the wind turbine. The high wind...

  5. Modelling and control of a microgrid including photovoltaic and wind generation

    Science.gov (United States)

    Hussain, Mohammed Touseef

    Extensive increase of distributed generation (DG) penetration and the existence of multiple DG units at distribution level have introduced the notion of micro-grid. This thesis develops a detailed non-linear and small-signal dynamic model of a microgrid that includes PV, wind and conventional small scale generation along with their power electronics interfaces and the filters. The models developed evaluate the amount of generation mix from various DGs for satisfactory steady state operation of the microgrid. In order to understand the interaction of the DGs on microgrid system initially two simpler configurations were considered. The first one consists of microalternator, PV and their electronics, and the second system consists of microalternator and wind system each connected to the power system grid. Nonlinear and linear state space model of each microgrid are developed. Small signal analysis showed that the large participation of PV/wind can drive the microgrid to the brink of unstable region without adequate control. Non-linear simulations are carried out to verify the results obtained through small-signal analysis. The role of the extent of generation mix of a composite microgrid consisting of wind, PV and conventional generation was investigated next. The findings of the smaller systems were verified through nonlinear and small signal modeling. A central supervisory capacitor energy storage controller interfaced through a STATCOM was proposed to monitor and enhance the microgrid operation. The potential of various control inputs to provide additional damping to the system has been evaluated through decomposition techniques. The signals identified to have damping contents were employed to design the supervisory control system. The controller gains were tuned through an optimal pole placement technique. Simulation studies demonstrate that the STATCOM voltage phase angle and PV inverter phase angle were the best inputs for enhanced stability boundaries.

  6. Importance of including small-scale tile drain discharge in the calibration of a coupled groundwater-surface water catchment model

    DEFF Research Database (Denmark)

    Hansen, Anne Lausten; Refsgaard, Jens Christian; Christensen, Britt Stenhøj Baun

    2013-01-01

    the catchment. In this study, a coupled groundwater-surface water model based on the MIKE SHE code was developed for the 4.7 km2 Lillebæk catchment in Denmark, where tile drain flow is a major contributor to the stream discharge. The catchment model was calibrated in several steps by incrementally including...... the observation data into the calibration to see the effect on model performance of including diverse data types, especially tile drain discharge. For the Lillebæk catchment, measurements of hydraulic head, daily stream discharge, and daily tile drain discharge from five small (1–4 ha) drainage areas exist....... The results showed that including tile drain data in the calibration of the catchment model improved its general performance for hydraulic heads and stream discharges. However, the model failed to correctly describe the local-scale dynamics of the tile drain discharges, and, furthermore, including the drain...

  7. Development of realistic concrete models including scaling effects

    International Nuclear Information System (INIS)

    Carpinteri, A.

    1989-09-01

    Progressive cracking in structural elements of concrete is considered. Two simple models are applied, which, even though different, lead to similar predictions for the fracture behaviour. Both Virtual Crack Propagation Model and Cohesive Limit Analysis (Section 2), show a trend towards brittle behaviour and catastrophical events for large structural sizes. A numerical Cohesive Crack Model is proposed (Section 3) to describe strain softening and strain localization in concrete. Such a model is able to predict the size effects of fracture mechanics accurately. Whereas for Mode I, only untieing of the finite element nodes is applied to simulate crack growth, for Mixed Mode a topological variation is required at each step (Section 4). In the case of the four point shear specimen, the load vs. deflection diagrams reveal snap-back instability for large sizes. By increasing the specimen sizes, such instability tends to reproduce the classical LEFM instability. Remarkable size effects are theoretically predicted and experimentally confirmed also for reinforced concrete (Section 5). The brittleness of the flexural members increases by increasing size and/or decreasing steel content. On the basis of these results, the empirical code rules regarding the minimum amount of reinforcement could be considerably revised

  8. Comparison between MRI and 3D-SSP in olivopontocerebellar atrophy and cortical cerebellar atrophy

    International Nuclear Information System (INIS)

    Hamaguchi, Hirotoshi; Kanda, Fumio; Hosaka, Kayo; Fujii, Masahiko; Chihara, Kazuo

    2004-01-01

    We compared images of three-dimensional stereotactic surface projections (3D-SSP) of SPECT with MRI images in spinocerebellar degeneration patients (13 olivopontocerebellar atrophy (OPCA) and 7 cortical cerebellar atrophy (CCA)). We analyzed a brain blood flow pattern with an image of statistics by 123 I-IMP SPECT. In OPCA patients, a blood flow reduction was more remarkable in 3D-SSP than a degree of cerebellar atrophy in MRI. In patients with CCA, the cerebellum showed little blood flow reduction in 3D-SSP despite of apparent atrophy in MRI. Simultaneous examination both MRI and 3D-SSP might be useful for differential diagnosis of spinocerebellar degenerations. (author)

  9. MHD model including small-scale perturbations in a plasma with temperature variations

    International Nuclear Information System (INIS)

    Kuvshinov, B.N.; Mikhailovskii, A.B.

    1996-01-01

    The possibility is studied of using a hydrodynamic model to describe a magnetized plasma with density and temperature variations on scales that are arbitrary with respect to the ion Larmor radius. It is shown that the inertial component of the transverse ion thermal flux should be taken into account. This component is found from the collisionless kinetic equation. It can also be obtained from the equations of the Grad type. A set of two-dimensional hydrodynamic equations for ions is obtained with this component taken into account. These equations are used to derive model hydrodynamic expressions for the density and temperature variations. It is shown that, for large-scale perturbations (when the wavelengths are longer than the ion Larmor radius), the expressions derived coincide with the corresponding kinetic expressions and, for perturbations on sub-Larmor scales (when the wavelengths are shorter than the Larmor radius), they agree qualitatively. Hydrodynamic dispersion relations are derived for several types of drift waves with arbitrary wavenumbers. The range of applicability of the MHD model is determined from a comparison of these dispersion relations with the kinetic ones. It is noted that, on the basis of results obtained, drift effects can be included in numerical MHD codes for studying plasma instabilities in high-temperature regimes in tokamaks

  10. Mathematical multi-scale model of the cardiovascular system including mitral valve dynamics. Application to ischemic mitral insufficiency

    Directory of Open Access Journals (Sweden)

    Moonen Marie

    2011-09-01

    Full Text Available Abstract Background Valve dysfunction is a common cardiovascular pathology. Despite significant clinical research, there is little formal study of how valve dysfunction affects overall circulatory dynamics. Validated models would offer the ability to better understand these dynamics and thus optimize diagnosis, as well as surgical and other interventions. Methods A cardiovascular and circulatory system (CVS model has already been validated in silico, and in several animal model studies. It accounts for valve dynamics using Heaviside functions to simulate a physiologically accurate "open on pressure, close on flow" law. However, it does not consider real-time valve opening dynamics and therefore does not fully capture valve dysfunction, particularly where the dysfunction involves partial closure. This research describes an updated version of this previous closed-loop CVS model that includes the progressive opening of the mitral valve, and is defined over the full cardiac cycle. Results Simulations of the cardiovascular system with healthy mitral valve are performed, and, the global hemodynamic behaviour is studied compared with previously validated results. The error between resulting pressure-volume (PV loops of already validated CVS model and the new CVS model that includes the progressive opening of the mitral valve is assessed and remains within typical measurement error and variability. Simulations of ischemic mitral insufficiency are also performed. Pressure-Volume loops, transmitral flow evolution and mitral valve aperture area evolution follow reported measurements in shape, amplitude and trends. Conclusions The resulting cardiovascular system model including mitral valve dynamics provides a foundation for clinical validation and the study of valvular dysfunction in vivo. The overall models and results could readily be generalised to other cardiac valves.

  11. Nonlocal continuum-based modeling of breathing mode of nanowires including surface stress and surface inertia effects

    Science.gov (United States)

    Ghavanloo, Esmaeal; Fazelzadeh, S. Ahmad; Rafii-Tabar, Hashem

    2014-05-01

    Nonlocal and surface effects significantly influence the mechanical response of nanomaterials and nanostructures. In this work, the breathing mode of a circular nanowire is studied on the basis of the nonlocal continuum model. Both the surface elastic properties and surface inertia effect are included. Nanowires can be modeled as long cylindrical solid objects. The classical model is reformulated using the nonlocal differential constitutive relations of Eringen and Gurtin-Murdoch surface continuum elasticity formalism. A new frequency equation for the breathing mode of nanowires, including small scale effect, surface stress and surface inertia is presented by employing the Bessel functions. Numerical results are computed, and are compared to confirm the validity and accuracy of the proposed method. Furthermore, the model is used to elucidate the effect of nonlocal parameter, the surface stress, the surface inertia and the nanowire orientation on the breathing mode of several types of nanowires with size ranging from 0.5 to 4 nm. Our results reveal that the combined surface and small scale effects are significant for nanowires with diameter smaller than 4 nm.

  12. Nonlocal continuum-based modeling of breathing mode of nanowires including surface stress and surface inertia effects

    International Nuclear Information System (INIS)

    Ghavanloo, Esmaeal; Fazelzadeh, S. Ahmad; Rafii-Tabar, Hashem

    2014-01-01

    Nonlocal and surface effects significantly influence the mechanical response of nanomaterials and nanostructures. In this work, the breathing mode of a circular nanowire is studied on the basis of the nonlocal continuum model. Both the surface elastic properties and surface inertia effect are included. Nanowires can be modeled as long cylindrical solid objects. The classical model is reformulated using the nonlocal differential constitutive relations of Eringen and Gurtin–Murdoch surface continuum elasticity formalism. A new frequency equation for the breathing mode of nanowires, including small scale effect, surface stress and surface inertia is presented by employing the Bessel functions. Numerical results are computed, and are compared to confirm the validity and accuracy of the proposed method. Furthermore, the model is used to elucidate the effect of nonlocal parameter, the surface stress, the surface inertia and the nanowire orientation on the breathing mode of several types of nanowires with size ranging from 0.5 to 4 nm. Our results reveal that the combined surface and small scale effects are significant for nanowires with diameter smaller than 4 nm.

  13. Nonlocal continuum-based modeling of breathing mode of nanowires including surface stress and surface inertia effects

    Energy Technology Data Exchange (ETDEWEB)

    Ghavanloo, Esmaeal, E-mail: ghavanloo@shirazu.ac.ir [School of Mechanical Engineering, Shiraz University, Shiraz 71963-16548 (Iran, Islamic Republic of); Fazelzadeh, S. Ahmad [School of Mechanical Engineering, Shiraz University, Shiraz 71963-16548 (Iran, Islamic Republic of); Rafii-Tabar, Hashem [Department of Medical Physics and Biomedical Engineering, Research Center for Medical Nanotechnology and Tissue Engineering, Shahid Beheshti University of Medical Sciences, Evin, Tehran (Iran, Islamic Republic of); Computational Physical Sciences Research Laboratory, School of Nano-Science, Institute for Research in Fundamental Sciences (IPM), Tehran (Iran, Islamic Republic of)

    2014-05-01

    Nonlocal and surface effects significantly influence the mechanical response of nanomaterials and nanostructures. In this work, the breathing mode of a circular nanowire is studied on the basis of the nonlocal continuum model. Both the surface elastic properties and surface inertia effect are included. Nanowires can be modeled as long cylindrical solid objects. The classical model is reformulated using the nonlocal differential constitutive relations of Eringen and Gurtin–Murdoch surface continuum elasticity formalism. A new frequency equation for the breathing mode of nanowires, including small scale effect, surface stress and surface inertia is presented by employing the Bessel functions. Numerical results are computed, and are compared to confirm the validity and accuracy of the proposed method. Furthermore, the model is used to elucidate the effect of nonlocal parameter, the surface stress, the surface inertia and the nanowire orientation on the breathing mode of several types of nanowires with size ranging from 0.5 to 4 nm. Our results reveal that the combined surface and small scale effects are significant for nanowires with diameter smaller than 4 nm.

  14. Influence of structural parameter included in nonlocal rock mass model on stress concentration around circular tunnel

    Science.gov (United States)

    Lavrikov, SV; Mikenina, OA; Revuzhenko, AF

    2018-03-01

    A model of elastic body, including local curvature of elementary volume, is matched with a nonlocal model with a linear structural parameter in the differential approximation. The problem on deformation of rock mass around a circular cross section tunnel is solved numerically. The contours of the calculated stresses are plotted. It is shown that inclusion of local bends in the model results in expansion of influence zone of the tunnel and reduces stress concentration factor at the tunnel boundary.

  15. Model for safety reports including descriptive examples; Mall foer saekerhetsrapporter med beskrivande exempel

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1995-12-01

    Several safety reports will be produced in the process of planning and constructing the system for disposal of high-level radioactive waste in Sweden. The present report gives a model, with detailed examples, of how these reports should be organized and what steps they should include. In the near future safety reports will deal with the encapsulation plant and the repository. Later reports will treat operation of the handling systems and the repository.

  16. Modeling of Pem Fuel Cell Systems Including Controls and Reforming Effects for Hybrid Automotive Applications

    National Research Council Canada - National Science Library

    Boettner, Daisie

    2001-01-01

    .... This study develops models for a stand-alone Proton Exchange Membrane (PEM) fuel cell stack, a direct-hydrogen fuel cell system including auxiliaries, and a methanol reforming fuel cell system for integration into a vehicle performance simulator...

  17. An Integrated Biochemistry Laboratory, Including Molecular Modeling

    Science.gov (United States)

    Hall, Adele J. Wolfson Mona L.; Branham, Thomas R.

    1996-11-01

    ) experience with methods of protein purification; (iii) incorporation of appropriate controls into experiments; (iv) use of basic statistics in data analysis; (v) writing papers and grant proposals in accepted scientific style; (vi) peer review; (vii) oral presentation of results and proposals; and (viii) introduction to molecular modeling. Figure 1 illustrates the modular nature of the lab curriculum. Elements from each of the exercises can be separated and treated as stand-alone exercises, or combined into short or long projects. We have been able to offer the opportunity to use sophisticated molecular modeling in the final module through funding from an NSF-ILI grant. However, many of the benefits of the research proposal can be achieved with other computer programs, or even by literature survey alone. Figure 1.Design of project-based biochemistry laboratory. Modules (projects, or portions of projects) are indicated as boxes. Each of these can be treated independently, or used as part of a larger project. Solid lines indicate some suggested paths from one module to the next. The skills and knowledge required for protein purification and design are developed in three units: (i) an introduction to critical assays needed to monitor degree of purification, including an evaluation of assay parameters; (ii) partial purification by ion-exchange techniques; and (iii) preparation of a grant proposal on protein design by mutagenesis. Brief descriptions of each of these units follow, with experimental details of each project at the end of this paper. Assays for Lysozyme Activity and Protein Concentration (4 weeks) The assays mastered during the first unit are a necessary tool for determining the purity of the enzyme during the second unit on purification by ion exchange. These assays allow an introduction to the concept of specific activity (units of enzyme activity per milligram of total protein) as a measure of purity. In this first sequence, students learn a turbidimetric assay

  18. Including the effects of filamentous bulking sludge during the simulation of wastewater treatment plants using a risk assessment model

    DEFF Research Database (Denmark)

    Flores Alsina, Xavier; Comas, J.; Rodriquez-Roda, I.

    2009-01-01

    The main objective of this paper is to demonstrate how including the occurrence of filamentous bulking sludge in a secondary clarifier model will affect the predicted process performance during the simulation of WWTPs. The IWA Benchmark Simulation Model No. 2 (BSM2) is hereby used as a simulation...... are automatically changed during the simulation by modifying the settling model parameters to mimic the effect of growth of filamentous bacteria. The simulation results demonstrate that including effects of filamentous bulking in the secondary clarifier model results in a more realistic plant performance...

  19. Include dispersion in quantum chemical modeling of enzymatic reactions: the case of isoaspartyl dipeptidase.

    Science.gov (United States)

    Zhang, Hai-Mei; Chen, Shi-Lu

    2015-06-09

    The lack of dispersion in the B3LYP functional has been proposed to be the main origin of big errors in quantum chemical modeling of a few enzymes and transition metal complexes. In this work, the essential dispersion effects that affect quantum chemical modeling are investigated. With binuclear zinc isoaspartyl dipeptidase (IAD) as an example, dispersion is included in the modeling of enzymatic reactions by two different procedures, i.e., (i) geometry optimizations followed by single-point calculations of dispersion (approach I) and (ii) the inclusion of dispersion throughout geometry optimization and energy evaluation (approach II). Based on a 169-atom chemical model, the calculations show a qualitative consistency between approaches I and II in energetics and most key geometries, demonstrating that both approaches are available with the latter preferential since both geometry and energy are dispersion-corrected in approach II. When a smaller model without Arg233 (147 atoms) was used, an inconsistency was observed, indicating that the missing dispersion interactions are essentially responsible for determining equilibrium geometries. Other technical issues and mechanistic characteristics of IAD are also discussed, in particular with respect to the effects of Arg233.

  20. Mean field diffusion models for precipitation in crystalline GaAs including surface tension and bulk stresses

    Energy Technology Data Exchange (ETDEWEB)

    Dreyer, Wolfgang [Weierstrass-Institut fuer Angewandte Analysis und Stochastik (WIAS) im Forschungsverbund Berlin e.V. (Germany); Kimmerle, Sven-Joachim [Humboldt-Univ. Berlin (Germany). Dept. of Mathematics

    2009-07-01

    Based on a thermodynamically consistent model for precipitation in gallium arsenide crystals including surface tension and bulk stresses by Dreyer and Duderstadt, we propose different mathematical models to describe the size evolution of liquid droplets in a crystalline solid. The first class of models treats the diffusion-controlled regime of interface motion, while the second class is concerned with the interface-controlled regime of interface motion. Our models take care of conservation of mass and substance. We consider homogenised models, where different length scales of the experimental situation have been exploited in order to simplify the equations. These homogenised models generalise the well-known Lifshitz-Slyozov-Wagner model for Ostwald ripening. Mean field models capture the main properties of our system and are well adapted for numerics and further analysis. Numerical evidence suggests in which case which one of the two regimes might be appropriate to the experimental situation. (orig.)

  1. Habitability of super-Earth planets around other suns: models including Red Giant Branch evolution.

    Science.gov (United States)

    von Bloh, W; Cuntz, M; Schröder, K-P; Bounama, C; Franck, S

    2009-01-01

    The unexpected diversity of exoplanets includes a growing number of super-Earth planets, i.e., exoplanets with masses of up to several Earth masses and a similar chemical and mineralogical composition as Earth. We present a thermal evolution model for a 10 Earth-mass planet orbiting a star like the Sun. Our model is based on the integrated system approach, which describes the photosynthetic biomass production and takes into account a variety of climatological, biogeochemical, and geodynamical processes. This allows us to identify a so-called photosynthesis-sustaining habitable zone (pHZ), as determined by the limits of biological productivity on the planetary surface. Our model considers solar evolution during the main-sequence stage and along the Red Giant Branch as described by the most recent solar model. We obtain a large set of solutions consistent with the principal possibility of life. The highest likelihood of habitability is found for "water worlds." Only mass-rich water worlds are able to realize pHZ-type habitability beyond the stellar main sequence on the Red Giant Branch.

  2. Empirical Validation of a Thermal Model of a Complex Roof Including Phase Change Materials

    Directory of Open Access Journals (Sweden)

    Stéphane Guichard

    2015-12-01

    Full Text Available This paper deals with the empirical validation of a building thermal model of a complex roof including a phase change material (PCM. A mathematical model dedicated to PCMs based on the heat apparent capacity method was implemented in a multi-zone building simulation code, the aim being to increase the understanding of the thermal behavior of the whole building with PCM technologies. In order to empirically validate the model, the methodology is based both on numerical and experimental studies. A parametric sensitivity analysis was performed and a set of parameters of the thermal model has been identified for optimization. The use of the generic optimization program called GenOpt® coupled to the building simulation code enabled to determine the set of adequate parameters. We first present the empirical validation methodology and main results of previous work. We then give an overview of GenOpt® and its coupling with the building simulation code. Finally, once the optimization results are obtained, comparisons of the thermal predictions with measurements are found to be acceptable and are presented.

  3. TS Fuzzy Model-Based Controller Design for a Class of Nonlinear Systems Including Nonsmooth Functions

    DEFF Research Database (Denmark)

    Vafamand, Navid; Asemani, Mohammad Hassan; Khayatiyan, Alireza

    2018-01-01

    This paper proposes a novel robust controller design for a class of nonlinear systems including hard nonlinearity functions. The proposed approach is based on Takagi-Sugeno (TS) fuzzy modeling, nonquadratic Lyapunov function, and nonparallel distributed compensation scheme. In this paper, a novel...... criterion, new robust controller design conditions in terms of linear matrix inequalities are derived. Three practical case studies, electric power steering system, a helicopter model and servo-mechanical system, are presented to demonstrate the importance of such class of nonlinear systems comprising...

  4. Improvement in genetic evaluation of female fertility in dairy cattle using multiple-trait models including milk production traits

    DEFF Research Database (Denmark)

    Sun, C; Madsen, P; Lund, M S

    2010-01-01

    This study investigated the improvement in genetic evaluation of fertility traits by using production traits as secondary traits (MILK = 305-d milk yield, FAT = 305-d fat yield, and PROT = 305-d protein yield). Data including 471,742 records from first lactations of Denmark Holstein cows, covering...... the years of inseminations during first lactations from 1995 to 2004, were analyzed. Six fertility traits (i.e., interval in days from calving to first insemination, calving interval, days open, interval in days from first to last insemination, numbers of inseminations per conception, and nonreturn rate...... stability and predictive ability than single-trait models for all the fertility traits, except for nonreturn rate within 56 d after first service. The stability and predictive ability for the model including MILK or PROT were similar to the model including all 3 milk production traits and better than...

  5. Validation on groundwater flow model including sea level change. Modeling on groundwater flow in coastal granite area

    International Nuclear Information System (INIS)

    Hasegawa, Takuma; Miyakawa, Kimio

    2009-01-01

    It is important to verify the groundwater flow model that reproduces pressure head, water chemistry, and groundwater age. However, water chemistry and groundwater age are considered to be influenced by historical events. In this study, sea level change during glacial-interglacial cycle was taken into account for simulating salinity and groundwater age at coastal granite area. As a result of simulation, salinity movement could not catch up with sea level changes, and mixing zone was formed below the fresh-water zone. This mixing zone was observed in the field measurement, and the observed salinities were agreed with simulated results including sea level change. The simulated residence time including sea level change is one-tenth of steady state. The reason is that the saline water was washed out during regression and modern sea-water was infiltrated during transgression. As mentioned before, considering sea level change are important to reproduce salinity and helium age at coastal area. (author)

  6. Cerebellar ataxia of early onset. Clinical symptoms and MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Yamashita, Sumimasa; Miyake, Shota; Yamada, Michiko; Iwamoto, Hiroko (Kanagawa Children' s Medical Center, Yokohama (Japan)); Yamada, Kazuhiko

    1989-07-01

    Eight cases of childhood cerebellar ataxia were reported. All these cases showed chronic cerebellar ataxia with early onset, and the other diseases of cerebellum such as infections, neoplasms and storage diseases were excluded by clinical symptoms and laboratory findings including blood counts, blood chemistry, lactate, pyruvate, ceruloplasmine, urinalysis, serum immunoglobulins, amino acid analysis in blood and urine, CSF analysis, leukocyte lysosomal enzymes, MCV, EMG, EEG and brain X-CT. Two pairs of siblings were included in this study. The clinical diagnosis were cerebellar type (5), spinocerebellar type (1), one Marinesco-Sjoegren syndrome and undetermined type (1). The age of onset was 1 to 5 years. The chief complaint was motor developmental delay in 6 cases; among them 5 patients could walk alone at the ages of 2 to 3 years'. Mental retardation was observed in 7 cases and epilepsy in 2. TRH was effective in 5 cases. The MRI study revealed that the area of medial sagittal slice of the cerebellum was reduced significantly in all cases and also that of pons was reduced in 5 cases. Different from typical adult onset spinocerebellar degenerations, most of the present cases have achieved slow developmental milestones and the clinical course was not progressive. Genetic factors are suspected in the pathogenesis of this disease in some cases. (author).

  7. A New Circuit Model for Spin-Torque Oscillator Including Perpendicular Torque of Magnetic Tunnel Junction

    Directory of Open Access Journals (Sweden)

    Hyein Lim

    2013-01-01

    Full Text Available Spin-torque oscillator (STO is a promising new technology for the future RF oscillators, which is based on the spin-transfer torque (STT effect in magnetic multilayered nanostructure. It is expected to provide a larger tunability, smaller size, lower power consumption, and higher level of integration than the semiconductor-based oscillators. In our previous work, a circuit-level model of the giant magnetoresistance (GMR STO was proposed. In this paper, we present a physics-based circuit-level model of the magnetic tunnel junction (MTJ-based STO. MTJ-STO model includes the effect of perpendicular torque that has been ignored in the GMR-STO model. The variations of three major characteristics, generation frequency, mean oscillation power, and generation linewidth of an MTJ-STO with respect to the amount of perpendicular torque, are investigated, and the results are applied to our model. The operation of the model was verified by HSPICE simulation, and the results show an excellent agreement with the experimental data. The results also prove that a full circuit-level simulation with MJT-STO devices can be made with our proposed model.

  8. Dynamic experiments with high bisphenol-A concentrations modelled with an ASM model extended to include a separate XOC degrading microorganism

    DEFF Research Database (Denmark)

    Lindblom, Erik Ulfson; Press-Kristensen, Kåre; Vanrolleghem, P.A.

    2009-01-01

    The perspective of this work is to develop a model, which can be used to better understand and optimize wastewater treatment plants that are able to remove xenobiotic organic compounds (XOCs) in combination with removal of traditional pollutants. Results from dynamic experiments conducted...... with the endocrine disrupting XOC bisphenol-A (BPA) in an activated sludge process with real wastewater were used to hypothesize an ASM-based process model including aerobic growth of a specific BPA-degrading microorganism and sorption of BPA to sludge. A parameter estimation method was developed, which...... simultaneously utilizes steady-state background concentrations and dynamic step response data, as well as conceptual simplifications of the plant configuration. Validation results show that biodegradation of BPA is sensitive to operational conditions before and during the experiment and that the proposed model...

  9. Challenges of including nitrogen effects on decomposition in earth system models

    Science.gov (United States)

    Hobbie, S. E.

    2011-12-01

    Despite the importance of litter decomposition for ecosystem fertility and carbon balance, key uncertainties remain about how this fundamental process is affected by nitrogen (N) availability. Nevertheless, resolving such uncertainties is critical for mechanistic inclusion of such processes in earth system models, towards predicting the ecosystem consequences of increased anthropogenic reactive N. Towards that end, we have conducted a series of experiments examining nitrogen effects on litter decomposition. We found that both substrate N and externally supplied N (regardless of form) accelerated the initial decomposition rate. Faster initial decomposition rates were linked to the higher activity of carbohydrate-degrading enzymes associated with externally supplied N and the greater relative abundances of Gram negative and Gram positive bacteria associated with green leaves and externally supplied organic N (assessed using phospholipid fatty acid analysis, PLFA). By contrast, later in decomposition, externally supplied N slowed decomposition, increasing the fraction of slowly decomposing litter and reducing lignin-degrading enzyme activity and relative abundances of Gram negative and Gram positive bacteria. Our results suggest that elevated atmospheric N deposition may have contrasting effects on the dynamics of different soil carbon pools, decreasing mean residence times of active fractions comprising very fresh litter, while increasing those of more slowly decomposing fractions including more processed litter. Incorporating these contrasting effects of N on decomposition processes into models is complicated by lingering uncertainties about how these effects generalize across ecosystems and substrates.

  10. A generalized model for optimal transport of images including dissipation and density modulation

    KAUST Repository

    Maas, Jan

    2015-11-01

    © EDP Sciences, SMAI 2015. In this paper the optimal transport and the metamorphosis perspectives are combined. For a pair of given input images geodesic paths in the space of images are defined as minimizers of a resulting path energy. To this end, the underlying Riemannian metric measures the rate of transport cost and the rate of viscous dissipation. Furthermore, the model is capable to deal with strongly varying image contrast and explicitly allows for sources and sinks in the transport equations which are incorporated in the metric related to the metamorphosis approach by Trouvé and Younes. In the non-viscous case with source term existence of geodesic paths is proven in the space of measures. The proposed model is explored on the range from merely optimal transport to strongly dissipative dynamics. For this model a robust and effective variational time discretization of geodesic paths is proposed. This requires to minimize a discrete path energy consisting of a sum of consecutive image matching functionals. These functionals are defined on corresponding pairs of intensity functions and on associated pairwise matching deformations. Existence of time discrete geodesics is demonstrated. Furthermore, a finite element implementation is proposed and applied to instructive test cases and to real images. In the non-viscous case this is compared to the algorithm proposed by Benamou and Brenier including a discretization of the source term. Finally, the model is generalized to define discrete weighted barycentres with applications to textures and objects.

  11. Global atmospheric model for mercury including oxidation by bromine atoms

    Directory of Open Access Journals (Sweden)

    C. D. Holmes

    2010-12-01

    Full Text Available Global models of atmospheric mercury generally assume that gas-phase OH and ozone are the main oxidants converting Hg0 to HgII and thus driving mercury deposition to ecosystems. However, thermodynamic considerations argue against the importance of these reactions. We demonstrate here the viability of atomic bromine (Br as an alternative Hg0 oxidant. We conduct a global 3-D simulation with the GEOS-Chem model assuming gas-phase Br to be the sole Hg0 oxidant (Hg + Br model and compare to the previous version of the model with OH and ozone as the sole oxidants (Hg + OH/O3 model. We specify global 3-D Br concentration fields based on our best understanding of tropospheric and stratospheric Br chemistry. In both the Hg + Br and Hg + OH/O3 models, we add an aqueous photochemical reduction of HgII in cloud to impose a tropospheric lifetime for mercury of 6.5 months against deposition, as needed to reconcile observed total gaseous mercury (TGM concentrations with current estimates of anthropogenic emissions. This added reduction would not be necessary in the Hg + Br model if we adjusted the Br oxidation kinetics downward within their range of uncertainty. We find that the Hg + Br and Hg + OH/O3 models are equally capable of reproducing the spatial distribution of TGM and its seasonal cycle at northern mid-latitudes. The Hg + Br model shows a steeper decline of TGM concentrations from the tropics to southern mid-latitudes. Only the Hg + Br model can reproduce the springtime depletion and summer rebound of TGM observed at polar sites; the snowpack component of GEOS-Chem suggests that 40% of HgII deposited to snow in the Arctic is transferred to the ocean and land reservoirs, amounting to a net deposition flux to the Arctic of 60 Mg a−1. Summertime events of depleted Hg0 at Antarctic sites due to subsidence are much better simulated by

  12. Web-accessible molecular modeling with Rosetta: The Rosetta Online Server that Includes Everyone (ROSIE).

    Science.gov (United States)

    Moretti, Rocco; Lyskov, Sergey; Das, Rhiju; Meiler, Jens; Gray, Jeffrey J

    2018-01-01

    The Rosetta molecular modeling software package provides a large number of experimentally validated tools for modeling and designing proteins, nucleic acids, and other biopolymers, with new protocols being added continually. While freely available to academic users, external usage is limited by the need for expertise in the Unix command line environment. To make Rosetta protocols available to a wider audience, we previously created a web server called Rosetta Online Server that Includes Everyone (ROSIE), which provides a common environment for hosting web-accessible Rosetta protocols. Here we describe a simplification of the ROSIE protocol specification format, one that permits easier implementation of Rosetta protocols. Whereas the previous format required creating multiple separate files in different locations, the new format allows specification of the protocol in a single file. This new, simplified protocol specification has more than doubled the number of Rosetta protocols available under ROSIE. These new applications include pK a determination, lipid accessibility calculation, ribonucleic acid redesign, protein-protein docking, protein-small molecule docking, symmetric docking, antibody docking, cyclic toxin docking, critical binding peptide determination, and mapping small molecule binding sites. ROSIE is freely available to academic users at http://rosie.rosettacommons.org. © 2017 The Protein Society.

  13. Time domain contact model for tyre/road interaction including nonlinear contact stiffness due to small-scale roughness

    Science.gov (United States)

    Andersson, P. B. U.; Kropp, W.

    2008-11-01

    Rolling resistance, traction, wear, excitation of vibrations, and noise generation are all attributes to consider in optimisation of the interaction between automotive tyres and wearing courses of roads. The key to understand and describe the interaction is to include a wide range of length scales in the description of the contact geometry. This means including scales on the order of micrometres that have been neglected in previous tyre/road interaction models. A time domain contact model for the tyre/road interaction that includes interfacial details is presented. The contact geometry is discretised into multiple elements forming pairs of matching points. The dynamic response of the tyre is calculated by convolving the contact forces with pre-calculated Green's functions. The smaller-length scales are included by using constitutive interfacial relations, i.e. by using nonlinear contact springs, for each pair of contact elements. The method is presented for normal (out-of-plane) contact and a method for assessing the stiffness of the nonlinear springs based on detailed geometry and elastic data of the tread is suggested. The governing equations of the nonlinear contact problem are solved with the Newton-Raphson iterative scheme. Relations between force, indentation, and contact stiffness are calculated for a single tread block in contact with a road surface. The calculated results have the same character as results from measurements found in literature. Comparison to traditional contact formulations shows that the effect of the small-scale roughness is large; the contact stiffness is only up to half of the stiffness that would result if contact is made over the whole element directly to the bulk of the tread. It is concluded that the suggested contact formulation is a suitable model to include more details of the contact interface. Further, the presented result for the tread block in contact with the road is a suitable input for a global tyre/road interaction model

  14. A new model for including the effect of fly ash on biochemical methane potential.

    Science.gov (United States)

    Gertner, Pablo; Huiliñir, César; Pinto-Villegas, Paula; Castillo, Alejandra; Montalvo, Silvio; Guerrero, Lorna

    2017-10-01

    The modelling of the effect of trace elements on anaerobic digestion, and specifically the effect of fly ash, has been scarcely studied. Thus, the present work was aimed at the development of a new function that allows accumulated methane models to predict the effect of FA on the volume of methane accumulation. For this, purpose five fly ash concentrations (10, 25, 50, 250 and 500mg/L) using raw and pre-treated sewage sludge were used to calibrate the new function, while three fly ash concentrations were used (40, 150 and 350mg/L) for validation. Three models for accumulated methane volume (the modified Gompertz equation, the logistic function, and the transfer function) were evaluated. The results showed that methane production increased in the presence of FA when the sewage sludge was not pre-treated, while with pretreated sludge there is inhibition of methane production at FA concentrations higher than 50mg/L. In the calibration of the proposed function, it fits well with the experimental data under all the conditions, including the inhibition and stimulating zones, with the values of the parameters of the methane production models falling in the range of those reported in the literature. For validation experiments, the model succeeded in representing the behavior of new experiments in both the stimulating and inhibiting zones, with NRMSE and R 2 ranging from 0.3577 to 0.03714 and 0.2209 to 0.9911, respectively. Thus, the proposed model is robust and valid for the studied conditions. Copyright © 2017 Elsevier Ltd. All rights reserved.

  15. A Hydrological Concept including Lateral Water Flow Compatible with the Biogeochemical Model ForSAFE

    Directory of Open Access Journals (Sweden)

    Giuliana Zanchi

    2016-03-01

    Full Text Available The study presents a hydrology concept developed to include lateral water flow in the biogeochemical model ForSAFE. The hydrology concept was evaluated against data collected at Svartberget in the Vindeln Research Forest in Northern Sweden. The results show that the new concept allows simulation of a saturated and an unsaturated zone in the soil as well as water flow that reaches the stream comparable to measurements. The most relevant differences compared to streamflow measurements are that the model simulates a higher base flow in winter and lower flow peaks after snowmelt. These differences are mainly caused by the assumptions made to regulate the percolation at the bottom of the simulated soil columns. The capability for simulating lateral flows and a saturated zone in ForSAFE can greatly improve the simulation of chemical exchange in the soil and export of elements from the soil to watercourses. Such a model can help improve the understanding of how environmental changes in the forest landscape will influence chemical loads to surface waters.

  16. Constitutive modeling of multiphase materials including phase transformations

    NARCIS (Netherlands)

    Perdahcioglu, Emin Semih; Geijselaers, Hubertus J.M.; Khan, A.S.; Meredith, C; Farrokh, B

    2011-01-01

    A constitutive model is developed for materials involving two or more different phases in their microstructure such as DP (Dual Phase) or TRIP (TRansformation Induced Plasticity) steels. Homogenization of the response of the phases is achieved by the Mean-Field method. One of the phases in TRIP

  17. Fuzzy Control of Yaw and Roll Angles of a Simulated Helicopter Model Includes Articulated Manipulators

    Directory of Open Access Journals (Sweden)

    Hossein Sadegh Lafmejani

    2015-09-01

    Full Text Available Fuzzy logic controller (FLC is a heuristic method by If-Then Rules which resembles human intelligence and it is a good method for designing Non-linear control systems. In this paper, an arbitrary helicopter model includes articulated manipulators has been simulated with Matlab SimMechanics toolbox. Due to the difficulties of modeling this complex system, a fuzzy controller with simple fuzzy rules has been designed for its yaw and roll angles in order to stabilize the helicopter while it is in the presence of disturbances or its manipulators are moving for a task. Results reveal that a simple FLC can appropriately control this system.

  18. Effect of including decay chains on predictions of equilibrium-type terrestrial food chain models

    International Nuclear Information System (INIS)

    Kirchner, G.

    1990-01-01

    Equilibrium-type food chain models are commonly used for assessing the radiological impact to man from environmental releases of radionuclides. Usually these do not take into account build-up of radioactive decay products during environmental transport. This may be a potential source of underprediction. For estimating consequences of this simplification, the equations of an internationally recognised terrestrial food chain model have been extended to include decay chains of variable length. Example calculations show that for releases from light water reactors as expected both during routine operation and in the case of severe accidents, the build-up of decay products during environmental transport is generally of minor importance. However, a considerable number of radionuclides of potential radiological significance have been identified which show marked contributions of decay products to calculated contamination of human food and resulting radiation dose rates. (author)

  19. Modelling of Water Cooled Fuel Including Design Basis and Severe Accidents. Proceedings of a Technical Meeting

    International Nuclear Information System (INIS)

    2015-11-01

    The demands on nuclear fuel have recently been increasing, and include transient regimes, higher discharge burnup and longer fuel cycles. This has resulted in an increase of loads on fuel and core internals. In order to satisfy these demands while ensuring compliance with safety criteria, new national and international programmes have been launched and advanced modelling codes are being developed. The Fukushima Daiichi accident has particularly demonstrated the need for adequate analysis of all aspects of fuel performance to prevent a failure and also to predict fuel behaviour were an accident to occur.This publication presents the Proceedings of the Technical Meeting on Modelling of Water Cooled Fuel Including Design Basis and Severe Accidents, which was hosted by the Nuclear Power Institute of China (NPIC) in Chengdu, China, following the recommendation made in 2013 at the IAEA Technical Working Group on Fuel Performance and Technology. This recommendation was in agreement with IAEA mid-term initiatives, linked to the post-Fukushima IAEA Nuclear Safety Action Plan, as well as the forthcoming Coordinated Research Project (CRP) on Fuel Modelling in Accident Conditions. At the technical meeting in Chengdu, major areas and physical phenomena, as well as types of code and experiment to be studied and used in the CRP, were discussed. The technical meeting provided a forum for international experts to review the state of the art of code development for modelling fuel performance of nuclear fuel for water cooled reactors with regard to steady state and transient conditions, and for design basis and early phases of severe accidents, including experimental support for code validation. A round table discussion focused on the needs and perspectives on fuel modelling in accident conditions. This meeting was the ninth in a series of IAEA meetings, which reflects Member States’ continuing interest in nuclear fuel issues. The previous meetings were held in 1980 (jointly with

  20. Can a one-layer optical skin model including melanin and inhomogeneously distributed blood explain spatially resolved diffuse reflectance spectra?

    Science.gov (United States)

    Karlsson, Hanna; Pettersson, Anders; Larsson, Marcus; Strömberg, Tomas

    2011-02-01

    Model based analysis of calibrated diffuse reflectance spectroscopy can be used for determining oxygenation and concentration of skin chromophores. This study aimed at assessing the effect of including melanin in addition to hemoglobin (Hb) as chromophores and compensating for inhomogeneously distributed blood (vessel packaging), in a single-layer skin model. Spectra from four humans were collected during different provocations using a twochannel fiber optic probe with source-detector separations 0.4 and 1.2 mm. Absolute calibrated spectra using data from either a single distance or both distances were analyzed using inverse Monte Carlo for light transport and Levenberg-Marquardt for non-linear fitting. The model fitting was excellent using a single distance. However, the estimated model failed to explain spectra from the other distance. The two-distance model did not fit the data well at either distance. Model fitting was significantly improved including melanin and vessel packaging. The most prominent effect when fitting data from the larger separation compared to the smaller separation was a different light scattering decay with wavelength, while the tissue fraction of Hb and saturation were similar. For modeling spectra at both distances, we propose using either a multi-layer skin model or a more advanced model for the scattering phase function.

  1. A temperature dependent cyclic plasticity model for hot work tool steel including particle coarsening

    Science.gov (United States)

    Jilg, Andreas; Seifert, Thomas

    2018-05-01

    Hot work tools are subjected to complex thermal and mechanical loads during hot forming processes. Locally, the stresses can exceed the material's yield strength in highly loaded areas as e.g. in small radii in die cavities. To sustain the high loads, the hot forming tools are typically made of martensitic hot work steels. While temperatures for annealing of the tool steels usually lie in the range between 400 and 600 °C, the steels may experience even higher temperatures during hot forming, resulting in softening of the material due to coarsening of strengthening particles. In this paper, a temperature dependent cyclic plasticity model for the martensitic hot work tool steel 1.2367 (X38CrMoV5-3) is presented that includes softening due to particle coarsening and that can be applied in finite-element calculations to assess the effect of softening on the thermomechanical fatigue life of hot work tools. To this end, a kinetic model for the evolution of the mean size of secondary carbides based on Ostwald ripening is coupled with a cyclic plasticity model with kinematic hardening. Mechanism-based relations are developed to describe the dependency of the mechanical properties on carbide size and temperature. The material properties of the mechanical and kinetic model are determined on the basis of tempering hardness curves as well as monotonic and cyclic tests.

  2. A model for Huanglongbing spread between citrus plants including delay times and human intervention

    Science.gov (United States)

    Vilamiu, Raphael G. d'A.; Ternes, Sonia; Braga, Guilherme A.; Laranjeira, Francisco F.

    2012-09-01

    The objective of this work was to present a compartmental deterministic mathematical model for representing the dynamics of HLB disease in a citrus orchard, including delay in the disease's incubation phase in the plants, and a delay period on the nymphal stage of Diaphorina citri, the most important HLB insect vector in Brazil. Numerical simulations were performed to assess the possible impacts of human detection efficiency of symptomatic plants, as well as the influence of a long incubation period of HLB in the plant.

  3. ATEFlap aerodynamic model, a dynamic stall model including the effects of trailing edge flap deflection

    Energy Technology Data Exchange (ETDEWEB)

    Bergami, L.; Gaunaa, M.

    2012-02-15

    The report presents the ATEFlap aerodynamic model, which computes the unsteady lift, drag and moment on a 2D airfoil section equipped with Adaptive Trailing Edge Flap. The model captures the unsteady response related to the effects of the vorticity shed into the wake, and the dynamics of flow separation a thin-airfoil potential flow model is merged with a dynamic stall model of the Beddoes-Leishmann type. The inputs required by the model are steady data for lift, drag, and moment coefficients as function of angle of attack and flap deflection. Further steady data used by the Beddoes- Leishmann dynamic stall model are computed in an external preprocessor application, which gives the user the possibility to verify, and eventually correct, the steady data passed to the aerodynamic model. The ATEFlap aerodynamic model is integrated in the aeroelastic simulation tool HAWC2, thus al- lowing to simulate the response of a wind turbine with trailing edge flaps on the rotor. The algorithms used by the preprocessor, and by aerodynamic model are presented, and modifications to previous implementations of the aerodynamic model are briefly discussed. The performance and the validity of the model are verified by comparing the dynamic response computed by the ATEFlap with solutions from CFD simulations. (Author)

  4. Transport modelling including radial electric field and plasma rotation

    International Nuclear Information System (INIS)

    Fukuyama, A.; Fuji, Y.; Itoh, S.-I.

    1994-01-01

    Using a simple turbulent transport model with a constant diffusion coefficient and a fixed temperature profile, the density profile in a steady state and the transient behaviour during the co and counter neutral beam injection are studied. More consistent analysis has been initiated with a turbulent transport model based on the current diffusive high-n ballooning mode. The enhancement of the radial electric field due to ion orbit losses and the reduction of the transport due to the poloidal rotation shear are demonstrated. The preliminary calculation indicates a sensitive temperature dependence of the density profile. (author)

  5. How to include frequency dependent complex permeability Into SPICE models to improve EMI filters design?

    Science.gov (United States)

    Sixdenier, Fabien; Yade, Ousseynou; Martin, Christian; Bréard, Arnaud; Vollaire, Christian

    2018-05-01

    Electromagnetic interference (EMI) filters design is a rather difficult task where engineers have to choose adequate magnetic materials, design the magnetic circuit and choose the size and number of turns. The final design must achieve the attenuation requirements (constraints) and has to be as compact as possible (goal). Alternating current (AC) analysis is a powerful tool to predict global impedance or attenuation of any filter. However, AC analysis are generally performed without taking into account the frequency-dependent complex permeability behaviour of soft magnetic materials. That's why, we developed two frequency-dependent complex permeability models able to be included into SPICE models. After an identification process, the performances of each model are compared to measurements made on a realistic EMI filter prototype in common mode (CM) and differential mode (DM) to see the benefit of the approach. Simulation results are in good agreement with the measured ones especially in the middle frequency range.

  6. Modelling the residual stresses and microstructural evolution in Friction Stir Welding of AA2024-T3 including the Wagner-Kampmann precipitation model

    DEFF Research Database (Denmark)

    Sonne, Mads Rostgaard; Hattel, Jesper Henri

    In this work, a numerical finite element model for friction stir welding of 2024-T3 aluminum alloy, consisting of a heat transfer analysis and a sequentially coupled quasi-static stress analysis is proposed. Metallurgical softening of the material is properly considered and included...

  7. Including model uncertainty in the model predictive control with output feedback

    Directory of Open Access Journals (Sweden)

    Rodrigues M.A.

    2002-01-01

    Full Text Available This paper addresses the development of an efficient numerical output feedback robust model predictive controller for open-loop stable systems. Stability of the closed loop is guaranteed by using an infinite horizon predictive controller and a stable state observer. The performance and the computational burden of this approach are compared to a robust predictive controller from the literature. The case used for this study is based on an industrial gasoline debutanizer column.

  8. The prediction of the cavitation phenomena including population balance modeling

    Science.gov (United States)

    Bannari, Rachid; Hliwa, Ghizlane Zineb; Bannari, Abdelfettah; Belghiti, Mly Taib

    2017-07-01

    Cavitation is the principal reason behind the behavior's modification of the hydraulic turbines. However, the experimental observations can not be appropriate to all cases due to the limitations in the measurement techniques. The mathematical models which have been implemented, use the mixture multiphase frame. As well as, most of the published work is limited by considering a constant bubble size distribution. However, this assumption is not realist. The aim of this article is the implementation and the use of a non-homogeneous multiphase model which solve two phases transport equation. The evolution of bubble size is considered by the population balance equation. This study is based on the eulerian-eulerian model, associated to the cavitation model. All the inter-phase forces such as drag, lift and virtual mass are used.

  9. Static, Lightweight Includes Resolution for PHP

    NARCIS (Netherlands)

    M.A. Hills (Mark); P. Klint (Paul); J.J. Vinju (Jurgen)

    2014-01-01

    htmlabstractDynamic languages include a number of features that are challenging to model properly in static analysis tools. In PHP, one of these features is the include expression, where an arbitrary expression provides the path of the file to include at runtime. In this paper we present two

  10. Indole and synthetic derivative activate chaperone expression to reduce polyQ aggregation in SCA17 neuronal cell and slice culture models

    Directory of Open Access Journals (Sweden)

    Kung PJ

    2014-10-01

    Full Text Available Pin-Jui Kung,1,* Yu-Chen Tao,1,* Ho-Chiang Hsu,1 Wan-Ling Chen,1 Te-Hsien Lin,1 Donala Janreddy,2 Ching-Fa Yao,2 Kuo-Hsuan Chang,3 Jung-Yaw Lin,1 Ming-Tsan Su,1 Chung-Hsin Wu,1 Guey-Jen Lee-Chen,1 Hsiu-Mei Hsieh-Li1 1Department of Life Science, 2Department of Chemistry, National Taiwan Normal University, Taipei, Taiwan; 3Department of Neurology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taipei, Taiwan *These authors contributed equally to this work Abstract: In spinocerebellar ataxia type 17 (SCA17, the expansion of a translated CAG repeat in the TATA box binding protein (TBP gene results in a long polyglutamine (polyQ tract in the TBP protein, leading to intracellular accumulation of aggregated TBP and cell death. The molecular chaperones act in preventing protein aggregation to ameliorate downstream harmful events. In this study, we used Tet-On SH-SY5Y cells with inducible SCA17 TBP/Q79-green fluorescent protein (GFP expression to test indole and synthetic derivative NC001-8 for neuroprotection. We found that indole and NC001-8 up-regulated chaperone expression to reduce polyQ aggregation in neuronal differentiated TBP/Q79 cells. The effects on promoting neurite outgrowth and on reduction of aggregation on Purkinje cells were also confirmed with cerebellar primary and slice cultures of SCA17 transgenic mice. Our results demonstrate how indole and derivative NC001-8 reduce polyQ aggregation to support their therapeutic potentials in SCA17 treatment. Keywords: spinocerebellar ataxia type 17, TATA box binding protein, polyQ aggregation, indole and derivative, therapeutics

  11. Iwamoto-Harada coalescence/pickup model for cluster emission: state density approach including angular momentum variables

    Directory of Open Access Journals (Sweden)

    Běták Emil

    2014-04-01

    Full Text Available For low-energy nuclear reactions well above the resonance region, but still below the pion threshold, statistical pre-equilibrium models (e.g., the exciton and the hybrid ones are a frequent tool for analysis of energy spectra and the cross sections of cluster emission. For α’s, two essentially distinct approaches are popular, namely the preformed one and the different versions of coalescence approaches, whereas only the latter group of models can be used for other types of cluster ejectiles. The original Iwamoto-Harada model of pre-equilibrium cluster emission was formulated using the overlap of the cluster and its constituent nucleons in momentum space. Transforming it into level or state densities is not a straigthforward task; however, physically the same model was presented at a conference on reaction models five years earlier. At that time, only the densities without spin were used. The introduction of spin variables into the exciton model enabled detailed calculation of the γ emission and its competition with nucleon channels, and – at the same time – it stimulated further developments of the model. However – to the best of our knowledge – no spin formulation has been presented for cluster emission till recently, when the first attempts have been reported, but restricted to the first emission only. We have updated this effort now and we are able to handle (using the same simplifications as in our previous work pre-equilibrium cluster emission with spin including all nuclei in the reaction chain.

  12. Including spatial data in nutrient balance modelling on dairy farms

    Science.gov (United States)

    van Leeuwen, Maricke; van Middelaar, Corina; Stoof, Cathelijne; Oenema, Jouke; Stoorvogel, Jetse; de Boer, Imke

    2017-04-01

    The Annual Nutrient Cycle Assessment (ANCA) calculates the nitrogen (N) and phosphorus (P) balance at a dairy farm, while taking into account the subsequent nutrient cycles of the herd, manure, soil and crop components. Since January 2016, Dutch dairy farmers are required to use ANCA in order to increase understanding of nutrient flows and to minimize nutrient losses to the environment. A nutrient balance calculates the difference between nutrient inputs and outputs. Nutrients enter the farm via purchased feed, fertilizers, deposition and fixation by legumes (nitrogen), and leave the farm via milk, livestock, manure, and roughages. A positive balance indicates to which extent N and/or P are lost to the environment via gaseous emissions (N), leaching, run-off and accumulation in soil. A negative balance indicates that N and/or P are depleted from soil. ANCA was designed to calculate average nutrient flows on farm level (for the herd, manure, soil and crop components). ANCA was not designed to perform calculations of nutrient flows at the field level, as it uses averaged nutrient inputs and outputs across all fields, and it does not include field specific soil characteristics. Land management decisions, however, such as the level of N and P application, are typically taken at the field level given the specific crop and soil characteristics. Therefore the information that ANCA provides is likely not sufficient to support farmers' decisions on land management to minimize nutrient losses to the environment. This is particularly a problem when land management and soils vary between fields. For an accurate estimate of nutrient flows in a given farming system that can be used to optimize land management, the spatial scale of nutrient inputs and outputs (and thus the effect of land management and soil variation) could be essential. Our aim was to determine the effect of the spatial scale of nutrient inputs and outputs on modelled nutrient flows and nutrient use efficiencies

  13. The Model of the Software Running on a Computer Equipment Hardware Included in the Grid network

    Directory of Open Access Journals (Sweden)

    T. A. Mityushkina

    2012-12-01

    Full Text Available A new approach to building a cloud computing environment using Grid networks is proposed in this paper. The authors describe the functional capabilities, algorithm, model of software running on a computer equipment hardware included in the Grid network, that will allow to implement cloud computing environment using Grid technologies.

  14. A nonlinear model for fluid flow in a multiple-zone composite reservoir including the quadratic gradient term

    International Nuclear Information System (INIS)

    Wang, Xiao-Lu; Fan, Xiang-Yu; Nie, Ren-Shi; Huang, Quan-Hua; He, Yong-Ming

    2013-01-01

    Based on material balance and Darcy's law, the governing equation with the quadratic pressure gradient term was deduced. Then the nonlinear model for fluid flow in a multiple-zone composite reservoir including the quadratic gradient term was established and solved using a Laplace transform. A series of standard log–log type curves of 1-zone (homogeneous), 2-zone and 3-zone reservoirs were plotted and nonlinear flow characteristics were analysed. The type curves governed by the coefficient of the quadratic gradient term (β) gradually deviate from those of a linear model with time elapsing. Qualitative and quantitative analyses were implemented to compare the solutions of the linear and nonlinear models. The results showed that differences of pressure transients between the linear and nonlinear models increase with elapsed time and β. At the end, a successful application of the theoretical model data against the field data shows that the nonlinear model will be a good tool to evaluate formation parameters more accurately. (paper)

  15. [An early history of Japanese amyotrophic lateral sclerosis (ALS)-related diseases and the current development].

    Science.gov (United States)

    Abe, Koji

    2018-03-28

    The present review focuses an early history of Japanese amyotrophic lateral sclerosis (ALS)-related diseases and the current development. In relation to foreign previous reports, five topics are introduced and discussed on ALS with dementia, ALS/Parkinsonism dementia complex (ALS/PDC), familial ALS (FALS), spinal bulbar muscular atrophy (SBMA), and multisystem involvement especially in cerebellar system of ALS including ALS/SCA (spinocerebellar ataxia) crossroad mutation Asidan. This review found the great contribution of Japanese reports on the above five topics, and confirmed the great development of ALS-related diseases over the past 120 years.

  16. CFD simulations and reduced order modeling of a refrigerator compartment including radiation effects

    International Nuclear Information System (INIS)

    Bayer, Ozgur; Oskay, Ruknettin; Paksoy, Akin; Aradag, Selin

    2013-01-01

    Highlights: ► Free convection in a refrigerator is simulated including radiation effects. ► Heat rates are affected drastically when radiation effects are considered. ► 95% of the flow energy can be represented by using one spatial POD mode. - Abstract: Considering the engineering problem of natural convection in domestic refrigerator applications, this study aims to simulate the fluid flow and temperature distribution in a single commercial refrigerator compartment by using the experimentally determined temperature values as the specified constant wall temperature boundary conditions. The free convection in refrigerator applications is evaluated as a three-dimensional (3D), turbulent, transient and coupled non-linear flow problem. Radiation heat transfer mode is also included in the analysis. According to the results, taking radiation effects into consideration does not change the temperature distribution inside the refrigerator significantly; however the heat rates are affected drastically. The flow inside the compartment is further analyzed with a reduced order modeling method called Proper Orthogonal Decomposition (POD) and the energy contents of several spatial and temporal modes that exist in the flow are examined. The results show that approximately 95% of all the flow energy can be represented by only using one spatial mode

  17. Integrated Sachs-Wolfe effect in a quintessence cosmological model: Including anisotropic stress of dark energy

    International Nuclear Information System (INIS)

    Wang, Y. T.; Xu, L. X.; Gui, Y. X.

    2010-01-01

    In this paper, we investigate the integrated Sachs-Wolfe effect in the quintessence cold dark matter model with constant equation of state and constant speed of sound in dark energy rest frame, including dark energy perturbation and its anisotropic stress. Comparing with the ΛCDM model, we find that the integrated Sachs-Wolfe (ISW)-power spectrums are affected by different background evolutions and dark energy perturbation. As we change the speed of sound from 1 to 0 in the quintessence cold dark matter model with given state parameters, it is found that the inclusion of dark energy anisotropic stress makes the variation of magnitude of the ISW source uncertain due to the anticorrelation between the speed of sound and the ratio of dark energy density perturbation contrast to dark matter density perturbation contrast in the ISW-source term. Thus, the magnitude of the ISW-source term is governed by the competition between the alterant multiple of (1+3/2xc-circumflex s 2 ) and that of δ de /δ m with the variation of c-circumflex s 2 .

  18. Health Promotion Behavior of Chinese International Students in Korea Including Acculturation Factors: A Structural Equation Model.

    Science.gov (United States)

    Kim, Sun Jung; Yoo, Il Young

    2016-03-01

    The purpose of this study was to explain the health promotion behavior of Chinese international students in Korea using a structural equation model including acculturation factors. A survey using self-administered questionnaires was employed. Data were collected from 272 Chinese students who have resided in Korea for longer than 6 months. The data were analyzed using structural equation modeling. The p value of final model is .31. The fitness parameters of the final model such as goodness of fit index, adjusted goodness of fit index, normed fit index, non-normed fit index, and comparative fit index were more than .95. Root mean square of residual and root mean square error of approximation also met the criteria. Self-esteem, perceived health status, acculturative stress and acculturation level had direct effects on health promotion behavior of the participants and the model explained 30.0% of variance. The Chinese students in Korea with higher self-esteem, perceived health status, acculturation level, and lower acculturative stress reported higher health promotion behavior. The findings can be applied to develop health promotion strategies for this population. Copyright © 2016. Published by Elsevier B.V.

  19. Modelling the long-term consequences of a hypothetical dispersal of radioactivity in an urban area including remediation alternatives

    DEFF Research Database (Denmark)

    Thiessen, K.M.; Andersson, Kasper Grann; Batandjieva, B.

    2009-01-01

    The Urban Remediation Working Group of the International Atomic Energy Agency's EMRAS (Environmental Modelling for Radiation Safety) program was organized to address issues of remediation assessment modelling for urban areas contaminated with dispersed radionuclides. The present paper describes...... the second of two modelling exercises. This exercise was based on a hypothetical dispersal of radioactivity in an urban area from a radiological dispersal device, with reference surface contamination at selected sites used as the primary input information. Modelling endpoints for the exercise included...... radionuclide concentrations and external dose rates at specified locations, contributions to the dose rates from individual surfaces, and annual and cumulative external doses to specified reference individuals. Model predictions were performed for a "no action" situation (with no remedial measures...

  20. Transverse Crack Modeling and Validation in Rotor Systems, Including Thermal Effects

    Directory of Open Access Journals (Sweden)

    N. Bachschmid

    2003-01-01

    Full Text Available This article describes a model that allows the simulation of the static behavior of a transverse crack in a horizontal rotor under the action of weight and other possible static loads and the dynamic behavior of cracked rotating shaft. The crack breathes—that is, the mechanism of the crack's opening and closing is ruled by the stress on the cracked section exerted by the external loads. In a rotor, the stresses are time-dependent and have a period equal to the period of rotation; thus, the crack periodically breathes. An original, simplified model allows cracks of various shapes to be modeled and thermal stresses to be taken into account, as they may influence the opening and closing mechanism. The proposed method was validated by using two criteria. First the crack's breathing mechanism, simulated by the model, was compared with the results obtained by a nonlinear, threedimensional finite element model calculation, and a good agreement in the results was observed. Then the proposed model allowed the development of the equivalent cracked beam. The results of this model were compared with those obtained by the three-dimensional finite element model. Also in this case, there was a good agreement in the results.

  1. Modeling of in-vessel fission product release including fuel morphology effects for severe accident analyses

    International Nuclear Information System (INIS)

    Suh, K.Y.

    1989-10-01

    A new in-vessel fission product release model has been developed and implemented to perform best-estimate calculations of realistic source terms including fuel morphology effects. The proposed bulk mass transfer correlation determines the product of fission product release and equiaxed grain size as a function of the inverse fuel temperature. The model accounts for the fuel-cladding interaction over the temperature range between 770 K and 3000 K in the steam environment. A separate driver has been developed for the in-vessel thermal hydraulic and fission product behavior models that were developed by the Department of Energy for the Modular Accident Analysis Package (MAAP). Calculational results of these models have been compared to the results of the Power Burst Facility Severe Fuel Damage tests. The code predictions utilizing the mass transfer correlation agreed with the experimentally determined fractional release rates during the course of the heatup, power hold, and cooldown phases of the high temperature transients. Compared to such conventional literature correlations as the steam oxidation model and the NUREG-0956 correlation, the mass transfer correlation resulted in lower and less rapid releases in closer agreement with the on-line and grab sample data from the Severe Fuel Damage tests. The proposed mass transfer correlation can be applied for best-estimate calculations of fission products release from the UO 2 fuel in both nominal and severe accident conditions. 15 refs., 10 figs., 2 tabs

  2. Speech changes after coordinative training in patients with cerebellar ataxia: a pilot study

    Czech Academy of Sciences Publication Activity Database

    Tykalová, T.; Pospíšilová, M.; Čmejla, R.; Jeřábek, J.; Mareš, Pavel; Rusz, J.

    2016-01-01

    Roč. 37, č. 2 (2016), s. 293-296 ISSN 1590-1874 Institutional support: RVO:67985823 Keywords : spinocerebellar ataxia * rehabilitation * physiotherapy * ataxic dysarthria * postural alignment * acoustic analysis Subject RIV: FH - Neurology Impact factor: 1.749, year: 2016

  3. High performance computation of landscape genomic models including local indicators of spatial association.

    Science.gov (United States)

    Stucki, S; Orozco-terWengel, P; Forester, B R; Duruz, S; Colli, L; Masembe, C; Negrini, R; Landguth, E; Jones, M R; Bruford, M W; Taberlet, P; Joost, S

    2017-09-01

    With the increasing availability of both molecular and topo-climatic data, the main challenges facing landscape genomics - that is the combination of landscape ecology with population genomics - include processing large numbers of models and distinguishing between selection and demographic processes (e.g. population structure). Several methods address the latter, either by estimating a null model of population history or by simultaneously inferring environmental and demographic effects. Here we present samβada, an approach designed to study signatures of local adaptation, with special emphasis on high performance computing of large-scale genetic and environmental data sets. samβada identifies candidate loci using genotype-environment associations while also incorporating multivariate analyses to assess the effect of many environmental predictor variables. This enables the inclusion of explanatory variables representing population structure into the models to lower the occurrences of spurious genotype-environment associations. In addition, samβada calculates local indicators of spatial association for candidate loci to provide information on whether similar genotypes tend to cluster in space, which constitutes a useful indication of the possible kinship between individuals. To test the usefulness of this approach, we carried out a simulation study and analysed a data set from Ugandan cattle to detect signatures of local adaptation with samβada, bayenv, lfmm and an F ST outlier method (FDIST approach in arlequin) and compare their results. samβada - an open source software for Windows, Linux and Mac OS X available at http://lasig.epfl.ch/sambada - outperforms other approaches and better suits whole-genome sequence data processing. © 2016 The Authors. Molecular Ecology Resources Published by John Wiley & Sons Ltd.

  4. Validation of lumbar spine loading from a musculoskeletal model including the lower limbs and lumbar spine.

    Science.gov (United States)

    Actis, Jason A; Honegger, Jasmin D; Gates, Deanna H; Petrella, Anthony J; Nolasco, Luis A; Silverman, Anne K

    2018-02-08

    Low back mechanics are important to quantify to study injury, pain and disability. As in vivo forces are difficult to measure directly, modeling approaches are commonly used to estimate these forces. Validation of model estimates is critical to gain confidence in modeling results across populations of interest, such as people with lower-limb amputation. Motion capture, ground reaction force and electromyographic data were collected from ten participants without an amputation (five male/five female) and five participants with a unilateral transtibial amputation (four male/one female) during trunk-pelvis range of motion trials in flexion/extension, lateral bending and axial rotation. A musculoskeletal model with a detailed lumbar spine and the legs including 294 muscles was used to predict L4-L5 loading and muscle activations using static optimization. Model estimates of L4-L5 intervertebral joint loading were compared to measured intradiscal pressures from the literature and muscle activations were compared to electromyographic signals. Model loading estimates were only significantly different from experimental measurements during trunk extension for males without an amputation and for people with an amputation, which may suggest a greater portion of L4-L5 axial load transfer through the facet joints, as facet loads are not captured by intradiscal pressure transducers. Pressure estimates between the model and previous work were not significantly different for flexion, lateral bending or axial rotation. Timing of model-estimated muscle activations compared well with electromyographic activity of the lumbar paraspinals and upper erector spinae. Validated estimates of low back loading can increase the applicability of musculoskeletal models to clinical diagnosis and treatment. Copyright © 2017 Elsevier Ltd. All rights reserved.

  5. Hardware-in-the-loop vehicle system including dynamic fuel cell model

    Energy Technology Data Exchange (ETDEWEB)

    Lemes, Z.; Lenhart, T.; Braun, M.; Maencher, H. [MAGNUM Automatisierungstechnik GmbH, Darmstadt (Germany)

    2005-07-01

    In order to reduce costs and accelerate the development of fuel cells and systems the usage of hardware-in-the-loop (HIL) testing and dynamic modelling opens new possibilities. The dynamic model of a proton exchange membrane fuel cell (PEMFC) together with a vehicle model is used to carry out a comprehensive system investigation, which allows designing and optimising the behaviour of the components and the entire fuel cell system. The set-up of a HIL system enables real time interaction between the selected hardware and the model. (orig.)

  6. Unsteady free surface flow in porous media: One-dimensional model equations including vertical effects and seepage face

    Science.gov (United States)

    Di Nucci, Carmine

    2018-05-01

    This note examines the two-dimensional unsteady isothermal free surface flow of an incompressible fluid in a non-deformable, homogeneous, isotropic, and saturated porous medium (with zero recharge and neglecting capillary effects). Coupling a Boussinesq-type model for nonlinear water waves with Darcy's law, the two-dimensional flow problem is solved using one-dimensional model equations including vertical effects and seepage face. In order to take into account the seepage face development, the system equations (given by the continuity and momentum equations) are completed by an integral relation (deduced from the Cauchy theorem). After testing the model against data sets available in the literature, some numerical simulations, concerning the unsteady flow through a rectangular dam (with an impermeable horizontal bottom), are presented and discussed.

  7. Model for prognostication of population irradiation dose at the soil way of long-living radionuclides including in food chains

    International Nuclear Information System (INIS)

    Prister, B.S.; Vinogradskaya, V.D.

    2009-01-01

    On the basis of modern pictures of cesium and strontium ion absorption mechanisms a soil taking complex was build the kinetic model of radionuclide migration from soil to plants. Model parameter association with the agricultural chemistry properties of soil, represented by complex estimation of soil properties S e f. The example of model application for prognostication of population internal irradiation dose due to consumption of milk at the soil way of long-living radionuclides including in food chains

  8. SCA28

    DEFF Research Database (Denmark)

    Svenstrup, Kirsten; Nielsen, Troels Tolstrup; Aidt, Frederik

    2017-01-01

    The spinocerebellar ataxias (SCA) are a group of rare inherited neurodegenerative diseases characterized by slowly progressive cerebellar ataxia, resulting in unsteady gait, clumsiness, and dysarthria. The disorders are predominantly inherited in an autosomal dominant manner. Mutations in the gen...

  9. Extending PSA models including ageing and asset management - 15291

    International Nuclear Information System (INIS)

    Martorell, S.; Marton, I.; Carlos, S.; Sanchez, A.I.

    2015-01-01

    This paper proposes a new approach to Ageing Probabilistic Safety Assessment (APSA) modelling, which is intended to be used to support risk-informed decisions on the effectiveness of maintenance management programs and technical specification requirements of critical equipment of Nuclear Power Plants (NPP) within the framework of the Risk Informed Decision Making according to R.G. 1.174 principles. This approach focuses on the incorporation of not only equipment ageing but also effectiveness of maintenance and efficiency of surveillance testing explicitly into APSA models and data. This methodology is applied to a motor-operated valve of the auxiliary feed water system (AFWS) of a PWR. This simple example of application focuses on a critical safety-related equipment of a NPP in order to evaluate the risk impact of considering different approaches to APSA and the combined effect of equipment ageing and maintenance and testing alternatives along NPP design life. The risk impact of several alternatives in maintenance strategy is discussed

  10. Modelling the long-term consequences of a hypothetical dispersal of radioactivity in an urban area including remediation alternatives

    International Nuclear Information System (INIS)

    Thiessen, K.M.; Andersson, K.G.; Batandjieva, B.; Cheng, J.-J.; Hwang, W.T.; Kaiser, J.C.; Kamboj, S.; Steiner, M.; Tomas, J.; Trifunovic, D.; Yu, C.

    2009-01-01

    The Urban Remediation Working Group of the International Atomic Energy Agency's EMRAS (Environmental Modelling for Radiation Safety) program was organized to address issues of remediation assessment modelling for urban areas contaminated with dispersed radionuclides. The present paper describes the second of two modelling exercises. This exercise was based on a hypothetical dispersal of radioactivity in an urban area from a radiological dispersal device, with reference surface contamination at selected sites used as the primary input information. Modelling endpoints for the exercise included radionuclide concentrations and external dose rates at specified locations, contributions to the dose rates from individual surfaces, and annual and cumulative external doses to specified reference individuals. Model predictions were performed for a 'no action' situation (with no remedial measures) and for selected countermeasures. The exercise provided an opportunity for comparison of three modelling approaches, as well as a comparison of the predicted effectiveness of various countermeasures in terms of their short-term and long-term effects on predicted doses to humans.

  11. Time-domain simulation of constitutive relations for nonlinear acoustics including relaxation for frequency power law attenuation media modeling

    Science.gov (United States)

    Jiménez, Noé; Camarena, Francisco; Redondo, Javier; Sánchez-Morcillo, Víctor; Konofagou, Elisa E.

    2015-10-01

    We report a numerical method for solving the constitutive relations of nonlinear acoustics, where multiple relaxation processes are included in a generalized formulation that allows the time-domain numerical solution by an explicit finite differences scheme. Thus, the proposed physical model overcomes the limitations of the one-way Khokhlov-Zabolotskaya-Kuznetsov (KZK) type models and, due to the Lagrangian density is implicitly included in the calculation, the proposed method also overcomes the limitations of Westervelt equation in complex configurations for medical ultrasound. In order to model frequency power law attenuation and dispersion, such as observed in biological media, the relaxation parameters are fitted to both exact frequency power law attenuation/dispersion media and also empirically measured attenuation of a variety of tissues that does not fit an exact power law. Finally, a computational technique based on artificial relaxation is included to correct the non-negligible numerical dispersion of the finite difference scheme, and, on the other hand, improve stability trough artificial attenuation when shock waves are present. This technique avoids the use of high-order finite-differences schemes leading to fast calculations. The present algorithm is especially suited for practical configuration where spatial discontinuities are present in the domain (e.g. axisymmetric domains or zero normal velocity boundary conditions in general). The accuracy of the method is discussed by comparing the proposed simulation solutions to one dimensional analytical and k-space numerical solutions.

  12. Identifying Clusters with Mixture Models that Include Radial Velocity Observations

    Science.gov (United States)

    Czarnatowicz, Alexis; Ybarra, Jason E.

    2018-01-01

    The study of stellar clusters plays an integral role in the study of star formation. We present a cluster mixture model that considers radial velocity data in addition to spatial data. Maximum likelihood estimation through the Expectation-Maximization (EM) algorithm is used for parameter estimation. Our mixture model analysis can be used to distinguish adjacent or overlapping clusters, and estimate properties for each cluster.Work supported by awards from the Virginia Foundation for Independent Colleges (VFIC) Undergraduate Science Research Fellowship and The Research Experience @Bridgewater (TREB).

  13. Analytical and numerical modelling of thermoviscous shocks in their interactions in nonlinear fluids including dissipation

    DEFF Research Database (Denmark)

    Rasmussen, Anders Rønne; Sørensen, Mads Peter; Gaididei, Yuri Borisovich

    2010-01-01

    A wave equation, that governs finite amplitude acoustic disturbances in a thermoviscous Newtonian fluid, and includes nonlinear terms up to second order, is proposed. The equation preserves the Hamiltonian structure of the fundamental fluid dynamical equations in the non dissipative limit. An exact...... thermoviscous shock solution is derived. This solution is, in an overall sense, equivalent to the Taylor shock solution of the Burgers equation. However, in contrast to the Burgers equation, the model equation considered here is capable to describe waves propagating in opposite directions. Studies of head...

  14. Analytical linear energy transfer model including secondary particles: calculations along the central axis of the proton pencil beam

    International Nuclear Information System (INIS)

    Marsolat, F; De Marzi, L; Mazal, A; Pouzoulet, F

    2016-01-01

    In proton therapy, the relative biological effectiveness (RBE) depends on various types of parameters such as linear energy transfer (LET). An analytical model for LET calculation exists (Wilkens’ model), but secondary particles are not included in this model. In the present study, we propose a correction factor, L sec , for Wilkens’ model in order to take into account the LET contributions of certain secondary particles. This study includes secondary protons and deuterons, since the effects of these two types of particles can be described by the same RBE-LET relationship. L sec was evaluated by Monte Carlo (MC) simulations using the GATE/GEANT4 platform and was defined by the ratio of the LET d distributions of all protons and deuterons and only primary protons. This method was applied to the innovative Pencil Beam Scanning (PBS) delivery systems and L sec was evaluated along the beam axis. This correction factor indicates the high contribution of secondary particles in the entrance region, with L sec values higher than 1.6 for a 220 MeV clinical pencil beam. MC simulations showed the impact of pencil beam parameters, such as mean initial energy, spot size, and depth in water, on L sec . The variation of L sec with these different parameters was integrated in a polynomial function of the L sec factor in order to obtain a model universally applicable to all PBS delivery systems. The validity of this correction factor applied to Wilkens’ model was verified along the beam axis of various pencil beams in comparison with MC simulations. A good agreement was obtained between the corrected analytical model and the MC calculations, with mean-LET deviations along the beam axis less than 0.05 keV μm −1 . These results demonstrate the efficacy of our new correction of the existing LET model in order to take into account secondary protons and deuterons along the pencil beam axis. (paper)

  15. Exergoeconomic performance optimization for a steady-flow endoreversible refrigeration model including six typical cycles

    Energy Technology Data Exchange (ETDEWEB)

    Chen, Lingen; Kan, Xuxian; Sun, Fengrui; Wu, Feng [College of Naval Architecture and Power, Naval University of Engineering, Wuhan 430033 (China)

    2013-07-01

    The operation of a universal steady flow endoreversible refrigeration cycle model consisting of a constant thermal-capacity heating branch, two constant thermal-capacity cooling branches and two adiabatic branches is viewed as a production process with exergy as its output. The finite time exergoeconomic performance optimization of the refrigeration cycle is investigated by taking profit rate optimization criterion as the objective. The relations between the profit rate and the temperature ratio of working fluid, between the COP (coefficient of performance) and the temperature ratio of working fluid, as well as the optimal relation between profit rate and the COP of the cycle are derived. The focus of this paper is to search the compromised optimization between economics (profit rate) and the utilization factor (COP) for endoreversible refrigeration cycles, by searching the optimum COP at maximum profit, which is termed as the finite-time exergoeconomic performance bound. Moreover, performance analysis and optimization of the model are carried out in order to investigate the effect of cycle process on the performance of the cycles using numerical example. The results obtained herein include the performance characteristics of endoreversible Carnot, Diesel, Otto, Atkinson, Dual and Brayton refrigeration cycles.

  16. A panel study on patients with dominant cerebellar ataxia highlights the frequency of channelopathies.

    Science.gov (United States)

    Coutelier, Marie; Coarelli, Giulia; Monin, Marie-Lorraine; Konop, Juliette; Davoine, Claire-Sophie; Tesson, Christelle; Valter, Rémi; Anheim, Mathieu; Behin, Anthony; Castelnovo, Giovanni; Charles, Perrine; David, Albert; Ewenczyk, Claire; Fradin, Mélanie; Goizet, Cyril; Hannequin, Didier; Labauge, Pierre; Riant, Florence; Sarda, Pierre; Sznajer, Yves; Tison, François; Ullmann, Urielle; Van Maldergem, Lionel; Mochel, Fanny; Brice, Alexis; Stevanin, Giovanni; Durr, Alexandra

    2017-06-01

    Autosomal dominant cerebellar ataxias have a marked heterogeneous genetic background, with mutations in 34 genes identified so far. This large amount of implicated genes accounts for heterogeneous clinical presentations, making genotype-phenotype correlations a major challenge in the field. While polyglutamine ataxias, linked to CAG repeat expansions in genes such as ATXN1, ATXN2, ATXN3, ATXN7, CACNA1A and TBP, have been extensively characterized in large cohorts, there is a need for comprehensive assessment of frequency and phenotype of more 'conventional' ataxias. After exclusion of CAG/polyglutamine expansions in spinocerebellar ataxia genes in 412 index cases with dominantly inherited cerebellar ataxias, we aimed to establish the relative frequencies of mutations in other genes, with an approach combining panel sequencing and TaqMan® polymerase chain reaction assay. We found relevant genetic variants in 59 patients (14.3%). The most frequently mutated were channel genes [CACNA1A (n = 16), KCND3 (n = 4), KCNC3 (n = 2) and KCNA1 (n = 2)]. Deletions in ITPR1 (n = 11) were followed by biallelic variants in SPG7 (n = 9). Variants in AFG3L2 (n = 7) came next in frequency, and variants were rarely found in STBN2 (n = 2), ELOVL5, FGF14, STUB1 and TTBK2 (n = 1 each). Interestingly, possible risk factor variants were detected in SPG7 and POLG. Clinical comparisons showed that ataxias due to channelopathies had a significantly earlier age at onset with an average of 24.6 years, versus 40.9 years for polyglutamine expansion spinocerebellar ataxias and 37.8 years for SPG7-related forms (P = 0.001). In contrast, disease duration was significantly longer in the former (20.5 years versus 9.3 and 13.7, P=0.001), though for similar functional stages, indicating slower progression of the disease. Of interest, intellectual deficiency was more frequent in channel spinocerebellar ataxias, while cognitive impairment in adulthood was similar among the three groups. Similar

  17. Transverse Crack Modeling and Validation in Rotor Systems Including Thermal Effects

    Directory of Open Access Journals (Sweden)

    N. Bachschmid

    2004-01-01

    Full Text Available In this article, a model is described that allows one to simulate the static behavior of a transversal crack in a horizontal rotor, under the action of the weight and other possible static loads and the dynamical behavior of the rotating cracked shaft. The crack “breaths,” i.e., the mechanism of opening and closing of the crack, is ruled by the stress acting on the cracked section due to the external loads; in a rotor the stress is time-depending with a period equal to the period of rotation, thus the crack “periodically breaths.” An original simplified model is described that allows cracks of different shape to be modeled and thermal stresses to be taken into account, since they may influence the opening and closing mechanism. The proposed method has been validated using two criteria. Firstly, the crack “breathing” mechanism, simulated with the model, has been compared with the results obtained by a nonlinear 3-D FEM calculation and a good agreement in the results has been observed. Secondly, the proposed model allows the development of the equivalent cracked beam. The results of this model are compared with those obtained by the above-mentioned 3-D FEM. There is a good agreement in the results, of this case as well.

  18. Including irrigation in niche modelling of the invasive wasp Vespula germanica (Fabricius) improves model fit to predict potential for further spread.

    Science.gov (United States)

    de Villiers, Marelize; Kriticos, Darren J; Veldtman, Ruan

    2017-01-01

    The European wasp, Vespula germanica (Fabricius) (Hymenoptera: Vespidae), is of Palaearctic origin, being native to Europe, northern Africa and Asia, and introduced into North America, Chile, Argentina, Iceland, Ascension Island, South Africa, Australia and New Zealand. Due to its polyphagous nature and scavenging behaviour, V. germanica threatens agriculture and silviculture, and negatively affects biodiversity, while its aggressive nature and venomous sting pose a health risk to humans. In areas with warmer winters and longer summers, queens and workers can survive the winter months, leading to the build-up of large nests during the following season; thereby increasing the risk posed by this species. To prevent or prepare for such unwanted impacts it is important to know where the wasp may be able to establish, either through natural spread or through introduction as a result of human transport. Distribution data from Argentina and Australia, and seasonal phenology data from Argentina were used to determine the potential distribution of V. germanica using CLIMEX modelling. In contrast to previous models, the influence of irrigation on its distribution was also investigated. Under a natural rainfall scenario, the model showed similarities to previous models. When irrigation is applied, dry stress is alleviated, leading to larger areas modelled climatically suitable compared with previous models, which provided a better fit with the actual distribution of the species. The main areas at risk of invasion by V. germanica include western USA, Mexico, small areas in Central America and in the north-western region of South America, eastern Brazil, western Russia, north-western China, Japan, the Mediterranean coastal regions of North Africa, and parts of southern and eastern Africa.

  19. Including irrigation in niche modelling of the invasive wasp Vespula germanica (Fabricius improves model fit to predict potential for further spread.

    Directory of Open Access Journals (Sweden)

    Marelize de Villiers

    Full Text Available The European wasp, Vespula germanica (Fabricius (Hymenoptera: Vespidae, is of Palaearctic origin, being native to Europe, northern Africa and Asia, and introduced into North America, Chile, Argentina, Iceland, Ascension Island, South Africa, Australia and New Zealand. Due to its polyphagous nature and scavenging behaviour, V. germanica threatens agriculture and silviculture, and negatively affects biodiversity, while its aggressive nature and venomous sting pose a health risk to humans. In areas with warmer winters and longer summers, queens and workers can survive the winter months, leading to the build-up of large nests during the following season; thereby increasing the risk posed by this species. To prevent or prepare for such unwanted impacts it is important to know where the wasp may be able to establish, either through natural spread or through introduction as a result of human transport. Distribution data from Argentina and Australia, and seasonal phenology data from Argentina were used to determine the potential distribution of V. germanica using CLIMEX modelling. In contrast to previous models, the influence of irrigation on its distribution was also investigated. Under a natural rainfall scenario, the model showed similarities to previous models. When irrigation is applied, dry stress is alleviated, leading to larger areas modelled climatically suitable compared with previous models, which provided a better fit with the actual distribution of the species. The main areas at risk of invasion by V. germanica include western USA, Mexico, small areas in Central America and in the north-western region of South America, eastern Brazil, western Russia, north-western China, Japan, the Mediterranean coastal regions of North Africa, and parts of southern and eastern Africa.

  20. Finite Element Modeling and Analysis of Nonlinear Impact and Frictional Motion Responses Including Fluid—Structure Coupling Effects

    Directory of Open Access Journals (Sweden)

    Yong Zhao

    1997-01-01

    Full Text Available A nonlinear three dimensional (3D single rack model and a nonlinear 3D whole pool multi-rack model are developed for the spent fuel storage racks of a nuclear power plant (NPP to determine impacts and frictional motion responses when subjected to 3D excitations from the supporting building floor. The submerged free standing rack system and surrounding water are coupled due to hydrodynamic fluid-structure interaction (FSI using potential theory. The models developed have features that allow consideration of geometric and material nonlinearities including (1 the impacts of fuel assemblies to rack cells, a rack to adjacent racks or pool walls, and rack support legs to the pool floor; (2 the hydrodynamic coupling of fuel assemblies with their storing racks, and of a rack with adjacent racks, pool walls, and the pool floor; and (3 the dynamic motion behavior of rocking, twisting, and frictional sliding of rack modules. Using these models 3D nonlinear time history dynamic analyses are performed per the U.S. Nuclear Regulatory Commission (USNRC criteria. Since few such modeling, analyses, and results using both the 3D single and whole pool multiple rack models are available in the literature, this paper emphasizes description of modeling and analysis techniques using the SOLVIA general purpose nonlinear finite element code. Typical response results with different Coulomb friction coefficients are presented and discussed.

  1. HPTAM, a two-dimensional Heat Pipe Transient Analysis Model, including the startup from a frozen state

    Science.gov (United States)

    Tournier, Jean-Michel; El-Genk, Mohamed S.

    1995-01-01

    A two-dimensional Heat Pipe Transient Analysis Model, 'HPTAM,' was developed to simulate the transient operation of fully-thawed heat pipes and the startup of heat pipes from a frozen state. The model incorporates: (a) sublimation and resolidification of working fluid; (b) melting and freezing of the working fluid in the porous wick; (c) evaporation of thawed working fluid and condensation as a thin liquid film on a frozen substrate; (d) free-molecule, transition, and continuum vapor flow regimes, using the Dusty Gas Model; (e) liquid flow and heat transfer in the porous wick; and (f) thermal and hydrodynamic couplings of phases at their respective interfaces. HPTAM predicts the radius of curvature of the liquid meniscus at the liquid-vapor interface and the radial location of the working fluid level (liquid or solid) in the wick. It also includes the transverse momentum jump condition (capillary relationship of Pascal) at the liquid-vapor interface and geometrically relates the radius of curvature of the liquid meniscus to the volume fraction of vapor in the wick. The present model predicts the capillary limit and partial liquid recess (dryout) in the evaporator wick, and incorporates a liquid pooling submodel, which simulates accumulation of the excess liquid in the vapor core at the condenser end.

  2. Microstructure modeling and crystal plasticity simulations for the evaluation of fatigue crack initiation in α-iron specimen including an elliptic defect

    Energy Technology Data Exchange (ETDEWEB)

    Briffod, Fabien, E-mail: briffod@rme.mm.t.u-tokyo.ac.jp; Shiraiwa, Takayuki; Enoki, Manabu

    2017-05-17

    In this study, fatigue crack initiation in pure α-iron is investigated through a microstructure-sensitive framework. At first, synthetic microstructures are modeled based on an anisotropic tessellation that accounts for the information of the grains morphology extracted from electron backscatter diffraction (EBSD) analysis. Low-cycle fatigue experiments under strain-controlled conditions are conducted in order to calibrate a crystal plasticity model and a J{sub 2} model including isotropic and kinematic hardening. A critical plane fatigue indicator parameter (FIP) based on the Tanaka-Mura model is then presented to evaluate the location and quantify the driving force for the formation of a crack. The FIP is averaged over several potential crack paths within each grain defined by the intersection between a given slip plane and the plane of the model thus accounting for both the lattice orientation and morphology of the grain. Several fatigue simulations at various stress amplitudes are conducted using a sub-modeling technique for the attribution of boundary conditions on the polycrystalline aggregate models including an elliptic defect. The influence of the microstructure attributes and stress level on the location and amplitude of the FIP are then quantified and discussed.

  3. NASTRAN thermal analyzer: Theory and application including a guide to modeling engineering problems, volume 1. [thermal analyzer manual

    Science.gov (United States)

    Lee, H. P.

    1977-01-01

    The NASTRAN Thermal Analyzer Manual describes the fundamental and theoretical treatment of the finite element method, with emphasis on the derivations of the constituent matrices of different elements and solution algorithms. Necessary information and data relating to the practical applications of engineering modeling are included.

  4. Tracer experiment data sets for the verification of local and meso-scale atmospheric dispersion models including topographic effects

    International Nuclear Information System (INIS)

    Sartori, E.; Schuler, W.

    1992-01-01

    Software and data for nuclear energy applications are acquired, tested and distributed by several information centres; in particular, relevant computer codes are distributed internationally by the OECD/NEA Data Bank (France) and by ESTSC and EPIC/RSIC (United States). This activity is coordinated among the centres and is extended outside the OECD area through an arrangement with the IAEA. This article proposes more specifically a scheme for acquiring, storing and distributing atmospheric tracer experiment data (ATE) required for verification of atmospheric dispersion models especially the most advanced ones including topographic effects and specific to the local and meso-scale. These well documented data sets will form a valuable complement to the set of atmospheric dispersion computer codes distributed internationally. Modellers will be able to gain confidence in the predictive power of their models or to verify their modelling skills. (au)

  5. Deterministic Model for Rubber-Metal Contact Including the Interaction Between Asperities

    NARCIS (Netherlands)

    Deladi, E.L.; de Rooij, M.B.; Schipper, D.J.

    2005-01-01

    Rubber-metal contact involves relatively large deformations and large real contact areas compared to metal-metal contact. Here, a deterministic model is proposed for the contact between rubber and metal surfaces, which takes into account the interaction between neighboring asperities. In this model,

  6. Kinetic modelling of anaerobic hydrolysis of solid wastes, including disintegration processes

    Energy Technology Data Exchange (ETDEWEB)

    García-Gen, Santiago [Department of Chemical Engineering, Institute of Technology, University of Santiago de Compostela, 15782 Santiago de Compostela (Spain); Sousbie, Philippe; Rangaraj, Ganesh [INRA, UR50, Laboratoire de Biotechnologie de l’Environnement, Avenue des Etangs, Narbonne F-11100 (France); Lema, Juan M. [Department of Chemical Engineering, Institute of Technology, University of Santiago de Compostela, 15782 Santiago de Compostela (Spain); Rodríguez, Jorge, E-mail: jrodriguez@masdar.ac.ae [Department of Chemical Engineering, Institute of Technology, University of Santiago de Compostela, 15782 Santiago de Compostela (Spain); Institute Centre for Water and Environment (iWater), Masdar Institute of Science and Technology, PO Box 54224 Abu Dhabi (United Arab Emirates); Steyer, Jean-Philippe; Torrijos, Michel [INRA, UR50, Laboratoire de Biotechnologie de l’Environnement, Avenue des Etangs, Narbonne F-11100 (France)

    2015-01-15

    Highlights: • Fractionation of solid wastes into readily and slowly biodegradable fractions. • Kinetic coefficients estimation from mono-digestion batch assays. • Validation of kinetic coefficients with a co-digestion continuous experiment. • Simulation of batch and continuous experiments with an ADM1-based model. - Abstract: A methodology to estimate disintegration and hydrolysis kinetic parameters of solid wastes and validate an ADM1-based anaerobic co-digestion model is presented. Kinetic parameters of the model were calibrated from batch reactor experiments treating individually fruit and vegetable wastes (among other residues) following a new protocol for batch tests. In addition, decoupled disintegration kinetics for readily and slowly biodegradable fractions of solid wastes was considered. Calibrated parameters from batch assays of individual substrates were used to validate the model for a semi-continuous co-digestion operation treating simultaneously 5 fruit and vegetable wastes. The semi-continuous experiment was carried out in a lab-scale CSTR reactor for 15 weeks at organic loading rate ranging between 2.0 and 4.7 g VS/L d. The model (built in Matlab/Simulink) fit to a large extent the experimental results in both batch and semi-continuous mode and served as a powerful tool to simulate the digestion or co-digestion of solid wastes.

  7. Cascade Controller Including Back-stepping for Hydraulic-Mechanical Systems

    DEFF Research Database (Denmark)

    Choux, Martin; Hovland, Geir; Blanke, Mogens

    2012-01-01

    Development of a cascade controller structure including adaptive backstepping for a nonlinear hydraulic-mechanical system is considered in this paper where a dynamic friction (LuGre) model is included to obtain the necessary accuracy. The paper compares the performance of two variants of an adapt......Development of a cascade controller structure including adaptive backstepping for a nonlinear hydraulic-mechanical system is considered in this paper where a dynamic friction (LuGre) model is included to obtain the necessary accuracy. The paper compares the performance of two variants...... of an adaptive backstepping tracking controller with earlier results. The new control architecture is analysed and enhanced tracking performance is demonstrated when including the extended friction model. The complexity of the backstepping procedure is significantly reduced due to the cascade structure. Hence...

  8. Molecular Modeling of Aerospace Polymer Matrices Including Carbon Nanotube-Enhanced Epoxy

    Science.gov (United States)

    Radue, Matthew S.

    Carbon fiber (CF) composites are increasingly replacing metals used in major structural parts of aircraft, spacecraft, and automobiles. The current limitations of carbon fiber composites are addressed through computational material design by modeling the salient aerospace matrix materials. Molecular Dynamics (MD) models of epoxies with and without carbon nanotube (CNT) reinforcement and models of pure bismaleimides (BMIs) were developed to elucidate structure-property relationships for improved selection and tailoring of matrices. The influence of monomer functionality on the mechanical properties of epoxies is studied using the Reax Force Field (ReaxFF). From deformation simulations, the Young's modulus, yield point, and Poisson's ratio are calculated and analyzed. The results demonstrate an increase in stiffness and yield strength with increasing resin functionality. Comparison between the network structures of distinct epoxies is further advanced by the Monomeric Degree Index (MDI). Experimental validation demonstrates the MD results correctly predict the relationship in Young's moduli for all epoxies modeled. Therefore, the ReaxFF is confirmed to be a useful tool for studying the mechanical behavior of epoxies. While epoxies have been well-studied using MD, there has been no concerted effort to model cured BMI polymers due to the complexity of the network-forming reactions. A novel, adaptable crosslinking framework is developed for implementing 5 distinct cure reactions of Matrimid-5292 (a BMI resin) and investigating the network structure using MD simulations. The influence of different cure reactions and extent of curing are analyzed on the several thermo-mechanical properties such as mass density, glass transition temperature, coefficient of thermal expansion, elastic moduli, and thermal conductivity. The developed crosslinked models correctly predict experimentally observed trends for various properties. Finally, the epoxies modeled (di-, tri-, and tetra

  9. A void ratio dependent water retention curve model including hydraulic hysteresis

    Directory of Open Access Journals (Sweden)

    Pasha Amin Y.

    2016-01-01

    Full Text Available Past experimental evidence has shown that Water Retention Curve (WRC evolves with mechanical stress and structural changes in soil matrix. Models currently available in the literature for capturing the volume change dependency of WRC are mainly empirical in nature requiring an extensive experimental programme for parameter identification which renders them unsuitable for practical applications. In this paper, an analytical model for the evaluation of the void ratio dependency of WRC in deformable porous media is presented. The approach proposed enables quantification of the dependency of WRC on void ratio solely based on the form of WRC at the reference void ratio and requires no additional parameters. The effect of hydraulic hysteresis on the evolution process is also incorporated in the model, an aspect rarely addressed in the literature. Expressions are presented for the evolution of main and scanning curves due to loading and change in the hydraulic path from scanning to main wetting/drying and vice versa as well as the WRC parameters such as air entry value, air expulsion value, pore size distribution index and slope of the scanning curve. The model is validated using experimental data on compacted and reconstituted soils subjected to various hydro-mechanical paths. Good agreement is obtained between model predictions and experimental data in all the cases considered.

  10. Development of numerical dispersion model for radioactive nuclei including resuspension processes

    International Nuclear Information System (INIS)

    Chiba, Masaru; Kurita, Susumu; Sasaki, Hidetaka

    2003-01-01

    Global-scale and local-scale dispersion model are developed combining to global and local scale meteorological forecasting model. By applying this system to another miner constituent such as mineral dust blowing by strong wind in arid region, this system shows very good performance to watch and predict the distribution of it. (author)

  11. A surplus production model including environmental effects: Application to the Senegalese white shrimp stocks

    Science.gov (United States)

    Thiaw, Modou; Gascuel, Didier; Jouffre, Didier; Thiaw, Omar Thiom

    2009-12-01

    In Senegal, two stocks of white shrimp ( Penaeusnotialis) are intensively exploited, one in the north and another in the south. We used surplus production models including environmental effects to analyse their changes in abundance over the past 10 years and to estimate their Maximum Sustainable Yield (MSY) and the related fishing effort ( EMSY). First, yearly abundance indices were estimated from commercial statistics using GLM techniques. Then, two environmental indices were alternatively tested in the model: the coastal upwelling intensity from wind speeds provided by the SeaWifs database and the primary production derived from satellite infrared images of chlorophyll a. Models were fitted, with or without the environmental effect, to the 1996-2005 time series. They express stock abundance and catches as functions of the fishing effort and the environmental index (when considered). For the northern stock, fishing effort and abundance fluctuate over the period without any clear trends. The model based on the upwelling index explains 64.9% of the year-to-year variability. It shows that the stock was slightly overexploited in 2002-2003 and is now close to full exploitation. Stock abundance strongly depends on environmental conditions; consequently, the MSY estimate varies from 300 to 900 tons according to the upwelling intensity. For the southern stock, fishing effort has strongly increased over the past 10 years, while abundance has been reduced 4-fold. The environment has a significant effect on abundance but only explains a small part of the year-to-year variability. The best fit is obtained using the primary production index ( R2 = 0.75), and the stock is now significantly overfished regardless of environmental conditions. MSY varies from 1200 to 1800 tons according to environmental conditions. Finally, in northern Senegal, the upwelling is highly variable from year to year and constitutes the major factor determining productivity. In the south, hydrodynamic

  12. A statistical model for estimation of fish density including correlation in size, space, time and between species from research survey data

    DEFF Research Database (Denmark)

    Nielsen, J. Rasmus; Kristensen, Kasper; Lewy, Peter

    2014-01-01

    Trawl survey data with high spatial and seasonal coverage were analysed using a variant of the Log Gaussian Cox Process (LGCP) statistical model to estimate unbiased relative fish densities. The model estimates correlations between observations according to time, space, and fish size and includes...

  13. Static aeroelastic analysis including geometric nonlinearities based on reduced order model

    Directory of Open Access Journals (Sweden)

    Changchuan Xie

    2017-04-01

    Full Text Available This paper describes a method proposed for modeling large deflection of aircraft in nonlinear aeroelastic analysis by developing reduced order model (ROM. The method is applied for solving the static aeroelastic and static aeroelastic trim problems of flexible aircraft containing geometric nonlinearities; meanwhile, the non-planar effects of aerodynamics and follower force effect have been considered. ROMs are computational inexpensive mathematical representations compared to traditional nonlinear finite element method (FEM especially in aeroelastic solutions. The approach for structure modeling presented here is on the basis of combined modal/finite element (MFE method that characterizes the stiffness nonlinearities and we apply that structure modeling method as ROM to aeroelastic analysis. Moreover, the non-planar aerodynamic force is computed by the non-planar vortex lattice method (VLM. Structure and aerodynamics can be coupled with the surface spline method. The results show that both of the static aeroelastic analysis and trim analysis of aircraft based on structure ROM can achieve a good agreement compared to analysis based on the FEM and experimental result.

  14. Standardized Competencies for Parenteral Nutrition Order Review and Parenteral Nutrition Preparation, Including Compounding: The ASPEN Model.

    Science.gov (United States)

    Boullata, Joseph I; Holcombe, Beverly; Sacks, Gordon; Gervasio, Jane; Adams, Stephen C; Christensen, Michael; Durfee, Sharon; Ayers, Phil; Marshall, Neil; Guenter, Peggi

    2016-08-01

    Parenteral nutrition (PN) is a high-alert medication with a complex drug use process. Key steps in the process include the review of each PN prescription followed by the preparation of the formulation. The preparation step includes compounding the PN or activating a standardized commercially available PN product. The verification and review, as well as preparation of this complex therapy, require competency that may be determined by using a standardized process for pharmacists and for pharmacy technicians involved with PN. An American Society for Parenteral and Enteral Nutrition (ASPEN) standardized model for PN order review and PN preparation competencies is proposed based on a competency framework, the ASPEN-published interdisciplinary core competencies, safe practice recommendations, and clinical guidelines, and is intended for institutions and agencies to use with their staff. © 2016 American Society for Parenteral and Enteral Nutrition.

  15. Localized Cerebral Energy Failure in DNA Polymerase Gamma-Associated Encephalopathy Syndromes

    Science.gov (United States)

    Tzoulis, Charalampos; Neckelmann, Gesche; Mork, Sverre J.; Engelsen, Bernt E.; Viscomi, Carlo; Moen, Gunnar; Ersland, Lars; Zeviani, Massimo; Bindoff, Laurence A.

    2010-01-01

    Mutations in the catalytic subunit of the mitochondrial DNA-polymerase gamma cause a wide spectrum of clinical disease ranging from infantile hepato-encephalopathy to juvenile/adult-onset spinocerebellar ataxia and late onset progressive external ophthalmoplegia. Several of these syndromes are associated with an encephalopathy that…

  16. Paula Coutinho's outstanding contribution to the definition of Machado-Joseph disease.

    Science.gov (United States)

    Garcia, Bruno Carniatto Marques; Germiniani, Francisco Manoel Branco; Marques, Paula; Sequeiros, Jorge; Teive, Hélio Afonso Ghizoni

    2017-10-01

    Machado-Joseph disease, also known as spinocerebellar ataxia type 3, is the most common form of autosomal dominant ataxia in the world. Paula Coutinho, a highly-regarded Portuguese neurologist worldwide, had a seminal participation in the definition of this disease, more than 40 years ago.

  17. Mathematical modeling of HIV prevention measures including pre-exposure prophylaxis on HIV incidence in South Korea.

    Science.gov (United States)

    Kim, Sun Bean; Yoon, Myoungho; Ku, Nam Su; Kim, Min Hyung; Song, Je Eun; Ahn, Jin Young; Jeong, Su Jin; Kim, Changsoo; Kwon, Hee-Dae; Lee, Jeehyun; Smith, Davey M; Choi, Jun Yong

    2014-01-01

    Multiple prevention measures have the possibility of impacting HIV incidence in South Korea, including early diagnosis, early treatment, and pre-exposure prophylaxis (PrEP). We investigated how each of these interventions could impact the local HIV epidemic, especially among men who have sex with men (MSM), who have become the major risk group in South Korea. A mathematical model was used to estimate the effects of each these interventions on the HIV epidemic in South Korea over the next 40 years, as compared to the current situation. We constructed a mathematical model of HIV infection among MSM in South Korea, dividing the MSM population into seven groups, and simulated the effects of early antiretroviral therapy (ART), early diagnosis, PrEP, and combination interventions on the incidence and prevalence of HIV infection, as compared to the current situation that would be expected without any new prevention measures. Overall, the model suggested that the most effective prevention measure would be PrEP. Even though PrEP effectiveness could be lessened by increased unsafe sex behavior, PrEP use was still more beneficial than the current situation. In the model, early diagnosis of HIV infection was also effectively decreased HIV incidence. However, early ART did not show considerable effectiveness. As expected, it would be most effective if all interventions (PrEP, early diagnosis and early treatment) were implemented together. This model suggests that PrEP and early diagnosis could be a very effective way to reduce HIV incidence in South Korea among MSM.

  18. An investigation of FLUENT's fan model including the effect of swirl velocity

    International Nuclear Information System (INIS)

    El Saheli, A.; Barron, R.M.

    2002-01-01

    The purpose of this paper is to investigate and discuss the reliability of simplified models for the computational fluid dynamics (CFD) simulation of air flow through automotive engine cooling fans. One of the most widely used simplified fan models in industry is a variant of the actuator disk model which is available in most commercial CFD software, such as FLUENT. In this model, the fan is replaced by an infinitely thin surface on which pressure rise across the fan is specified as a polynomial function of normal velocity or flow rate. The advantages of this model are that it is simple, it accurately predicts the pressure rise through the fan and the axial velocity, and it is robust

  19. Including lateral interactions into microkinetic models of catalytic reactions

    DEFF Research Database (Denmark)

    Hellman, Anders; Honkala, Johanna Karoliina

    2007-01-01

    In many catalytic reactions lateral interactions between adsorbates are believed to have a strong influence on the reaction rates. We apply a microkinetic model to explore the effect of lateral interactions and how to efficiently take them into account in a simple catalytic reaction. Three differ...... different approximations are investigated: site, mean-field, and quasichemical approximations. The obtained results are compared to accurate Monte Carlo numbers. In the end, we apply the approximations to a real catalytic reaction, namely, ammonia synthesis....

  20. Measurement network design including traveltime determinations to minimize model prediction uncertainty

    NARCIS (Netherlands)

    Janssen, G.M.C.M.; Valstar, J.R.; Zee, van der S.E.A.T.M.

    2008-01-01

    Traveltime determinations have found increasing application in the characterization of groundwater systems. No algorithms are available, however, to optimally design sampling strategies including this information type. We propose a first-order methodology to include groundwater age or tracer arrival

  1. Prediction of preterm birth in multiple pregnancies: development of a multivariable model including cervical length measurement at 16 to 21 weeks' gestation.

    Science.gov (United States)

    van de Mheen, Lidewij; Schuit, Ewoud; Lim, Arianne C; Porath, Martina M; Papatsonis, Dimitri; Erwich, Jan J; van Eyck, Jim; van Oirschot, Charlotte M; Hummel, Piet; Duvekot, Johannes J; Hasaart, Tom H M; Groenwold, Rolf H H; Moons, Karl G M; de Groot, Christianne J M; Bruinse, Hein W; van Pampus, Maria G; Mol, Ben W J

    2014-04-01

    To develop a multivariable prognostic model for the risk of preterm delivery in women with multiple pregnancy that includes cervical length measurement at 16 to 21 weeks' gestation and other variables. We used data from a previous randomized trial. We assessed the association between maternal and pregnancy characteristics including cervical length measurement at 16 to 21 weeks' gestation and time to delivery using multivariable Cox regression modelling. Performance of the final model was assessed for the outcomes of preterm and very preterm delivery using calibration and discrimination measures. We studied 507 women, of whom 270 (53%) delivered models for preterm and very preterm delivery had a c-index of 0.68 (95% CI 0.63 to 0.72) and 0.68 (95% CI 0.62 to 0.75), respectively, and showed good calibration. In women with a multiple pregnancy, the risk of preterm delivery can be assessed with a multivariable model incorporating cervical length and other predictors.

  2. A comparison of different ways of including baseline counts in negative binomial models for data from falls prevention trials.

    Science.gov (United States)

    Zheng, Han; Kimber, Alan; Goodwin, Victoria A; Pickering, Ruth M

    2018-01-01

    A common design for a falls prevention trial is to assess falling at baseline, randomize participants into an intervention or control group, and ask them to record the number of falls they experience during a follow-up period of time. This paper addresses how best to include the baseline count in the analysis of the follow-up count of falls in negative binomial (NB) regression. We examine the performance of various approaches in simulated datasets where both counts are generated from a mixed Poisson distribution with shared random subject effect. Including the baseline count after log-transformation as a regressor in NB regression (NB-logged) or as an offset (NB-offset) resulted in greater power than including the untransformed baseline count (NB-unlogged). Cook and Wei's conditional negative binomial (CNB) model replicates the underlying process generating the data. In our motivating dataset, a statistically significant intervention effect resulted from the NB-logged, NB-offset, and CNB models, but not from NB-unlogged, and large, outlying baseline counts were overly influential in NB-unlogged but not in NB-logged. We conclude that there is little to lose by including the log-transformed baseline count in standard NB regression compared to CNB for moderate to larger sized datasets. © 2017 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  3. A new mammalian circadian oscillator model including the cAMP module

    International Nuclear Information System (INIS)

    Jun-Wei, Wang; Tian-Shou, Zhou

    2009-01-01

    In this paper, we develop a new mathematical model for the mammalian circadian clock, which incorporates both transcriptional/translational feedback loops (TTFLs) and a cAMP-mediated feedback loop. The model shows that TTFLs and cAMP signalling cooperatively drive the circadian rhythms. It reproduces typical experimental observations with qualitative similarities, e.g. circadian oscillations in constant darkness and entrainment to light–dark cycles. In addition, it can explain the phenotypes of cAMP-mutant and Rev-erbα −/− -mutant mice, and help us make an experimentally-testable prediction: oscillations may be rescued when arrhythmic mice with constitutively low concentrations of cAMP are crossed with Rev-erbα −/− mutant mice. The model enhances our understanding of the mammalian circadian clockwork from the viewpoint of the entire cell. (cross-disciplinary physics and related areas of science and technology)

  4. Impact of including surface currents on simulation of Indian Ocean variability with the POAMA coupled model

    Energy Technology Data Exchange (ETDEWEB)

    Zhao, Mei; Wang, Guomin; Hendon, Harry H.; Alves, Oscar [Bureau of Meteorology, Centre for Australian Weather and Climate Research, Melbourne (Australia)

    2011-04-15

    Impacts on the coupled variability of the Indo-Pacific by including the effects of surface currents on surface stress are explored in four extended integrations of an experimental version of the Bureau of Meteorology's coupled seasonal forecast model POAMA. The first pair of simulations differs only in their treatment of momentum coupling: one version includes the effects of surface currents on the surface stress computation and the other does not. The version that includes the effect of surface currents has less mean-state bias in the equatorial Pacific cold tongue but produces relatively weak coupled variability in the Tropics, especially that related to the Indian Ocean dipole (IOD) and El Nino/Southern Oscillation (ENSO). The version without the effects of surface currents has greater bias in the Pacific cold tongue but stronger IOD and ENSO variability. In order to diagnose the role of changes in local coupling from changes in remote forcing by ENSO for causing changes in IOD variability, a second set of simulations is conducted where effects of surface currents are included only in the Indian Ocean and only in the Pacific Ocean. IOD variability is found to be equally reduced by inclusion of the local effects of surface currents in the Indian Ocean and by the reduction of ENSO variability as a result of including effects of surface currents in the Pacific. Some implications of these results for predictability of the IOD and its dependence on ENSO, and for ocean subsurface data assimilation are discussed. (orig.)

  5. Parkinsonian phenotype in Machado-Joseph disease (MJD/SCA3): a two-case report

    NARCIS (Netherlands)

    Bettencourt, C.; Santos, C.; Coutinho, P.; Rizzu, P.; Vasconcelos, J.; Kay, T.; Cymbron, T.; Raposo, M.; Heutink, P.; Lima, M.

    2011-01-01

    Background: Machado-Joseph disease (MJD), or spinocerebellar ataxia type 3 (SCA3), is an autosomal dominant neurodegenerative disorder of late onset, which is caused by a CAG repeat expansion in the coding region of the ATXN3 gene. This disease presents clinical heterogeneity, which cannot be

  6. Language Impairment in Cerebellar Ataxia

    NARCIS (Netherlands)

    van Gaalen, Judith; de Swart, Bert J. M.; Oostveen, Judith; Knuijt, Simone; van de Warrenburg, Bart P. C.; Kremer, Berry (H. ) P. H.

    Background: Several studies have suggested that language impairment can be observed in patients with cerebellar pathology. The aim of this study was to investigate language performance in patients with spinocerebellar ataxia type 6 (SCA6). Methods: We assessed speech and language in 29 SCA6 patients

  7. Dynamic model including piping acoustics of a centrifugal compression system

    NARCIS (Netherlands)

    Helvoirt, van J.; Jager, de A.G.

    2007-01-01

    This paper deals with low frequency pulsation phenomena in full-scale centrifugal compression systems associated with compressor surge. The Greitzer lumped parameter model is applied to describe the dynamic behavior of an industrial compressor test rig and experimental evidence is provided for the

  8. 3-D FEM Modeling of fiber/matrix interface debonding in UD composites including surface effects

    International Nuclear Information System (INIS)

    Pupurs, A; Varna, J

    2012-01-01

    Fiber/matrix interface debond growth is one of the main mechanisms of damage evolution in unidirectional (UD) polymer composites. Because for polymer composites the fiber strain to failure is smaller than for the matrix multiple fiber breaks occur at random positions when high mechanical stress is applied to the composite. The energy released due to each fiber break is usually larger than necessary for the creation of a fiber break therefore a partial debonding of fiber/matrix interface is typically observed. Thus the stiffness reduction of UD composite is contributed both from the fiber breaks and from the interface debonds. The aim of this paper is to analyze the debond growth in carbon fiber/epoxy and glass fiber/epoxy UD composites using fracture mechanics principles by calculation of energy release rate G II . A 3-D FEM model is developed for calculation of energy release rate for fiber/matrix interface debonds at different locations in the composite including the composite surface region where the stress state differs from the one in the bulk composite. In the model individual partially debonded fiber is surrounded by matrix region and embedded in a homogenized composite.

  9. Extensions of the Rosner-Colditz breast cancer prediction model to include older women and type-specific predicted risk.

    Science.gov (United States)

    Glynn, Robert J; Colditz, Graham A; Tamimi, Rulla M; Chen, Wendy Y; Hankinson, Susan E; Willett, Walter W; Rosner, Bernard

    2017-08-01

    A breast cancer risk prediction rule previously developed by Rosner and Colditz has reasonable predictive ability. We developed a re-fitted version of this model, based on more than twice as many cases now including women up to age 85, and further extended it to a model that distinguished risk factor prediction of tumors with different estrogen/progesterone receptor status. We compared the calibration and discriminatory ability of the original, the re-fitted, and the type-specific models. Evaluation used data from the Nurses' Health Study during the period 1980-2008, when 4384 incident invasive breast cancers occurred over 1.5 million person-years. Model development used two-thirds of study subjects and validation used one-third. Predicted risks in the validation sample from the original and re-fitted models were highly correlated (ρ = 0.93), but several parameters, notably those related to use of menopausal hormone therapy and age, had different estimates. The re-fitted model was well-calibrated and had an overall C-statistic of 0.65. The extended, type-specific model identified several risk factors with varying associations with occurrence of tumors of different receptor status. However, this extended model relative to the prediction of any breast cancer did not meaningfully reclassify women who developed breast cancer to higher risk categories, nor women remaining cancer free to lower risk categories. The re-fitted Rosner-Colditz model has applicability to risk prediction in women up to age 85, and its discrimination is not improved by consideration of varying associations across tumor subtypes.

  10. Expansion Hamiltonian model for a diatomic molecule adsorbed on a surface: Vibrational states of the CO/Cu(100) system including surface vibrations

    Energy Technology Data Exchange (ETDEWEB)

    Meng, Qingyong, E-mail: mengqingyong@dicp.ac.cn [State Key Laboratory of Molecular Reaction Dynamics, Dalian Institute of Chemical Physics, Chinese Academy of Sciences, Zhongshan Road 457, 116023 Dalian (China); Meyer, Hans-Dieter, E-mail: hans-dieter.meyer@pci.uni-heidelberg.de [Theoretische Chemie, Physikalisch-Chemisches Institut, Ruprecht-Karls Universität Heidelberg, Im Neuenheimer Feld 229, D-69120 Heidelberg (Germany)

    2015-10-28

    Molecular-surface studies are often done by assuming a corrugated, static (i.e., rigid) surface. To be able to investigate the effects that vibrations of surface atoms may have on spectra and cross sections, an expansion Hamiltonian model is proposed on the basis of the recently reported [R. Marquardt et al., J. Chem. Phys. 132, 074108 (2010)] SAP potential energy surface (PES), which was built for the CO/Cu(100) system with a rigid surface. In contrast to other molecule-surface coupling models, such as the modified surface oscillator model, the coupling between the adsorbed molecule and the surface atoms is already included in the present expansion SAP-PES model, in which a Taylor expansion around the equilibrium positions of the surface atoms is performed. To test the quality of the Taylor expansion, a direct model, that is avoiding the expansion, is also studied. The latter, however, requests that there is only one movable surface atom included. On the basis of the present expansion and direct models, the effects of a moving top copper atom (the one to which CO is bound) on the energy levels of a bound CO/Cu(100) system are studied. For this purpose, the multiconfiguration time-dependent Hartree calculations are carried out to obtain the vibrational fundamentals and overtones of the CO/Cu(100) system including a movable top copper atom. In order to interpret the results, a simple model consisting of two coupled harmonic oscillators is introduced. From these calculations, the vibrational levels of the CO/Cu(100) system as function of the frequency of the top copper atom are discussed.

  11. Constraining the Magmatic System at Mount St. Helens (2004-2008) Using Bayesian Inversion With Physics-Based Models Including Gas Escape and Crystallization

    International Nuclear Information System (INIS)

    Wong, Ying-Qi; Segall, Paul; Bradley, Andrew; Anderson, Kyle

    2017-01-01

    Physics-based models of volcanic eruptions track conduit processes as functions of depth and time. When used in inversions, these models permit integration of diverse geological and geophysical data sets to constrain important parameters of magmatic systems. We develop a 1-D steady state conduit model for effusive eruptions including equilibrium crystallization and gas transport through the conduit and compare with the quasi-steady dome growth phase of Mount St. Helens in 2005. Viscosity increase resulting from pressure-dependent crystallization leads to a natural transition from viscous flow to frictional sliding on the conduit margin. Erupted mass flux depends strongly on wall rock and magma permeabilities due to their impact on magma density. Including both lateral and vertical gas transport reveals competing effects that produce nonmonotonic behavior in the mass flux when increasing magma permeability. Using this physics-based model in a Bayesian inversion, we link data sets from Mount St. Helens such as extrusion flux and earthquake depths with petrological data to estimate unknown model parameters, including magma chamber pressure and water content, magma permeability constants, conduit radius, and friction along the conduit walls. Even with this relatively simple model and limited data, we obtain improved constraints on important model parameters. We find that the magma chamber had low (<5 wt %) total volatiles and that the magma permeability scale is well constrained at ~10 –11.4 m 2 to reproduce observed dome rock porosities. Here, compared with previous results, higher magma overpressure and lower wall friction are required to compensate for increased viscous resistance while keeping extrusion rate at the observed value.

  12. XFEL OSCILLATOR SIMULATION INCLUDING ANGLE-DEPENDENT CRYSTAL REFLECTIVITY

    International Nuclear Information System (INIS)

    Fawley, William; Lindberg, Ryan; Kim, K.-J.; Shvyd'ko, Yuri

    2010-01-01

    The oscillator package within the GINGER FEL simulation code has now been extended to include angle-dependent reflectivity properties of Bragg crystals. Previously, the package was modified to include frequencydependent reflectivity in order to model x-ray FEL oscillators from start-up from shot noise through to saturation. We present a summary of the algorithms used for modeling the crystal reflectivity and radiation propagation outside the undulator, discussing various numerical issues relevant to the domain of high Fresnel number and efficient Hankel transforms. We give some sample XFEL-O simulation results obtained with the angle-dependent reflectivity model, with particular attention directed to the longitudinal and transverse coherence of the radiation output.

  13. [Spinocerebellar ataxia type 8: the case of a Spanish family].

    Science.gov (United States)

    Mayo-Cabrero, D; Sánchez-Migallón, M; Cantarero, S; García-Ruiz Espiga, P J; Giménez-Pardo, A; Trujillo-Tiebas, M; Ayuso-García, C

    Dominant autosomic ataxias include a group of neurodegenerative diseases characterized by the abnormal expansion of triplets. Male aged 33, with expansion of the SCA 8 gene (100 repetitions), who presented a clinical picture compatible with a pancerebellar syndrome. The patient had been diagnosed 11 years earlier as suffering from previously of histiocytosis X. A clinico genetic study was conducted on the patient and several members of his family (parents and two sisters). Both sisters and the father were found to be carriers of the expansion (110 and 150 repetitions, respectively), and are currently asymptomatic. There is no relation between the number of repetitions and the age of onset of the disease. The normal interval in our population oscillates between 16 37 repetitions, and the pathological interval has not been well determined. There may be a relation between the SCA 8 form and histiocytosis X.

  14. Multiple sclerosis impairs regional functional connectivity in the cerebellum

    DEFF Research Database (Denmark)

    Dogonowski, Anne-Marie; Andersen, Kasper Winther; Madsen, Kristoffer Hougaard

    2013-01-01

    in the cerebellum in MS. This might be caused by a functional disruption of cortico-ponto-cerebellar and spino-cerebellar inputs, since patients with higher lesion load in the left cerebellar peduncles showed a stronger reduction in cerebellar homogeneity. In patients, two clusters in the left posterior cerebellum...

  15. Spectral element modelling of seismic wave propagation in visco-elastoplastic media including excess-pore pressure development

    Science.gov (United States)

    Oral, Elif; Gélis, Céline; Bonilla, Luis Fabián; Delavaud, Elise

    2017-12-01

    Numerical modelling of seismic wave propagation, considering soil nonlinearity, has become a major topic in seismic hazard studies when strong shaking is involved under particular soil conditions. Indeed, when strong ground motion propagates in saturated soils, pore pressure is another important parameter to take into account when successive phases of contractive and dilatant soil behaviour are expected. Here, we model 1-D seismic wave propagation in linear and nonlinear media using the spectral element numerical method. The study uses a three-component (3C) nonlinear rheology and includes pore-pressure excess. The 1-D-3C model is used to study the 1987 Superstition Hills earthquake (ML 6.6), which was recorded at the Wildlife Refuge Liquefaction Array, USA. The data of this event present strong soil nonlinearity involving pore-pressure effects. The ground motion is numerically modelled for different assumptions on soil rheology and input motion (1C versus 3C), using the recorded borehole signals as input motion. The computed acceleration-time histories show low-frequency amplification and strong high-frequency damping due to the development of pore pressure in one of the soil layers. Furthermore, the soil is found to be more nonlinear and more dilatant under triaxial loading compared to the classical 1C analysis, and significant differences in surface displacements are observed between the 1C and 3C approaches. This study contributes to identify and understand the dominant phenomena occurring in superficial layers, depending on local soil properties and input motions, conditions relevant for site-specific studies.

  16. Parton recombination model including resonance production. RL-78-040

    International Nuclear Information System (INIS)

    Roberts, R.G.; Hwa, R.C.; Matsuda, S.

    1978-05-01

    Possible effects of resonance production on the meson inclusive distribution in the fragmentation region are investigated in the framework of the parton recombination model. From a detailed study of the data on vector-meson production, a reliable ratio of the vector-to-pseudoscalar rates is determined. Then the influence of the decay of the vector mesons on the pseudoscalar spectrum is examined, and the effect found to be no more than 25% for x > 0.5. The normalization of the non-strange antiquark distributions are still higher than those in a quiescent proton. The agreement between the calculated results and data remain very good. 36 references

  17. Parton recombination model including resonance production. RL-78-040

    Energy Technology Data Exchange (ETDEWEB)

    Roberts, R. G.; Hwa, R. C.; Matsuda, S.

    1978-05-01

    Possible effects of resonance production on the meson inclusive distribution in the fragmentation region are investigated in the framework of the parton recombination model. From a detailed study of the data on vector-meson production, a reliable ratio of the vector-to-pseudoscalar rates is determined. Then the influence of the decay of the vector mesons on the pseudoscalar spectrum is examined, and the effect found to be no more than 25% for x > 0.5. The normalization of the non-strange antiquark distributions are still higher than those in a quiescent proton. The agreement between the calculated results and data remain very good. 36 references.

  18. A statistical model for estimation of fish density including correlation in size, space, time and between species from research survey data.

    Directory of Open Access Journals (Sweden)

    J Rasmus Nielsen

    Full Text Available Trawl survey data with high spatial and seasonal coverage were analysed using a variant of the Log Gaussian Cox Process (LGCP statistical model to estimate unbiased relative fish densities. The model estimates correlations between observations according to time, space, and fish size and includes zero observations and over-dispersion. The model utilises the fact the correlation between numbers of fish caught increases when the distance in space and time between the fish decreases, and the correlation between size groups in a haul increases when the difference in size decreases. Here the model is extended in two ways. Instead of assuming a natural scale size correlation, the model is further developed to allow for a transformed length scale. Furthermore, in the present application, the spatial- and size-dependent correlation between species was included. For cod (Gadus morhua and whiting (Merlangius merlangus, a common structured size correlation was fitted, and a separable structure between the time and space-size correlation was found for each species, whereas more complex structures were required to describe the correlation between species (and space-size. The within-species time correlation is strong, whereas the correlations between the species are weaker over time but strong within the year.

  19. Model Selection and Evaluation Based on Emerging Infectious Disease Data Sets including A/H1N1 and Ebola

    Directory of Open Access Journals (Sweden)

    Wendi Liu

    2015-01-01

    Full Text Available The aim of the present study is to apply simple ODE models in the area of modeling the spread of emerging infectious diseases and show the importance of model selection in estimating parameters, the basic reproduction number, turning point, and final size. To quantify the plausibility of each model, given the data and the set of four models including Logistic, Gompertz, Rosenzweg, and Richards models, the Bayes factors are calculated and the precise estimates of the best fitted model parameters and key epidemic characteristics have been obtained. In particular, for Ebola the basic reproduction numbers are 1.3522 (95% CI (1.3506, 1.3537, 1.2101 (95% CI (1.2084, 1.2119, 3.0234 (95% CI (2.6063, 3.4881, and 1.9018 (95% CI (1.8565, 1.9478, the turning points are November 7,November 17, October 2, and November 3, 2014, and the final sizes until December 2015 are 25794 (95% CI (25630, 25958, 3916 (95% CI (3865, 3967, 9886 (95% CI (9740, 10031, and 12633 (95% CI (12515, 12750 for West Africa, Guinea, Liberia, and Sierra Leone, respectively. The main results confirm that model selection is crucial in evaluating and predicting the important quantities describing the emerging infectious diseases, and arbitrarily picking a model without any consideration of alternatives is problematic.

  20. Kinetic model of water disinfection using peracetic acid including synergistic effects.

    Science.gov (United States)

    Flores, Marina J; Brandi, Rodolfo J; Cassano, Alberto E; Labas, Marisol D

    2016-01-01

    The disinfection efficiencies of a commercial mixture of peracetic acid against Escherichia coli were studied in laboratory scale experiments. The joint and separate action of two disinfectant agents, hydrogen peroxide and peracetic acid, were evaluated in order to observe synergistic effects. A kinetic model for each component of the mixture and for the commercial mixture was proposed. Through simple mathematical equations, the model describes different stages of attack by disinfectants during the inactivation process. Based on the experiments and the kinetic parameters obtained, it could be established that the efficiency of hydrogen peroxide was much lower than that of peracetic acid alone. However, the contribution of hydrogen peroxide was very important in the commercial mixture. It should be noted that this improvement occurred only after peracetic acid had initiated the attack on the cell. This synergistic effect was successfully explained by the proposed scheme and was verified by experimental results. Besides providing a clearer mechanistic understanding of water disinfection, such models may improve our ability to design reactors.

  1. ETM documentation update – including modelling conventions and manual for software tools

    DEFF Research Database (Denmark)

    Grohnheit, Poul Erik

    This is the final report for the DTU contribution to Socio Economic Research on Fusion (SERF), EFDA Technology Work programme 2013. The structure and contents of this report was presented at the EFDA-TIMES workshop in Garching 12-13 December 2013. This report gives further background and references......, it summarises the work done during 2013, and it also contains presentations for promotion of fusion as a future element in the electricity generation mix and presentations for the modelling community concerning model development and model documentation – in particular for TIAM collaboration workshops....

  2. Lack of miRNA misregulation at early pathological stages in Drosophila neurodegenerative disease models

    Directory of Open Access Journals (Sweden)

    Anita eReinhardt

    2012-10-01

    Full Text Available Late onset neurodegenerative diseases represent a major public health concern as the population in many countries ages. Both frequent diseases such as Alzheimer disease (AD, 14% incidence for 80-84 year old Europeans or Parkinson disease (PD, 1.4% prevalence for > 55 years old share, with other low-incidence neurodegenerative pathologies such as spinocerebellar ataxias (SCAs, 0.01% prevalence and frontotemporal lobar degeneration (FTLD, 0.02% prevalence, a lack of efficient treatment in spite of important research efforts. Besides significant progress, studies with animal models have revealed unexpected complexities in the degenerative process, emphasizing a need to better understand the underlying pathological mechanisms. Recently, microRNAs, a class of small regulatory non-coding RNAs, have been implicated in some neurodegenerative diseases. The current data supporting a role of miRNAs in PD, tauopathies, dominant ataxias and FTLD will first be discussed to emphasize the different levels of the pathological processes which may be affected by miRNAs. To investigate a potential involvement of miRNA dysregulation in the early stages of these neurodegenerative diseases we have used Drosophila models for 7 diseases (PD, 3 FTLD, 3 dominant ataxias that recapitulate many features of the human diseases. We performed deep sequencing of head small RNAs after 3 days of pathological protein expression in the fly head neurons. We found no evidence for a statistically significant difference in miRNA expression in this early stage of the pathological process. In addition, we could not identify small non coding CAG repeat RNAs (sCAG in polyQ disease models. Thus our data suggest that transcriptional deregulation of miRNAs or sCAG is unlikely to play a significant role in the initial stages of neurodegenerative diseases.

  3. Statistical methodology for discrete fracture model - including fracture size, orientation uncertainty together with intensity uncertainty and variability

    Energy Technology Data Exchange (ETDEWEB)

    Darcel, C. (Itasca Consultants SAS (France)); Davy, P.; Le Goc, R.; Dreuzy, J.R. de; Bour, O. (Geosciences Rennes, UMR 6118 CNRS, Univ. def Rennes, Rennes (France))

    2009-11-15

    the lineament scale (k{sub t} = 2) on the other, addresses the issue of the nature of the transition. We develop a new 'mechanistic' model that could help in modeling why and where this transition can occur. The transition between both regimes would occur for a fracture length of 1-10 m and even at a smaller scale for the few outcrops that follow the self-similar density model. A consequence for the disposal issue is that the model that is likely to apply in the 'blind' scale window between 10-100 m is the self-similar model as it is defined for large-scale lineaments. The self-similar model, as it is measured for some outcrops and most lineament maps, is definitely worth being investigated as a reference for scales above 1-10 m. In the rest of the report, we develop a methodology for incorporating uncertainty and variability into the DFN modeling. Fracturing properties arise from complex processes which produce an intrinsic variability; characterizing this variability as an admissible variation of model parameter or as the division of the site into subdomains with distinct DFN models is a critical point of the modeling effort. Moreover, the DFN model encompasses a part of uncertainty, due to data inherent uncertainties and sampling limits. Both effects must be quantified and incorporated into the DFN site model definition process. In that context, all available borehole data including recording of fracture intercept positions, pole orientation and relative uncertainties are used as the basis for the methodological development and further site model assessment. An elementary dataset contains a set of discrete fracture intercepts from which a parent orientation/density distribution can be computed. The elementary bricks of the site, from which these initial parent density distributions are computed, rely on the former Single Hole Interpretation division of the boreholes into sections whose local boundaries are expected to reflect - locally - geology

  4. NASTRAN thermal analyzer: Theory and application including a guide to modeling engineering problems, volume 2. [sample problem library guide

    Science.gov (United States)

    Jackson, C. E., Jr.

    1977-01-01

    A sample problem library containing 20 problems covering most facets of Nastran Thermal Analyzer modeling is presented. Areas discussed include radiative interchange, arbitrary nonlinear loads, transient temperature and steady-state structural plots, temperature-dependent conductivities, simulated multi-layer insulation, and constraint techniques. The use of the major control options and important DMAP alters is demonstrated.

  5. A Two-Account Life Insurance Model for Scenario-Based Valuation Including Event Risk

    Directory of Open Access Journals (Sweden)

    Ninna Reitzel Jensen

    2015-06-01

    Full Text Available Using a two-account model with event risk, we model life insurance contracts taking into account both guaranteed and non-guaranteed payments in participating life insurance as well as in unit-linked insurance. Here, event risk is used as a generic term for life insurance events, such as death, disability, etc. In our treatment of participating life insurance, we have special focus on the bonus schemes “consolidation” and “additional benefits”, and one goal is to formalize how these work and interact. Another goal is to describe similarities and differences between participating life insurance and unit-linked insurance. By use of a two-account model, we are able to illustrate general concepts without making the model too abstract. To allow for complicated financial markets without dramatically increasing the mathematical complexity, we focus on economic scenarios. We illustrate the use of our model by conducting scenario analysis based on Monte Carlo simulation, but the model applies to scenarios in general and to worst-case and best-estimate scenarios in particular. In addition to easy computations, our model offers a common framework for the valuation of life insurance payments across product types. This enables comparison of participating life insurance products and unit-linked insurance products, thus building a bridge between the two different ways of formalizing life insurance products. Finally, our model distinguishes itself from the existing literature by taking into account the Markov model for the state of the policyholder and, hereby, facilitating event risk.

  6. Laboratory Studies of the Reactive Chemistry and Changing CCN Properties of Secondary Organic Aerosol, Including Model Development

    Energy Technology Data Exchange (ETDEWEB)

    Scot Martin

    2013-01-31

    The chemical evolution of secondary-organic-aerosol (SOA) particles and how this evolution alters their cloud-nucleating properties were studied. Simplified forms of full Koehler theory were targeted, specifically forms that contain only those aspects essential to describing the laboratory observations, because of the requirement to minimize computational burden for use in integrated climate and chemistry models. The associated data analysis and interpretation have therefore focused on model development in the framework of modified kappa-Koehler theory. Kappa is a single parameter describing effective hygroscopicity, grouping together several separate physicochemical parameters (e.g., molar volume, surface tension, and van't Hoff factor) that otherwise must be tracked and evaluated in an iterative full-Koehler equation in a large-scale model. A major finding of the project was that secondary organic materials produced by the oxidation of a range of biogenic volatile organic compounds for diverse conditions have kappa values bracketed in the range of 0.10 +/- 0.05. In these same experiments, somewhat incongruently there was significant chemical variation in the secondary organic material, especially oxidation state, as was indicated by changes in the particle mass spectra. Taken together, these findings then support the use of kappa as a simplified yet accurate general parameter to represent the CCN activation of secondary organic material in large-scale atmospheric and climate models, thereby greatly reducing the computational burden while simultaneously including the most recent mechanistic findings of laboratory studies.

  7. Including Overweight or Obese Students in Physical Education: A Social Ecological Constraint Model

    Science.gov (United States)

    Li, Weidong; Rukavina, Paul

    2012-01-01

    In this review, we propose a social ecological constraint model to study inclusion of overweight or obese students in physical education by integrating key concepts and assumptions from ecological constraint theory in motor development and social ecological models in health promotion and behavior. The social ecological constraint model proposes…

  8. An approach for including the stiffness and damping of elastohydrodynamic point contacts in deep groove ball bearing equilibrium models

    Science.gov (United States)

    Nonato, Fábio; Cavalca, Katia L.

    2014-12-01

    This work presents a methodology for including the Elastohydrodynamic (EHD) film effects to a lateral vibration model of a deep groove ball bearing by using a novel approximation for the EHD contacts by a set of equivalent nonlinear spring and viscous damper. The fitting of the equivalent contact model used the results of a transient multi-level finite difference EHD algorithm to adjust the dynamic parameters. The comparison between the approximated model and the finite difference simulated results showed a suitable representation of the stationary and dynamic contact behaviors. The linear damping hypothesis could be shown as a rough representation of the actual hysteretic behavior of the EHD contact. Nevertheless, the overall accuracy of the model was not impaired by the use of such approximation. Further on, the inclusion of the equivalent EHD contact model is equated for both the restoring and the dissipative components of the bearing's lateral dynamics. The derived model was used to investigate the effects of the rolling element bearing lubrication on the vibration response of a rotor's lumped parameter model. The fluid film stiffening effect, previously only observable by experimentation, could be quantified using the proposed model, as well as the portion of the bearing damping provided by the EHD fluid film. Results from a laboratory rotor-bearing test rig were used to indirectly validate the proposed contact approximation. A finite element model of the rotor accounting for the lubricated bearing formulation adequately portrayed the frequency content of the bearing orbits observed on the test rig.

  9. A sub-circuit MOSFET model with a wide temperature range including cryogenic temperature

    Energy Technology Data Exchange (ETDEWEB)

    Jia Kan; Sun Weifeng; Shi Longxing, E-mail: jiakan.01@gmail.com [National ASIC System Engineering Research Center, Southeast University, Nanjing 210096 (China)

    2011-06-15

    A sub-circuit SPICE model of a MOSFET for low temperature operation is presented. Two resistors are introduced for the freeze-out effect, and the explicit behavioral models are developed for them. The model can be used in a wide temperature range covering both cryogenic temperature and regular temperatures. (semiconductor devices)

  10. Models of disordered media: some new results, including some new connections between composite-media, fluid-state, and random-flight theories

    International Nuclear Information System (INIS)

    Stell, G.

    1983-01-01

    Some new theoretical results on the microstructure of models of two-phase disordered media are given, as well as the new quantitative bounds on the thermal conductivity that follows for one such model (randomly centered spherical inclusions). A second set of results is then given for random flights, including random flights with hit expectancy prescribed in a unit hall around the flight origin. Finally, some interesting correspondences are demonstrated, via the Ornstein-Zernike equation, between random-flight results, liquid-state results and percolation-theory results. 27 references, 6 figures, 4 tables

  11. Modelled hydraulic redistribution by sunflower (Helianthus annuus L.) matches observed data only after including night-time transpiration.

    Science.gov (United States)

    Neumann, Rebecca B; Cardon, Zoe G; Teshera-Levye, Jennifer; Rockwell, Fulton E; Zwieniecki, Maciej A; Holbrook, N Michele

    2014-04-01

    The movement of water from moist to dry soil layers through the root systems of plants, referred to as hydraulic redistribution (HR), occurs throughout the world and is thought to influence carbon and water budgets and ecosystem functioning. The realized hydrologic, biogeochemical and ecological consequences of HR depend on the amount of redistributed water, whereas the ability to assess these impacts requires models that correctly capture HR magnitude and timing. Using several soil types and two ecotypes of sunflower (Helianthus annuus L.) in split-pot experiments, we examined how well the widely used HR modelling formulation developed by Ryel et al. matched experimental determination of HR across a range of water potential driving gradients. H. annuus carries out extensive night-time transpiration, and although over the last decade it has become more widely recognized that night-time transpiration occurs in multiple species and many ecosystems, the original Ryel et al. formulation does not include the effect of night-time transpiration on HR. We developed and added a representation of night-time transpiration into the formulation, and only then was the model able to capture the dynamics and magnitude of HR we observed as soils dried and night-time stomatal behaviour changed, both influencing HR. © 2013 John Wiley & Sons Ltd.

  12. Microfluidic System Simulation Including the Electro-Viscous Effect

    Science.gov (United States)

    Rojas, Eileen; Chen, C. P.; Majumdar, Alok

    2007-01-01

    This paper describes a practical approach using a general purpose lumped-parameter computer program, GFSSP (Generalized Fluid System Simulation Program) for calculating flow distribution in a network of micro-channels including electro-viscous effects due to the existence of electrical double layer (EDL). In this study, an empirical formulation for calculating an effective viscosity of ionic solutions based on dimensional analysis is described to account for surface charge and bulk fluid conductivity, which give rise to electro-viscous effect in microfluidics network. Two dimensional slit micro flow data was used to determine the model coefficients. Geometry effect is then included through a Poiseuille number correlation in GFSSP. The bi-power model was used to calculate flow distribution of isotropically etched straight channel and T-junction microflows involving ionic solutions. Performance of the proposed model is assessed against experimental test data.

  13. Statistical methodology for discrete fracture model - including fracture size, orientation uncertainty together with intensity uncertainty and variability

    International Nuclear Information System (INIS)

    Darcel, C.; Davy, P.; Le Goc, R.; Dreuzy, J.R. de; Bour, O.

    2009-11-01

    the other, addresses the issue of the nature of the transition. We develop a new 'mechanistic' model that could help in modeling why and where this transition can occur. The transition between both regimes would occur for a fracture length of 1-10 m and even at a smaller scale for the few outcrops that follow the self-similar density model. A consequence for the disposal issue is that the model that is likely to apply in the 'blind' scale window between 10-100 m is the self-similar model as it is defined for large-scale lineaments. The self-similar model, as it is measured for some outcrops and most lineament maps, is definitely worth being investigated as a reference for scales above 1-10 m. In the rest of the report, we develop a methodology for incorporating uncertainty and variability into the DFN modeling. Fracturing properties arise from complex processes which produce an intrinsic variability; characterizing this variability as an admissible variation of model parameter or as the division of the site into subdomains with distinct DFN models is a critical point of the modeling effort. Moreover, the DFN model encompasses a part of uncertainty, due to data inherent uncertainties and sampling limits. Both effects must be quantified and incorporated into the DFN site model definition process. In that context, all available borehole data including recording of fracture intercept positions, pole orientation and relative uncertainties are used as the basis for the methodological development and further site model assessment. An elementary dataset contains a set of discrete fracture intercepts from which a parent orientation/density distribution can be computed. The elementary bricks of the site, from which these initial parent density distributions are computed, rely on the former Single Hole Interpretation division of the boreholes into sections whose local boundaries are expected to reflect - locally - geology and fracturing properties main characteristics. From that

  14. A reanalysis of a behavioral intervention to prevent incident HIV infections: Including indirect effects in modeling outcomes of Project EXPLORE

    Science.gov (United States)

    Eaton, Lisa A.; Kalichman, Seth C.; Kenny, David A.; Harel, Ofer

    2013-01-01

    Background Project EXPLORE -- a large-scale, behavioral intervention tested among men who have sex with men (MSM) at-risk for HIV infection --was generally deemed as ineffective in reducing HIV incidence. Using novel and more precise data analytic techniques we reanalyzed Project EXPLORE by including both direct and indirect paths of intervention effects. Methods Data from 4,296 HIV negative MSM who participated in Project EXPLORE, which included ten sessions of behavioral risk reduction counseling completed from 1999-2005, were included in the analysis. We reanalyzed the data to include parameters that estimate the overtime effects of the intervention on unprotected anal sex and the over-time effects of the intervention on HIV status mediated by unprotected anal sex simultaneously in a single model. Results We found the indirect effect of intervention on HIV infection through unprotected anal sex to be statistically significant up through 12 months post-intervention, OR=.83, 95% CI=.72-.95. Furthermore, the intervention significantly reduced unprotected anal sex up through 18 months post-intervention, OR=.79, 95% CI=.63-.99. Discussion Our results reveal effects not tested in the original model that offer new insight into the effectiveness of a behavioral intervention for reducing HIV incidence. Project EXPLORE demonstrated that when tested against an evidence-based, effective control condition can result in reductions in rates of HIV acquisition at one year follow-up. Findings highlight the critical role of addressing behavioral risk reduction counseling in HIV prevention. PMID:23245226

  15. Classical model for nuclear collisions including the meson degree of freedom

    International Nuclear Information System (INIS)

    Babinet, R.; Kunz, J.; Mosel, U.; Wilets, L.

    1980-01-01

    Many different approaches have been taken to describe high energy heavy ion collisions. L. Wilets et al proposed a classical treatment of the problem. In his model non-relativistic nucleons move on classical trajectories. However, the Pauli-principle is simulated by a momentum dependent potential acting between the nucleons. This model is extended in two ways. The nucleons are coupled to a pionfield, which enables us to describe inelastic processes. Nucleons and pionfiled are treated completely relativistically, this also assures Lorentz invariance. We aim at a set of classical equations of motion describing the interacting system of nucleons and pionfield. These classical equations should have a quantum mechanical basis. Further, they should contain such fundamental properties of the pion-nucleon system as the Δ(3,3)-resonance, at least in a qualitative manner. (orig./FKS)

  16. LIDT-DD: A New Self-Consistent Debris Disc Model Including Radiation Pressure and Coupling Dynamical and Collisional Evolution

    Science.gov (United States)

    Kral, Q.; Thebault, P.; Charnoz, S.

    2014-01-01

    The first attempt at developing a fully self-consistent code coupling dynamics and collisions to study debris discs (Kral et al. 2013) is presented. So far, these two crucial mechanisms were studied separately, with N-body and statistical collisional codes respectively, because of stringent computational constraints. We present a new model named LIDT-DD which is able to follow over long timescales the coupled evolution of dynamics (including radiation forces) and collisions in a self-consistent way.

  17. A methodology for including wall roughness effects in k-ε low-Reynolds turbulence models

    International Nuclear Information System (INIS)

    Ambrosini, W.; Pucciarelli, A.; Borroni, I.

    2015-01-01

    Highlights: • A model for taking into account wall roughness in low-Reynolds k-ε models is presented. • The model is subjected to a first validation to show its potential in general applications. • The application of the model in predicting heat transfer to supercritical fluids is also discussed. - Abstract: A model accounting for wall roughness effects in k-ε low-Reynolds turbulence models is described in the present paper. In particular, the introduction in the transport equations of k and ε of additional source terms related to roughness, based on simple assumptions and dimensional relationships, is proposed. An objective of the present paper, in addition to obtaining more realistic predictions of wall friction, is the application of the proposed model to the study of heat transfer to supercritical fluids. A first validation of the model is reported. The model shows the capability of predicting, at least qualitatively, some of the most important trends observed when dealing with rough pipes in very different flow conditions. Qualitative comparisons with some DNS data available in literature are also performed. Further analyses provided promising results concerning the ability of the model in reproducing the trend of friction factor when varying the flow conditions, though improvements are necessary for achieving better quantitative accuracy. First applications of the model in simulating heat transfer to supercritical fluids are also described, showing the capability of the model to affect the predictions of these heat transfer phenomena, in particular in the vicinity of the pseudo-critical conditions. A more extended application of the model to relevant deteriorated heat transfer conditions will clarify the usefulness of this modelling methodology in improving predictions of these difficult phenomena. Whatever the possible success in this particular application that motivated its development, this approach suggests a general methodology for accounting

  18. Non-local modelling of cyclic thermal shock damage including parameter estimation

    NARCIS (Netherlands)

    Damhof, F.; Brekelmans, W.A.M.; Geers, M.G.D.

    2011-01-01

    In this paper, rate dependent evolution laws are identified and characterized to model the mechanical (elasticity-based) and thermal damage occurring in coarse grain refractory material subject to cyclic thermal shock. The interacting mechanisms for elastic deformation driven damage induced by

  19. Anharmonic 1D actuator model including electrostatic and Casimir forces with fractional damping perturbed by an external force

    Science.gov (United States)

    Mansoori Kermani, Maryam; Dehestani, Maryam

    2018-06-01

    We modeled a one-dimensional actuator including the Casimir and electrostatic forces perturbed by an external force with fractional damping. The movable electrode was assumed to oscillate by an anharmonic elastic force originated from Murrell-Mottram or Lippincott potential. The nonlinear equations have been solved via the Adomian decomposition method. The behavior of the displacement of the electrode from equilibrium position, its velocity and acceleration were described versus time. Also, the changes of the displacement have been investigated according to the frequency of the external force and the voltage of the electrostatic force. The convergence of the Adomian method and the effect of the orders of expansion on the displacement versus time, frequency, and voltage were discussed. The pull-in parameter was obtained and compared with the other models in the literature. This parameter was described versus the equilibrium position and anharmonicity constant.

  20. Anharmonic 1D actuator model including electrostatic and Casimir forces with fractional damping perturbed by an external force

    Science.gov (United States)

    Mansoori Kermani, Maryam; Dehestani, Maryam

    2018-03-01

    We modeled a one-dimensional actuator including the Casimir and electrostatic forces perturbed by an external force with fractional damping. The movable electrode was assumed to oscillate by an anharmonic elastic force originated from Murrell-Mottram or Lippincott potential. The nonlinear equations have been solved via the Adomian decomposition method. The behavior of the displacement of the electrode from equilibrium position, its velocity and acceleration were described versus time. Also, the changes of the displacement have been investigated according to the frequency of the external force and the voltage of the electrostatic force. The convergence of the Adomian method and the effect of the orders of expansion on the displacement versus time, frequency, and voltage were discussed. The pull-in parameter was obtained and compared with the other models in the literature. This parameter was described versus the equilibrium position and anharmonicity constant.

  1. Loss and thermal model for power semiconductors including device rating information

    DEFF Research Database (Denmark)

    Ma, Ke; Bahman, Amir Sajjad; Beczkowski, Szymon

    2014-01-01

    The electrical loading and device rating are both important factors that determine the loss and thermal behaviors of power semiconductor devices. In the existing loss and thermal models, only the electrical loadings are focused and treated as design variables, while the device rating is normally...

  2. Including sugar cane in the agro-ecosystem model ORCHIDEE-STICS: calibration and validation

    Science.gov (United States)

    Valade, A.; Vuichard, N.; Ciais, P.; Viovy, N.

    2011-12-01

    Sugarcane is currently the most efficient bioenergy crop with regards to the energy produced per hectare. With approximately half the global bioethanol production in 2005, and a devoted land area expected to expand globally in the years to come, sugar cane is at the heart of the biofuel debate. Dynamic global vegetation models coupled with agronomical models are powerful and novel tools to tackle many of the environmental issues related to biofuels if they are carefully calibrated and validated against field observations. Here we adapt the agro-terrestrial model ORCHIDEE-STICS for sugar cane simulations. Observation data of LAI are used to evaluate the sensitivity of the model to parameters of nitrogen absorption and phenology, which are calibrated in a systematic way for six sites in Australia and La Reunion. We find that the optimal set of parameters is highly dependent on the sites' characteristics and that the model can reproduce satisfactorily the evolution of LAI. This careful calibration of ORCHIDEE-STICS for sugar cane biomass production for different locations and technical itineraries provides a strong basis for further analysis of the impacts of bioenergy-related land use change on carbon cycle budgets. As a next step, a sensitivity analysis is carried out to estimate the uncertainty of the model in biomass and carbon flux simulation due to its parameterization.

  3. Simplification and Validation of a Spectral-Tensor Model for Turbulence Including Atmospheric Stability

    Science.gov (United States)

    Chougule, Abhijit; Mann, Jakob; Kelly, Mark; Larsen, Gunner C.

    2018-02-01

    A spectral-tensor model of non-neutral, atmospheric-boundary-layer turbulence is evaluated using Eulerian statistics from single-point measurements of the wind speed and temperature at heights up to 100 m, assuming constant vertical gradients of mean wind speed and temperature. The model has been previously described in terms of the dissipation rate ɛ , the length scale of energy-containing eddies L , a turbulence anisotropy parameter Γ, the Richardson number Ri, and the normalized rate of destruction of temperature variance η _θ ≡ ɛ _θ /ɛ . Here, the latter two parameters are collapsed into a single atmospheric stability parameter z / L using Monin-Obukhov similarity theory, where z is the height above the Earth's surface, and L is the Obukhov length corresponding to Ri,η _θ. Model outputs of the one-dimensional velocity spectra, as well as cospectra of the streamwise and/or vertical velocity components, and/or temperature, and cross-spectra for the spatial separation of all three velocity components and temperature, are compared with measurements. As a function of the four model parameters, spectra and cospectra are reproduced quite well, but horizontal temperature fluxes are slightly underestimated in stable conditions. In moderately unstable stratification, our model reproduces spectra only up to a scale ˜ 1 km. The model also overestimates coherences for vertical separations, but is less severe in unstable than in stable cases.

  4. Deep Brain Stimulation for Tremor Associated with Underlying Ataxia Syndromes: A Case Series and Discussion of Issues

    Directory of Open Access Journals (Sweden)

    Genko Oyama

    2014-07-01

    Full Text Available Background: Deep brain stimulation (DBS has been utilized to treat various symptoms in patients suffering from movement disorders such as Parkinson's disease, dystonia, and essential tremor. Though ataxia syndromes have not been formally or frequently addressed with DBS, there are patients with ataxia and associated medication refractory tremor or dystonia who may potentially benefit from therapy.Methods: A retrospective database review was performed, searching for cases of ataxia where tremor and/or dystonia were addressed by utilizing DBS at the University of Florida Center for Movement Disorders and Neurorestoration between 2008 and 2011. Five patients were found who had DBS implantation to address either medication refractory tremor or dystonia. The patient's underlying diagnoses included spinocerebellar ataxia type 2 (SCA2, fragile X associated tremor ataxia syndrome (FXTAS, a case of idiopathic ataxia (ataxia not otherwise specified [NOS], spinocerebellar ataxia type 17 (SCA17, and a senataxin mutation (SETX.Results: DBS improved medication refractory tremor in the SCA2 and the ataxia NOS patients. The outcome for the FXTAS patient was poor. DBS improved dystonia in the SCA17 and SETX patients, although dystonia did not improve in the lower extremities of the SCA17 patient. All patients reported a transient gait dysfunction postoperatively, and there were no reports of improvement in ataxia‐related symptoms.Discussion: DBS may be an option to treat tremor, inclusive of dystonic tremor in patients with underlying ataxia; however, gait and other symptoms may possibly be worsened.Erratum published on July 27, 2016

  5. Design and modeling of an advanced marine machinery system including waste heat recovery and removal of sulphur oxides

    DEFF Research Database (Denmark)

    Frimann Nielsen, Rasmus; Haglind, Fredrik; Larsen, Ulrik

    2014-01-01

    the efficiency of machinery systems. The wet sulphuric acid process is an effective way of removing flue gas sulphur oxides from land-based coal-fired power plants. Moreover, organic Rankine cycles (ORC) are suitable for heat to power conversion for low temperature heat sources. This paper describes the design...... that an ORC placed after the conventional waste heat recovery system is able to extract the sulphuric acid from the exhaust gas, while at the same time increase the combined cycle thermal efficiency by 2.6%. The findings indicate that the technology has potential in marine applications regarding both energy...... and modeling of a highly efficient machinery system which includes the removal of exhaust gas sulphur oxides. The system consists of a two-stroke diesel engine, the wet sulphuric process for sulphur removal, a conventional steam Rankine cycle and an ORC. Results of numerical modeling efforts suggest...

  6. Cracked rotors. A survey on static and dynamic behaviour including modelling and diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Bachschmid, Nicolo; Pennacchi, Paolo; Tanzi, Ezio [Politecnico di Milano (Italy). Dept. of Mechanical Engineering

    2010-07-01

    Cracks can develop in rotating shafts and can propagate to relevant depths without affecting consistently the normal operating conditions of the shaft. In order to avoid catastrophic failures, accurate vibration analyses have to be performed for crack detection. The identification of the crack location and depth is possible by means of a model based diagnostic approach, provided that the model of the crack and the model of the cracked shaft dynamical behavior are accurate and reliable. This monograph shows the typical dynamical behavior of cracked shafts and presents tests for detecting cracks. The book describes how to model cracks, how to simulate the dynamical behavior of cracked shaft, and compares the corresponding numerical with experimental results. All effects of cracks on the vibrations of rotating shafts are analyzed, and some results of a numerical sensitivity analysis of the vibrations to the presence and severity of the crack are shown. Finally the book describes some crack identification procedures and shows some results in model based crack identification in position and depth. The book is useful for higher university courses in mechanical and energetic engineering, but also for skilled technical people employed in power generation industries. (orig.)

  7. Extending flood damage assessment methodology to include ...

    African Journals Online (AJOL)

    Optimal and sustainable flood plain management, including flood control, can only be achieved when the impacts of flood control measures are considered for both the man-made and natural environments, and the sociological aspects are fully considered. Until now, methods/models developed to determine the influences ...

  8. A Two-Phase Solid/Fluid Model for Dense Granular Flows Including Dilatancy Effects

    Science.gov (United States)

    Mangeney, Anne; Bouchut, Francois; Fernandez-Nieto, Enrique; Narbona-Reina, Gladys

    2015-04-01

    We propose a thin layer depth-averaged two-phase model to describe solid-fluid mixtures such as debris flows. It describes the velocity of the two phases, the compression/dilatation of the granular media and its interaction with the pore fluid pressure, that itself modifies the friction within the granular phase (Iverson et al., 2010). The model is derived from a 3D two-phase model proposed by Jackson (2000) based on the 4 equations of mass and momentum conservation within the two phases. This system has 5 unknowns: the solid and fluid velocities, the solid and fluid pressures and the solid volume fraction. As a result, an additional equation inside the mixture is necessary to close the system. Surprisingly, this issue is inadequately accounted for in the models that have been developed on the basis of Jackson's work (Bouchut et al., 2014). In particular, Pitman and Le replaced this closure simply by imposing an extra boundary condition at the surface of the flow. When making a shallow expansion, this condition can be considered as a closure condition. However, the corresponding model cannot account for a dissipative energy balance. We propose here an approach to correctly deal with the thermodynamics of Jackson's equations. We close the mixture equations by a weak compressibility relation involving a critical density, or equivalently a critical pressure. Moreover, we relax one boundary condition, making it possible for the fluid to escape the granular media when compression of the granular mass occurs. Furthermore, we introduce second order terms in the equations making it possible to describe the evolution of the pore fluid pressure in response to the compression/dilatation of the granular mass without prescribing an extra ad-hoc equation for the pore pressure. We prove that the energy balance associated with this Jackson closure is dissipative, as well as its thin layer associated model. We present several numerical tests for the 1D case that are compared to the

  9. Information to cerebellum on spinal motor networks mediated by the dorsal spinocerebellar tract

    DEFF Research Database (Denmark)

    Stecina, Katinka; Fedirchuk, Brent; Hultborn, Hans

    2013-01-01

    of peripheral sensory input to the cerebellum in general, and during rhythmic movements such as locomotion and scratch. In contrast, the VSCT was seen as conveying a copy of the output of spinal neuronal circuitry, including those circuits generating rhythmic motor activity (the spinal central pattern generator...

  10. A model for firm-specific strategic wisdom : including illustrations and 49 guiding questions

    NARCIS (Netherlands)

    van Straten, Roeland Peter

    2017-01-01

    This PhD thesis provides an answer to the question ‘How may one think strategically’. It does so by presenting a new prescriptive ‘Model for Firm-Specific Strategic Wisdom’. This Model aims to guide any individual strategist in his or her thinking from a state of firm-specific ‘ignorance’ to a state

  11. Reliability–based economic model predictive control for generalised flow–based networks including actuators’ health–aware capabilities

    Directory of Open Access Journals (Sweden)

    Grosso Juan M.

    2016-09-01

    Full Text Available This paper proposes a reliability-based economic model predictive control (MPC strategy for the management of generalised flow-based networks, integrating some ideas on network service reliability, dynamic safety stock planning, and degradation of equipment health. The proposed strategy is based on a single-layer economic optimisation problem with dynamic constraints, which includes two enhancements with respect to existing approaches. The first enhancement considers chance-constraint programming to compute an optimal inventory replenishment policy based on a desired risk acceptability level, leading to dynamical allocation of safety stocks in flow-based networks to satisfy non-stationary flow demands. The second enhancement computes a smart distribution of the control effort and maximises actuators’ availability by estimating their degradation and reliability. The proposed approach is illustrated with an application of water transport networks using the Barcelona network as the case study considered.

  12. Including local rainfall dynamics and uncertain boundary conditions into a 2-D regional-local flood modelling cascade

    Science.gov (United States)

    Bermúdez, María; Neal, Jeffrey C.; Bates, Paul D.; Coxon, Gemma; Freer, Jim E.; Cea, Luis; Puertas, Jerónimo

    2016-04-01

    Flood inundation models require appropriate boundary conditions to be specified at the limits of the domain, which commonly consist of upstream flow rate and downstream water level. These data are usually acquired from gauging stations on the river network where measured water levels are converted to discharge via a rating curve. Derived streamflow estimates are therefore subject to uncertainties in this rating curve, including extrapolating beyond the maximum observed ratings magnitude. In addition, the limited number of gauges in reach-scale studies often requires flow to be routed from the nearest upstream gauge to the boundary of the model domain. This introduces additional uncertainty, derived not only from the flow routing method used, but also from the additional lateral rainfall-runoff contributions downstream of the gauging point. Although generally assumed to have a minor impact on discharge in fluvial flood modeling, this local hydrological input may become important in a sparse gauge network or in events with significant local rainfall. In this study, a method to incorporate rating curve uncertainty and the local rainfall-runoff dynamics into the predictions of a reach-scale flood inundation model is proposed. Discharge uncertainty bounds are generated by applying a non-parametric local weighted regression approach to stage-discharge measurements for two gauging stations, while measured rainfall downstream from these locations is cascaded into a hydrological model to quantify additional inflows along the main channel. A regional simplified-physics hydraulic model is then applied to combine these inputs and generate an ensemble of discharge and water elevation time series at the boundaries of a local-scale high complexity hydraulic model. Finally, the effect of these rainfall dynamics and uncertain boundary conditions are evaluated on the local-scale model. Improvements in model performance when incorporating these processes are quantified using observed

  13. European column buckling curves and finite element modelling including high strength steels

    DEFF Research Database (Denmark)

    Jönsson, Jeppe; Stan, Tudor-Cristian

    2017-01-01

    Eurocode allows for finite element modelling of plated steel structures, however the information in the code on how to perform the analysis or what assumptions to make is quite sparse. The present paper investigates the deterministic modelling of flexural column buckling using plane shell elements...... imperfections may be very conservative if considered by finite element analysis as described in the current Eurocode code. A suggestion is given for a slightly modified imperfection formula within the Ayrton-Perry formulation leading to adequate inclusion of modern high grade steels within the original four...... bucking curves. It is also suggested that finite element or frame analysis may be performed with equivalent column bow imperfections extracted directly from the Ayrton-Perry formulation....

  14. A revised dosimetric characterization of the model S700 electronic brachytherapy source containing an anode-centering plastic insert and other components not included in the 2006 model.

    Science.gov (United States)

    Hiatt, Jessica R; Davis, Stephen D; Rivard, Mark J

    2015-06-01

    The model S700 Axxent electronic brachytherapy source by Xoft, Inc., was characterized by Rivard et al. in 2006. Since then, the source design was modified to include a new insert at the source tip. Current study objectives were to establish an accurate source model for simulation purposes, dosimetrically characterize the new source and obtain its TG-43 brachytherapy dosimetry parameters, and determine dose differences between the original simulation model and the current model S700 source design. Design information from measurements of dissected model S700 sources and from vendor-supplied CAD drawings was used to aid establishment of an updated Monte Carlo source model, which included the complex-shaped plastic source-centering insert intended to promote water flow for cooling the source anode. These data were used to create a model for subsequent radiation transport simulations in a water phantom. Compared to the 2006 simulation geometry, the influence of volume averaging close to the source was substantially reduced. A track-length estimator was used to evaluate collision kerma as a function of radial distance and polar angle for determination of TG-43 dosimetry parameters. Results for the 50 kV source were determined every 0.1 cm from 0.3 to 15 cm and every 1° from 0° to 180°. Photon spectra in water with 0.1 keV resolution were also obtained from 0.5 to 15 cm and polar angles from 0° to 165°. Simulations were run for 10(10) histories, resulting in statistical uncertainties on the transverse plane of 0.04% at r = 1 cm and 0.06% at r = 5 cm. The dose-rate distribution ratio for the model S700 source as compared to the 2006 model exceeded unity by more than 5% for roughly one quarter of the solid angle surrounding the source, i.e., θ ≥ 120°. The radial dose function diminished in a similar manner as for an (125)I seed, with values of 1.434, 0.636, 0.283, and 0.0975 at 0.5, 2, 5, and 10 cm, respectively. The radial dose function ratio between the current

  15. A stepped leader model for lightning including charge distribution in branched channels

    Energy Technology Data Exchange (ETDEWEB)

    Shi, Wei; Zhang, Li [School of Electrical Engineering, Shandong University, Jinan 250061 (China); Li, Qingmin, E-mail: lqmeee@ncepu.edu.cn [Beijing Key Lab of HV and EMC, North China Electric Power University, Beijing 102206 (China); State Key Lab of Alternate Electrical Power System with Renewable Energy Sources, Beijing 102206 (China)

    2014-09-14

    The stepped leader process in negative cloud-to-ground lightning plays a vital role in lightning protection analysis. As lightning discharge usually presents significant branched or tortuous channels, the charge distribution along the branched channels and the stochastic feature of stepped leader propagation were investigated in this paper. The charge density along the leader channel and the charge in the leader tip for each lightning branch were approximated by introducing branch correlation coefficients. In combination with geometric characteristics of natural lightning discharge, a stochastic stepped leader propagation model was presented based on the fractal theory. By comparing simulation results with the statistics of natural lightning discharges, it was found that the fractal dimension of lightning trajectory in simulation was in the range of that observed in nature and the calculation results of electric field at ground level were in good agreement with the measurements of a negative flash, which shows the validity of this proposed model. Furthermore, a new equation to estimate the lightning striking distance to flat ground was suggested based on the present model. The striking distance obtained by this new equation is smaller than the value estimated by previous equations, which indicates that the traditional equations may somewhat overestimate the attractive effect of the ground.

  16. A stepped leader model for lightning including charge distribution in branched channels

    International Nuclear Information System (INIS)

    Shi, Wei; Zhang, Li; Li, Qingmin

    2014-01-01

    The stepped leader process in negative cloud-to-ground lightning plays a vital role in lightning protection analysis. As lightning discharge usually presents significant branched or tortuous channels, the charge distribution along the branched channels and the stochastic feature of stepped leader propagation were investigated in this paper. The charge density along the leader channel and the charge in the leader tip for each lightning branch were approximated by introducing branch correlation coefficients. In combination with geometric characteristics of natural lightning discharge, a stochastic stepped leader propagation model was presented based on the fractal theory. By comparing simulation results with the statistics of natural lightning discharges, it was found that the fractal dimension of lightning trajectory in simulation was in the range of that observed in nature and the calculation results of electric field at ground level were in good agreement with the measurements of a negative flash, which shows the validity of this proposed model. Furthermore, a new equation to estimate the lightning striking distance to flat ground was suggested based on the present model. The striking distance obtained by this new equation is smaller than the value estimated by previous equations, which indicates that the traditional equations may somewhat overestimate the attractive effect of the ground.

  17. A Thermal Evolution Model of the Earth Including the Biosphere, Continental Growth and Mantle Hydration

    Science.gov (United States)

    Höning, D.; Spohn, T.

    2014-12-01

    By harvesting solar energy and converting it to chemical energy, photosynthetic life plays an important role in the energy budget of Earth [2]. This leads to alterations of chemical reservoirs eventually affecting the Earth's interior [4]. It further has been speculated [3] that the formation of continents may be a consequence of the evolution life. A steady state model [1] suggests that the Earth without its biosphere would evolve to a steady state with a smaller continent coverage and a dryer mantle than is observed today. We present a model including (i) parameterized thermal evolution, (ii) continental growth and destruction, and (iii) mantle water regassing and outgassing. The biosphere enhances the production rate of sediments which eventually are subducted. These sediments are assumed to (i) carry water to depth bound in stable mineral phases and (ii) have the potential to suppress shallow dewatering of the underlying sediments and crust due to their low permeability. We run a Monte Carlo simulation for various initial conditions and treat all those parameter combinations as success which result in the fraction of continental crust coverage observed for present day Earth. Finally, we simulate the evolution of an abiotic Earth using the same set of parameters but a reduced rate of continental weathering and erosion. Our results suggest that the origin and evolution of life could have stabilized the large continental surface area of the Earth and its wet mantle, leading to the relatively low mantle viscosity we observe at present. Without photosynthetic life on our planet, the Earth would be geodynamical less active due to a dryer mantle, and would have a smaller fraction of continental coverage than observed today. References[1] Höning, D., Hansen-Goos, H., Airo, A., Spohn, T., 2014. Biotic vs. abiotic Earth: A model for mantle hydration and continental coverage. Planetary and Space Science 98, 5-13. [2] Kleidon, A., 2010. Life, hierarchy, and the

  18. [Degenerative cerebellar diseases and differential diagnoses].

    Science.gov (United States)

    Reith, W; Roumia, S; Dietrich, P

    2016-11-01

    Cerebellar syndromes result in distinct clinical symptoms, such as ataxia, dysarthria, dysmetria, intention tremor and eye movement disorders. In addition to the medical history and clinical examination, imaging is particularly important to differentiate other diseases, such as hydrocephalus and multi-infarct dementia from degenerative cerebellar diseases. Degenerative diseases with cerebellar involvement include Parkinson's disease, multiple system atrophy as well as other diseases including spinocerebellar ataxia. In addition to magnetic resonance imaging (MRI), nuclear medicine imaging investigations are also helpful for the differentiation. Axial fluid-attenuated inversion recovery (FLAIR) and T2-weighted sequences can sometimes show a signal increase in the pons as a sign of degeneration of pontine neurons and transverse fibers in the basilar part of the pons. The imaging is particularly necessary to exclude other diseases, such as normal pressure hydrocephalus (NPH), multi-infarct dementia and cerebellar lesions.

  19. Group-Level EEG-Processing Pipeline for Flexible Single Trial-Based Analyses Including Linear Mixed Models.

    Science.gov (United States)

    Frömer, Romy; Maier, Martin; Abdel Rahman, Rasha

    2018-01-01

    Here we present an application of an EEG processing pipeline customizing EEGLAB and FieldTrip functions, specifically optimized to flexibly analyze EEG data based on single trial information. The key component of our approach is to create a comprehensive 3-D EEG data structure including all trials and all participants maintaining the original order of recording. This allows straightforward access to subsets of the data based on any information available in a behavioral data structure matched with the EEG data (experimental conditions, but also performance indicators, such accuracy or RTs of single trials). In the present study we exploit this structure to compute linear mixed models (LMMs, using lmer in R) including random intercepts and slopes for items. This information can easily be read out from the matched behavioral data, whereas it might not be accessible in traditional ERP approaches without substantial effort. We further provide easily adaptable scripts for performing cluster-based permutation tests (as implemented in FieldTrip), as a more robust alternative to traditional omnibus ANOVAs. Our approach is particularly advantageous for data with parametric within-subject covariates (e.g., performance) and/or multiple complex stimuli (such as words, faces or objects) that vary in features affecting cognitive processes and ERPs (such as word frequency, salience or familiarity), which are sometimes hard to control experimentally or might themselves constitute variables of interest. The present dataset was recorded from 40 participants who performed a visual search task on previously unfamiliar objects, presented either visually intact or blurred. MATLAB as well as R scripts are provided that can be adapted to different datasets.

  20. Including gauge corrections to thermal leptogenesis

    International Nuclear Information System (INIS)

    Huetig, Janine

    2013-01-01

    This thesis provides the first approach of a systematic inclusion of gauge corrections to leading order to the ansatz of thermal leptogenesis. We have derived a complete expression for the integrated lepton number matrix including all resummations needed. For this purpose, a new class of diagram has been invented, namely the cylindrical diagram, which allows diverse investigations into the topic of leptogenesis such as the case of resonant leptogenesis. After a brief introduction of the topic of the baryon asymmetry in the universe and a discussion of its most promising solutions as well as their advantages and disadvantages, we have presented our framework of thermal leptogenesis. An effective model was described as well as the associated Feynman rules. The basis for using nonequilibrium quantum field theory has been built in chapter 3. At first, the main definitions have been presented for equilibrium thermal field theory, afterwards we have discussed the Kadanoff-Baym equations for systems out of equilibrium using the example of the Majorana neutrino. The equations have also been solved in the context of leptogenesis in chapter 4. Since gauge corrections play a crucial role throughout this thesis, we have also repeated the naive ansatz by replacing the free equilibrium propagator by propagators including thermal damping rates due to the Standard Model damping widths for lepton and Higgs fields. It is shown that this leads to a comparable result to the solutions of the Boltzmann equations for thermal leptogenesis. Thus it becomes obvious that Standard Model corrections are not negligible for thermal leptogenesis and therefore need to be included systematically from first principles. In order to achieve this we have started discussing the calculation of ladder rung diagrams for Majorana neutrinos using the HTL and the CTL approach in chapter 5. All gauge corrections are included in this framework and thus it has become the basis for the following considerations

  1. Including gauge corrections to thermal leptogenesis

    Energy Technology Data Exchange (ETDEWEB)

    Huetig, Janine

    2013-05-17

    This thesis provides the first approach of a systematic inclusion of gauge corrections to leading order to the ansatz of thermal leptogenesis. We have derived a complete expression for the integrated lepton number matrix including all resummations needed. For this purpose, a new class of diagram has been invented, namely the cylindrical diagram, which allows diverse investigations into the topic of leptogenesis such as the case of resonant leptogenesis. After a brief introduction of the topic of the baryon asymmetry in the universe and a discussion of its most promising solutions as well as their advantages and disadvantages, we have presented our framework of thermal leptogenesis. An effective model was described as well as the associated Feynman rules. The basis for using nonequilibrium quantum field theory has been built in chapter 3. At first, the main definitions have been presented for equilibrium thermal field theory, afterwards we have discussed the Kadanoff-Baym equations for systems out of equilibrium using the example of the Majorana neutrino. The equations have also been solved in the context of leptogenesis in chapter 4. Since gauge corrections play a crucial role throughout this thesis, we have also repeated the naive ansatz by replacing the free equilibrium propagator by propagators including thermal damping rates due to the Standard Model damping widths for lepton and Higgs fields. It is shown that this leads to a comparable result to the solutions of the Boltzmann equations for thermal leptogenesis. Thus it becomes obvious that Standard Model corrections are not negligible for thermal leptogenesis and therefore need to be included systematically from first principles. In order to achieve this we have started discussing the calculation of ladder rung diagrams for Majorana neutrinos using the HTL and the CTL approach in chapter 5. All gauge corrections are included in this framework and thus it has become the basis for the following considerations

  2. Modelling the effect of acoustic waves on the thermodynamics and kinetics of phase transformation in a solution: Including mass transportation.

    Science.gov (United States)

    Haqshenas, S R; Ford, I J; Saffari, N

    2018-01-14

    Effects of acoustic waves on a phase transformation in a metastable phase were investigated in our previous work [S. R. Haqshenas, I. J. Ford, and N. Saffari, "Modelling the effect of acoustic waves on nucleation," J. Chem. Phys. 145, 024315 (2016)]. We developed a non-equimolar dividing surface cluster model and employed it to determine the thermodynamics and kinetics of crystallisation induced by an acoustic field in a mass-conserved system. In the present work, we developed a master equation based on a hybrid Szilard-Fokker-Planck model, which accounts for mass transportation due to acoustic waves. This model can determine the kinetics of nucleation and the early stage of growth of clusters including the Ostwald ripening phenomenon. It was solved numerically to calculate the kinetics of an isothermal sonocrystallisation process in a system with mass transportation. The simulation results show that the effect of mass transportation for different excitations depends on the waveform as well as the imposed boundary conditions and tends to be noticeable in the case of shock waves. The derivations are generic and can be used with any acoustic source and waveform.

  3. Evaluation of the 235U prompt fission neutron spectrum including a detailed analysis of experimental data and improved model information

    Science.gov (United States)

    Neudecker, Denise; Talou, Patrick; Kahler, Albert C.; White, Morgan C.; Kawano, Toshihiko

    2017-09-01

    We present an evaluation of the 235U prompt fission neutron spectrum (PFNS) induced by thermal to 20-MeV neutrons. Experimental data and associated covariances were analyzed in detail. The incident energy dependence of the PFNS was modeled with an extended Los Alamos model combined with the Hauser-Feshbach and the exciton models. These models describe prompt fission, pre-fission compound nucleus and pre-equilibrium neutron emissions. The evaluated PFNS agree well with the experimental data included in this evaluation, preliminary data of the LANL and LLNL Chi-Nu measurement and recent evaluations by Capote et al. and Rising et al. However, they are softer than the ENDF/B-VII.1 (VII.1) and JENDL-4.0 PFNS for incident neutron energies up to 2 MeV. Simulated effective multiplication factors keff of the Godiva and Flattop-25 critical assemblies are further from the measured keff if the current data are used within VII.1 compared to using only VII.1 data. However, if this work is used with ENDF/B-VIII.0β2 data, simulated values of keff agree well with the measured ones.

  4. Framework of cloud parameterization including ice for 3-D mesoscale models

    Energy Technology Data Exchange (ETDEWEB)

    Levkov, L; Jacob, D; Eppel, D; Grassl, H

    1989-01-01

    A parameterization scheme for the simulation of ice in clouds incorporated into the hydrostatic version of the GKSS three-dimensional mesoscale model. Numerical simulations of precipitation are performed: over the Northe Sea, the Hawaiian trade wind area and in the region of the intertropical convergence zone. Not only some major features of convective structures in all three areas but also cloud-aerosol interactions have successfully been simulated. (orig.) With 19 figs., 2 tabs.

  5. Autosomal dominant hereditary ataxia in Sri Lanka

    OpenAIRE

    Sumathipala, Dulika S; Abeysekera, Gayan S; Jayasekara, Rohan W; Tallaksen, Chantal ME; Dissanayake, Vajira HW

    2013-01-01

    Background Spinocerebellar ataxias (SCA) are a group of hereditary neurodegenerative disorders. Prevalence of SCA subtypes differ worldwide. Autosomal dominant ataxias are the commonest types of inherited ataxias seen in Sri Lanka. The aim of the study is to determine the genetic etiology of patients with autosomal dominant ataxia in Sri Lanka and to describe the clinical features of each genetic subtype. Methods ...

  6. Triplet repeat DNA structures and human genetic disease: dynamic ...

    Indian Academy of Sciences (India)

    Unknown

    formed at the loop-outs. [Sinden R R, Potaman V N, Oussatcheva E A, Pearson C E, Lyubchenko Y L and Shlyakhtenko L S 2002 Triplet repeat DNA structures .... 36–39. 40–121 Huntingtin/polyglutamine expansion. Spinocerebellar ataxia 1. SCA1. 6p23. (CAG)n. 6–44. –. 39–82 (pure) Ataxin-1/polyglutamine expansion.

  7. Should All Patients Be Included in Alternative Payment Models for Primary Total Hip Arthroplasty and Total Knee Arthroplasty?

    Science.gov (United States)

    Rozell, Joshua C; Courtney, Paul M; Dattilo, Jonathan R; Wu, Chia H; Lee, Gwo-Chin

    2016-09-01

    Alternative payment models in total joint replacement incentivize cost effective health care delivery and reward reductions in length of stay (LOS), complications, and readmissions. If not adjusted for patient comorbidities, they may encourage restrictive access to health care. We prospectively evaluated 802 consecutive primary total hip arthroplasty and total knee arthroplasty patients evaluating comorbidities associated with increased LOS and readmissions. During this 9-month period, 115 patients (14.3%) required hospitalization >3 days and 16 (1.99%) were readmitted within 90 days. Univariate analysis demonstrated that preoperative narcotic use, heart failure, stroke, chronic kidney disease (CKD), chronic obstructive pulmonary disease (COPD), and liver disease were more likely to require hospitalization >3 days. In multivariate analysis, CKD and COPD were independent risk factors for LOS >3 days. A Charlson comorbidity index >5 points was associated with increased LOS and readmissions. Patients with CKD, COPD, and Charlson comorbidity index >5 points should not be included in alternative payment model for THA and TKA. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. A Two-Account Life Insurance Model for Scenario-Based Valuation Including Event Risk

    DEFF Research Database (Denmark)

    Jensen, Ninna Reitzel; Schomacker, Kristian Juul

    2015-01-01

    Using a two-account model with event risk, we model life insurance contracts taking into account both guaranteed and non-guaranteed payments in participating life insurance as well as in unit-linked insurance. Here, event risk is used as a generic term for life insurance events, such as death......, disability, etc. In our treatment of participating life insurance, we have special focus on the bonus schemes “consolidation” and “additional benefits”, and one goal is to formalize how these work and interact. Another goal is to describe similarities and differences between participating life insurance...... product types. This enables comparison of participating life insurance products and unit-linked insurance products, thus building a bridge between the two different ways of formalizing life insurance products. Finally, our model distinguishes itself from the existing literature by taking into account...

  9. A revised dosimetric characterization of the model S700 electronic brachytherapy source containing an anode-centering plastic insert and other components not included in the 2006 model

    International Nuclear Information System (INIS)

    Hiatt, Jessica R.; Davis, Stephen D.; Rivard, Mark J.

    2015-01-01

    Purpose: The model S700 Axxent electronic brachytherapy source by Xoft, Inc., was characterized by Rivard et al. in 2006. Since then, the source design was modified to include a new insert at the source tip. Current study objectives were to establish an accurate source model for simulation purposes, dosimetrically characterize the new source and obtain its TG-43 brachytherapy dosimetry parameters, and determine dose differences between the original simulation model and the current model S700 source design. Methods: Design information from measurements of dissected model S700 sources and from vendor-supplied CAD drawings was used to aid establishment of an updated Monte Carlo source model, which included the complex-shaped plastic source-centering insert intended to promote water flow for cooling the source anode. These data were used to create a model for subsequent radiation transport simulations in a water phantom. Compared to the 2006 simulation geometry, the influence of volume averaging close to the source was substantially reduced. A track-length estimator was used to evaluate collision kerma as a function of radial distance and polar angle for determination of TG-43 dosimetry parameters. Results for the 50 kV source were determined every 0.1 cm from 0.3 to 15 cm and every 1° from 0° to 180°. Photon spectra in water with 0.1 keV resolution were also obtained from 0.5 to 15 cm and polar angles from 0° to 165°. Simulations were run for 10 10 histories, resulting in statistical uncertainties on the transverse plane of 0.04% at r = 1 cm and 0.06% at r = 5 cm. Results: The dose-rate distribution ratio for the model S700 source as compared to the 2006 model exceeded unity by more than 5% for roughly one quarter of the solid angle surrounding the source, i.e., θ ≥ 120°. The radial dose function diminished in a similar manner as for an 125 I seed, with values of 1.434, 0.636, 0.283, and 0.0975 at 0.5, 2, 5, and 10 cm, respectively. The radial dose function

  10. A revised dosimetric characterization of the model S700 electronic brachytherapy source containing an anode-centering plastic insert and other components not included in the 2006 model

    Energy Technology Data Exchange (ETDEWEB)

    Hiatt, Jessica R. [Department of Radiation Oncology, Rhode Island Hospital, The Warren Alpert Medical School of Brown University, Providence, Rhode Island 02903 (United States); Davis, Stephen D. [Department of Medical Physics, McGill University Health Centre, Montreal, Quebec H3G 1A4 (Canada); Rivard, Mark J., E-mail: mark.j.rivard@gmail.com [Department of Radiation Oncology, Tufts University School of Medicine, Boston, Massachusetts 02111 (United States)

    2015-06-15

    Purpose: The model S700 Axxent electronic brachytherapy source by Xoft, Inc., was characterized by Rivard et al. in 2006. Since then, the source design was modified to include a new insert at the source tip. Current study objectives were to establish an accurate source model for simulation purposes, dosimetrically characterize the new source and obtain its TG-43 brachytherapy dosimetry parameters, and determine dose differences between the original simulation model and the current model S700 source design. Methods: Design information from measurements of dissected model S700 sources and from vendor-supplied CAD drawings was used to aid establishment of an updated Monte Carlo source model, which included the complex-shaped plastic source-centering insert intended to promote water flow for cooling the source anode. These data were used to create a model for subsequent radiation transport simulations in a water phantom. Compared to the 2006 simulation geometry, the influence of volume averaging close to the source was substantially reduced. A track-length estimator was used to evaluate collision kerma as a function of radial distance and polar angle for determination of TG-43 dosimetry parameters. Results for the 50 kV source were determined every 0.1 cm from 0.3 to 15 cm and every 1° from 0° to 180°. Photon spectra in water with 0.1 keV resolution were also obtained from 0.5 to 15 cm and polar angles from 0° to 165°. Simulations were run for 10{sup 10} histories, resulting in statistical uncertainties on the transverse plane of 0.04% at r = 1 cm and 0.06% at r = 5 cm. Results: The dose-rate distribution ratio for the model S700 source as compared to the 2006 model exceeded unity by more than 5% for roughly one quarter of the solid angle surrounding the source, i.e., θ ≥ 120°. The radial dose function diminished in a similar manner as for an {sup 125}I seed, with values of 1.434, 0.636, 0.283, and 0.0975 at 0.5, 2, 5, and 10 cm, respectively. The radial dose

  11. Including crystal structure attributes in machine learning models of formation energies via Voronoi tessellations

    Science.gov (United States)

    Ward, Logan; Liu, Ruoqian; Krishna, Amar; Hegde, Vinay I.; Agrawal, Ankit; Choudhary, Alok; Wolverton, Chris

    2017-07-01

    While high-throughput density functional theory (DFT) has become a prevalent tool for materials discovery, it is limited by the relatively large computational cost. In this paper, we explore using DFT data from high-throughput calculations to create faster, surrogate models with machine learning (ML) that can be used to guide new searches. Our method works by using decision tree models to map DFT-calculated formation enthalpies to a set of attributes consisting of two distinct types: (i) composition-dependent attributes of elemental properties (as have been used in previous ML models of DFT formation energies), combined with (ii) attributes derived from the Voronoi tessellation of the compound's crystal structure. The ML models created using this method have half the cross-validation error and similar training and evaluation speeds to models created with the Coulomb matrix and partial radial distribution function methods. For a dataset of 435 000 formation energies taken from the Open Quantum Materials Database (OQMD), our model achieves a mean absolute error of 80 meV/atom in cross validation, which is lower than the approximate error between DFT-computed and experimentally measured formation enthalpies and below 15% of the mean absolute deviation of the training set. We also demonstrate that our method can accurately estimate the formation energy of materials outside of the training set and be used to identify materials with especially large formation enthalpies. We propose that our models can be used to accelerate the discovery of new materials by identifying the most promising materials to study with DFT at little additional computational cost.

  12. A biologically inspired neural model for visual and proprioceptive integration including sensory training.

    Science.gov (United States)

    Saidi, Maryam; Towhidkhah, Farzad; Gharibzadeh, Shahriar; Lari, Abdolaziz Azizi

    2013-12-01

    Humans perceive the surrounding world by integration of information through different sensory modalities. Earlier models of multisensory integration rely mainly on traditional Bayesian and causal Bayesian inferences for single causal (source) and two causal (for two senses such as visual and auditory systems), respectively. In this paper a new recurrent neural model is presented for integration of visual and proprioceptive information. This model is based on population coding which is able to mimic multisensory integration of neural centers in the human brain. The simulation results agree with those achieved by casual Bayesian inference. The model can also simulate the sensory training process of visual and proprioceptive information in human. Training process in multisensory integration is a point with less attention in the literature before. The effect of proprioceptive training on multisensory perception was investigated through a set of experiments in our previous study. The current study, evaluates the effect of both modalities, i.e., visual and proprioceptive training and compares them with each other through a set of new experiments. In these experiments, the subject was asked to move his/her hand in a circle and estimate its position. The experiments were performed on eight subjects with proprioception training and eight subjects with visual training. Results of the experiments show three important points: (1) visual learning rate is significantly more than that of proprioception; (2) means of visual and proprioceptive errors are decreased by training but statistical analysis shows that this decrement is significant for proprioceptive error and non-significant for visual error, and (3) visual errors in training phase even in the beginning of it, is much less than errors of the main test stage because in the main test, the subject has to focus on two senses. The results of the experiments in this paper is in agreement with the results of the neural model

  13. Genetics Home Reference: spinocerebellar ataxia type 6

    Science.gov (United States)

    ... CaV2.1 channels play an essential role in communication between neurons in the brain. The CACNA1A gene mutations that ... calcium channels in the cell membrane impairs cell communication between neurons in the brain. Diminished cell communication leads to ...

  14. Genetics Home Reference: spinocerebellar ataxia type 3

    Science.gov (United States)

    ... time, individuals with SCA3 may develop loss of sensation and weakness in the limbs (peripheral neuropathy), muscle cramps, muscle twitches (fasciculations), and swallowing difficulties. Individuals with SCA3 may have problems with memory, planning, and problem solving. Signs and symptoms of ...

  15. Genetic testing for clinically suspected spinocerebellar ataxias ...

    Indian Academy of Sciences (India)

    Mahesh

    Research Article. Genetic ... Melbourne, Australia and Department of Animal Science, School of Life .... The patients were assessed according to the International Cooperative Ataxia Rating ..... The Indian Journal of Medical Research 126(5):.

  16. Genetic testing for clinically suspected spinocerebellar ataxias ...

    Indian Academy of Sciences (India)

    Sowmya Devatha Venkatesh

    2018-03-13

    Mar 13, 2018 ... 5 Department of Psychology, Florida State University, Tallahassee, FL 32304, USA ... 2000; Sinha et al. 2004). Most epidemiological studies on the preva- ... tion may be due to a very recent admixture event (Singh et al. 2010).

  17. Genetics Home Reference: spinocerebellar ataxia type 1

    Science.gov (United States)

    ... an earlier onset of signs and symptoms. This phenomenon is called anticipation. Anticipation tends to be more ... As the gene is passed from parent to child, the size of the CAG trinucleotide repeat may ...

  18. Genetics Home Reference: spinocerebellar ataxia type 2

    Science.gov (United States)

    ... Almira YR, Zaldivar YG, Almarales DC, Góngora EM, Herrera MP, Batallán KE, Armiñán RR, Manresa MV, Cruz ... Falcón NS, Góngora EM, Almarales DC, Pérez LV, Herrera MP. Age-dependent risks in genetic counseling for ...

  19. DEVELOPMENT OF A HYDRODYNAMIC MODEL OF A HYDROCYCLONE INCLUDING THE SIMULATION OF AIR-CORE EFFECT, USING THE FINITE VOLUME METHOD

    Directory of Open Access Journals (Sweden)

    Gabriel Felipe Aguilera

    2014-07-01

    Full Text Available The hydrocyclone is one of the most used classification equipment in industry, particularly in mineral processing. Maybe its main characteristic is to be a hydrodynamic separation equipment, whereby it has a high production capability and different levels of efficiency are depending on the geometrical configuration, operational parameters and the type of material to be processed. Nevertheless, there are a few successful studies regarding the modelling and simulation of its hydrodynamic principles, because the flow behavior inside is quite complex. Most of the current models are empirical and they are not applicable to all cases and types of minerals. One of the most important problems to be solved, besides the cut size and the effect of the physical properties of the particles, is the distribution of the flow inside the hydrocyclone, because if the work of the equipment is at low slurry densities, very clear for small hydrocyclones, its mechanic behavior is a consequence of the kind of liquid used as continuous phase, being water the most common liquid. This work shows the modelling and simulation of the hydrodynamic behavior of a suspension inside a hydrocyclone, including the air core effect, through the use of finite differences method. For the developing of the model, the Reynolds Stress Model (RSM for the evaluation of turbulence, and the Volume of Fluid (VOF to study the interaction between water and air were used. Finally, the model shows to be significant for experimental data, and for different conditions of an industrial plant.

  20. Modeling and Control of Risley Prism Beam Steering Including BLDC Motors

    OpenAIRE

    Gunnarsson, Oscar

    2016-01-01

    Saab AB Training & Simulation is specialized on military training, including laserbased training. To continue being the world leader in this area, a new generationof laser simulators needs to be developed. To simplify the development of thishighly complex system, this master thesis have resulted in a MATLAB/Simulinkmodel which simulates the electro-opto/mechanical system representing theirlaser based simulation platform. The focus of this master thesis has been to simulateand control the ...

  1. Analysis of shallow water experimental acoustic data including normal mode model comparisons

    NARCIS (Netherlands)

    McHugh, R.; Simons, D.G.

    2000-01-01

    Ss part of a propagation model validation exercise experimental acoustic and oceanographic data was collected from a shallow-water, long-range channel, off the west coast of Scotland. Temporal variability effects in this channel were assessed through visual inspection of stacked plots, each of which

  2. HIV Model Parameter Estimates from Interruption Trial Data including Drug Efficacy and Reservoir Dynamics

    Science.gov (United States)

    Luo, Rutao; Piovoso, Michael J.; Martinez-Picado, Javier; Zurakowski, Ryan

    2012-01-01

    Mathematical models based on ordinary differential equations (ODE) have had significant impact on understanding HIV disease dynamics and optimizing patient treatment. A model that characterizes the essential disease dynamics can be used for prediction only if the model parameters are identifiable from clinical data. Most previous parameter identification studies for HIV have used sparsely sampled data from the decay phase following the introduction of therapy. In this paper, model parameters are identified from frequently sampled viral-load data taken from ten patients enrolled in the previously published AutoVac HAART interruption study, providing between 69 and 114 viral load measurements from 3–5 phases of viral decay and rebound for each patient. This dataset is considerably larger than those used in previously published parameter estimation studies. Furthermore, the measurements come from two separate experimental conditions, which allows for the direct estimation of drug efficacy and reservoir contribution rates, two parameters that cannot be identified from decay-phase data alone. A Markov-Chain Monte-Carlo method is used to estimate the model parameter values, with initial estimates obtained using nonlinear least-squares methods. The posterior distributions of the parameter estimates are reported and compared for all patients. PMID:22815727

  3. Adult Mouse DRG Explant and Dissociated Cell Models to Investigate Neuroplasticity and Responses to Environmental Insults Including Viral Infection.

    Science.gov (United States)

    Fornaro, Michele; Sharthiya, Harsh; Tiwari, Vaibhav

    2018-03-09

    This protocol describes an ex vivo model of mouse-derived dorsal root ganglia (DRG) explant and in vitro DRG-derived co-culture of dissociated sensory neurons and glial satellite cells. These are useful and versatile models to investigate a variety of biological responses associated with physiological and pathological conditions of the peripheral nervous system (PNS) ranging from neuron-glial interaction, neuroplasticity, neuroinflammation, and viral infection. The usage of DRG explant is scientifically advantageous compared to simplistic single cells models for multiple reasons. For instance, as an organotypic culture, the DRG explant allows ex vivo transfer of an entire neuronal network including the extracellular microenvironment that play a significant role in all the neuronal and glial functions. Further, DRG explants can also be maintained ex vivo for several days and the culture conditions can be perturbed as desired. In addition, the harvested DRG can be further dissociated into an in vitro co-culture of primary sensory neurons and satellite glial cells to investigate neuronal-glial interaction, neuritogenesis, axonal cone interaction with the extracellular microenvironment, and more general, any aspect associated with the neuronal metabolism. Therefore, the DRG-explant system offers a great deal of flexibility to study a wide array of events related to biological, physiological, and pathological conditions in a cost-effective manner.

  4. Dusty Plasma Modeling of the Fusion Reactor Sheath Including Collisional-Radiative Effects

    International Nuclear Information System (INIS)

    Dezairi, Aouatif; Samir, Mhamed; Eddahby, Mohamed; Saifaoui, Dennoun; Katsonis, Konstantinos; Berenguer, Chloe

    2008-01-01

    The structure and the behavior of the sheath in Tokamak collisional plasmas has been studied. The sheath is modeled taking into account the presence of the dust 2 and the effects of the charged particle collisions and radiative processes. The latter may allow for optical diagnostics of the plasma.

  5. 3D numerical surface charge model including relative permeability : the general theory

    NARCIS (Netherlands)

    Casteren, van D.T.E.H.; Paulides, J.J.H.; Lomonova, E.A.

    2014-01-01

    One of the still "open" issues within low-frequency magnetics is the inclusion of µr in the calculations using the magnetic charge method. In this paper a new iterative method to take the relative permeability into account is investigated. Results show that the model accurately accounts for the

  6. A two-dimensional simulation model of phosphorus uptake including crop growth and P-response

    NARCIS (Netherlands)

    Mollier, A.; Willigen, de P.; Heinen, M.; Morel, C.; Schneider, A.; Pellerin, S.

    2008-01-01

    Modelling nutrient uptake by crops implies considering and integrating the processes controlling the soil nutrient supply, the uptake by the root system and relationships between the crop growth response and the amount of nutrient absorbed. We developed a model that integrates both dynamics of maize

  7. Areal rainfall estimation using moving cars - computer experiments including hydrological modeling

    Science.gov (United States)

    Rabiei, Ehsan; Haberlandt, Uwe; Sester, Monika; Fitzner, Daniel; Wallner, Markus

    2016-09-01

    The need for high temporal and spatial resolution precipitation data for hydrological analyses has been discussed in several studies. Although rain gauges provide valuable information, a very dense rain gauge network is costly. As a result, several new ideas have emerged to help estimating areal rainfall with higher temporal and spatial resolution. Rabiei et al. (2013) observed that moving cars, called RainCars (RCs), can potentially be a new source of data for measuring rain rate. The optical sensors used in that study are designed for operating the windscreen wipers and showed promising results for rainfall measurement purposes. Their measurement accuracy has been quantified in laboratory experiments. Considering explicitly those errors, the main objective of this study is to investigate the benefit of using RCs for estimating areal rainfall. For that, computer experiments are carried out, where radar rainfall is considered as the reference and the other sources of data, i.e., RCs and rain gauges, are extracted from radar data. Comparing the quality of areal rainfall estimation by RCs with rain gauges and reference data helps to investigate the benefit of the RCs. The value of this additional source of data is not only assessed for areal rainfall estimation performance but also for use in hydrological modeling. Considering measurement errors derived from laboratory experiments, the result shows that the RCs provide useful additional information for areal rainfall estimation as well as for hydrological modeling. Moreover, by testing larger uncertainties for RCs, they observed to be useful up to a certain level for areal rainfall estimation and discharge simulation.

  8. Results of including geometric nonlinearities in an aeroelastic model of an F/A-18

    Science.gov (United States)

    Buttrill, Carey S.

    1989-01-01

    An integrated, nonlinear simulation model suitable for aeroelastic modeling of fixed-wing aircraft has been developed. While the author realizes that the subject of modeling rotating, elastic structures is not closed, it is believed that the equations of motion developed and applied herein are correct to second order and are suitable for use with typical aircraft structures. The equations are not suitable for large elastic deformation. In addition, the modeling framework generalizes both the methods and terminology of non-linear rigid-body airplane simulation and traditional linear aeroelastic modeling. Concerning the importance of angular/elastic inertial coupling in the dynamic analysis of fixed-wing aircraft, the following may be said. The rigorous inclusion of said coupling is not without peril and must be approached with care. In keeping with the same engineering judgment that guided the development of the traditional aeroelastic equations, the effect of non-linear inertial effects for most airplane applications is expected to be small. A parameter does not tell the whole story, however, and modes flagged by the parameter as significant also need to be checked to see if the coupling is not a one-way path, i.e., the inertially affected modes can influence other modes.

  9. A modified Poisson-Boltmann model including charge regulation for the adsorption of ionizable polyelectrolytes to charged interfaces, applied to lysozyme adsorption on silica

    NARCIS (Netherlands)

    Biesheuvel, P.M.; Veen, van der M.; Norde, W.

    2005-01-01

    The equilibrium adsorption of polyelectrolytes with multiple types of ionizable groups is described using a modified Poisson-Boltzmann equation including charge regulation of both the polymer and the interface. A one-dimensional mean-field model is used in which the electrostatic potential is

  10. Large Eddy simulation of turbulence: A subgrid scale model including shear, vorticity, rotation, and buoyancy

    Science.gov (United States)

    Canuto, V. M.

    1994-01-01

    The Reynolds numbers that characterize geophysical and astrophysical turbulence (Re approximately equals 10(exp 8) for the planetary boundary layer and Re approximately equals 10(exp 14) for the Sun's interior) are too large to allow a direct numerical simulation (DNS) of the fundamental Navier-Stokes and temperature equations. In fact, the spatial number of grid points N approximately Re(exp 9/4) exceeds the computational capability of today's supercomputers. Alternative treatments are the ensemble-time average approach, and/or the volume average approach. Since the first method (Reynolds stress approach) is largely analytical, the resulting turbulence equations entail manageable computational requirements and can thus be linked to a stellar evolutionary code or, in the geophysical case, to general circulation models. In the volume average approach, one carries out a large eddy simulation (LES) which resolves numerically the largest scales, while the unresolved scales must be treated theoretically with a subgrid scale model (SGS). Contrary to the ensemble average approach, the LES+SGS approach has considerable computational requirements. Even if this prevents (for the time being) a LES+SGS model to be linked to stellar or geophysical codes, it is still of the greatest relevance as an 'experimental tool' to be used, inter alia, to improve the parameterizations needed in the ensemble average approach. Such a methodology has been successfully adopted in studies of the convective planetary boundary layer. Experienc e with the LES+SGS approach from different fields has shown that its reliability depends on the healthiness of the SGS model for numerical stability as well as for physical completeness. At present, the most widely used SGS model, the Smagorinsky model, accounts for the effect of the shear induced by the large resolved scales on the unresolved scales but does not account for the effects of buoyancy, anisotropy, rotation, and stable stratification. The

  11. Extending Galactic Habitable Zone Modeling to Include the Emergence of Intelligent Life.

    Science.gov (United States)

    Morrison, Ian S; Gowanlock, Michael G

    2015-08-01

    Previous studies of the galactic habitable zone have been concerned with identifying those regions of the Galaxy that may favor the emergence of complex life. A planet is deemed habitable if it meets a set of assumed criteria for supporting the emergence of such complex life. In this work, we extend the assessment of habitability to consider the potential for life to further evolve to the point of intelligence--termed the propensity for the emergence of intelligent life, φI. We assume φI is strongly influenced by the time durations available for evolutionary processes to proceed undisturbed by the sterilizing effects of nearby supernovae. The times between supernova events provide windows of opportunity for the evolution of intelligence. We developed a model that allows us to analyze these window times to generate a metric for φI, and we examine here the spatial and temporal variation of this metric. Even under the assumption that long time durations are required between sterilizations to allow for the emergence of intelligence, our model suggests that the inner Galaxy provides the greatest number of opportunities for intelligence to arise. This is due to the substantially higher number density of habitable planets in this region, which outweighs the effects of a higher supernova rate in the region. Our model also shows that φI is increasing with time. Intelligent life emerged at approximately the present time at Earth's galactocentric radius, but a similar level of evolutionary opportunity was available in the inner Galaxy more than 2 Gyr ago. Our findings suggest that the inner Galaxy should logically be a prime target region for searches for extraterrestrial intelligence and that any civilizations that may have emerged there are potentially much older than our own.

  12. Nuclear Reactor/Hydrogen Process Interface Including the HyPEP Model

    International Nuclear Information System (INIS)

    Steven R. Sherman

    2007-01-01

    The Nuclear Reactor/Hydrogen Plant interface is the intermediate heat transport loop that will connect a very high temperature gas-cooled nuclear reactor (VHTR) to a thermochemical, high-temperature electrolysis, or hybrid hydrogen production plant. A prototype plant called the Next Generation Nuclear Plant (NGNP) is planned for construction and operation at the Idaho National Laboratory in the 2018-2021 timeframe, and will involve a VHTR, a high-temperature interface, and a hydrogen production plant. The interface is responsible for transporting high-temperature thermal energy from the nuclear reactor to the hydrogen production plant while protecting the nuclear plant from operational disturbances at the hydrogen plant. Development of the interface is occurring under the DOE Nuclear Hydrogen Initiative (NHI) and involves the study, design, and development of high-temperature heat exchangers, heat transport systems, materials, safety, and integrated system models. Research and development work on the system interface began in 2004 and is expected to continue at least until the start of construction of an engineering-scale demonstration plant

  13. Double pendulum model for a tennis stroke including a collision process

    Science.gov (United States)

    Youn, Sun-Hyun

    2015-10-01

    By means of adding a collision process between the ball and racket in the double pendulum model, we analyzed the tennis stroke. The ball and the racket system may be accelerated during the collision time; thus, the speed of the rebound ball does not simply depend on the angular velocity of the racket. A higher angular velocity sometimes gives a lower rebound ball speed. We numerically showed that the proper time-lagged racket rotation increased the speed of the rebound ball by 20%. We also showed that the elbow should move in the proper direction in order to add the angular velocity of the racket.

  14. Calculation of Permeability inside the Basket including one Fuel Assembly

    Energy Technology Data Exchange (ETDEWEB)

    Yu, Seung Hwan; Bang, Kyung Sik; Lee, Ju an; Choi, Woo Seok [KAERI, Daejeon (Korea, Republic of)

    2016-05-15

    In general, the porous media model and the effective thermal conductivity were used to simply the fuel assembly. The methods of calculating permeability were compared considering the flow inside a basket which includes a nuclear fuel. Detailed fuel assembly was a computational modeling and the flow characteristics were investigated. The flow inside the basket which included a fuel assembly is analyzed by CFD. As the height of the fuel assembly increases, the pressure drop linearly increased. The inertia resistance could be neglected. Three methods to calculate the permeability were compared. The permeability by the friction factor is 50% less than the permeability by wall shear stress and pressure drop.

  15. Degenerative cerebellar diseases and differential diagnoses

    International Nuclear Information System (INIS)

    Reith, W.; Roumia, S.; Dietrich, P.

    2016-01-01

    Cerebellar syndromes result in distinct clinical symptoms, such as ataxia, dysarthria, dysmetria, intention tremor and eye movement disorders. In addition to the medical history and clinical examination, imaging is particularly important to differentiate other diseases, such as hydrocephalus and multi-infarct dementia from degenerative cerebellar diseases. Degenerative diseases with cerebellar involvement include Parkinson's disease, multiple system atrophy as well as other diseases including spinocerebellar ataxia. In addition to magnetic resonance imaging (MRI), nuclear medicine imaging investigations are also helpful for the differentiation. Axial fluid-attenuated inversion recovery (FLAIR) and T2-weighted sequences can sometimes show a signal increase in the pons as a sign of degeneration of pontine neurons and transverse fibers in the basilar part of the pons. The imaging is particularly necessary to exclude other diseases, such as normal pressure hydrocephalus (NPH), multi-infarct dementia and cerebellar lesions. (orig.) [de

  16. Spatial modelling of marine organisms in Forsmark and Oskarshamn. Including calculation of physical predictor variables

    Energy Technology Data Exchange (ETDEWEB)

    Carlen, Ida; Nikolopoulos, Anna; Isaeus, Martin (AquaBiota Water Research, Stockholm (SE))

    2007-06-15

    GIS grids (maps) of marine parameters were created using point data from previous site investigations in the Forsmark and Oskarshamn areas. The proportion of global radiation reaching the sea bottom in Forsmark and Oskarshamn was calculated in ArcView, using Secchi depth measurements and the digital elevation models for the respective area. The number of days per year when the incoming light exceeds 5 MJ/m2 at the bottom was then calculated using the result of the previous calculations together with measured global radiation. Existing modelled grid-point data on bottom and pelagic temperature for Forsmark were interpolated to create surface covering grids. Bottom and pelagic temperature grids for Oskarshamn were calculated using point measurements to achieve yearly averages for a few points and then using regressions with existing grids to create new maps. Phytoplankton primary production in Forsmark was calculated using point measurements of chlorophyll and irradiance, and a regression with a modelled grid of Secchi depth. Distribution of biomass of macrophyte communities in Forsmark and Oskarshamn was calculated using spatial modelling in GRASP, based on field data from previous surveys. Physical parameters such as those described above were used as predictor variables. Distribution of biomass of different functional groups of fish in Forsmark was calculated using spatial modelling based on previous surveys and with predictor variables such as physical parameters and results from macrophyte modelling. All results are presented as maps in the report. The quality of the modelled predictions varies as a consequence of the quality and amount of the input data, the ecology and knowledge of the predicted phenomena, and by the modelling technique used. A substantial part of the variation is not described by the models, which should be expected for biological modelling. Therefore, the resulting grids should be used with caution and with this uncertainty kept in mind. All

  17. Spatial modelling of marine organisms in Forsmark and Oskarshamn. Including calculation of physical predictor variables

    International Nuclear Information System (INIS)

    Carlen, Ida; Nikolopoulos, Anna; Isaeus, Martin

    2007-06-01

    GIS grids (maps) of marine parameters were created using point data from previous site investigations in the Forsmark and Oskarshamn areas. The proportion of global radiation reaching the sea bottom in Forsmark and Oskarshamn was calculated in ArcView, using Secchi depth measurements and the digital elevation models for the respective area. The number of days per year when the incoming light exceeds 5 MJ/m2 at the bottom was then calculated using the result of the previous calculations together with measured global radiation. Existing modelled grid-point data on bottom and pelagic temperature for Forsmark were interpolated to create surface covering grids. Bottom and pelagic temperature grids for Oskarshamn were calculated using point measurements to achieve yearly averages for a few points and then using regressions with existing grids to create new maps. Phytoplankton primary production in Forsmark was calculated using point measurements of chlorophyll and irradiance, and a regression with a modelled grid of Secchi depth. Distribution of biomass of macrophyte communities in Forsmark and Oskarshamn was calculated using spatial modelling in GRASP, based on field data from previous surveys. Physical parameters such as those described above were used as predictor variables. Distribution of biomass of different functional groups of fish in Forsmark was calculated using spatial modelling based on previous surveys and with predictor variables such as physical parameters and results from macrophyte modelling. All results are presented as maps in the report. The quality of the modelled predictions varies as a consequence of the quality and amount of the input data, the ecology and knowledge of the predicted phenomena, and by the modelling technique used. A substantial part of the variation is not described by the models, which should be expected for biological modelling. Therefore, the resulting grids should be used with caution and with this uncertainty kept in mind. All

  18. Discrete-fracture-model of multi–scale time-splitting two–phase flow including nanoparticles transport in fractured porous media

    KAUST Repository

    El-Amin, Mohamed

    2017-11-23

    In this article, we consider a two-phase immiscible incompressible flow including nanoparticles transport in fractured heterogeneous porous media. The system of the governing equations consists of water saturation, Darcy’s law, nanoparticles concentration in water, deposited nanoparticles concentration on the pore-wall, and entrapped nanoparticles concentration in the pore-throat, as well as, porosity and permeability variation due to the nanoparticles deposition/entrapment on/in the pores. The discrete-fracture model (DFM) is used to describe the flow and transport in fractured porous media. Moreover, multiscale time-splitting strategy has been employed to manage different time-step sizes for different physics, such as saturation, concentration, etc. Numerical examples are provided to demonstrate the efficiency of the proposed multi-scale time splitting approach.

  19. Discrete-fracture-model of multi–scale time-splitting two–phase flow including nanoparticles transport in fractured porous media

    KAUST Repository

    El-Amin, Mohamed; Kou, Jisheng; Sun, Shuyu

    2017-01-01

    In this article, we consider a two-phase immiscible incompressible flow including nanoparticles transport in fractured heterogeneous porous media. The system of the governing equations consists of water saturation, Darcy’s law, nanoparticles concentration in water, deposited nanoparticles concentration on the pore-wall, and entrapped nanoparticles concentration in the pore-throat, as well as, porosity and permeability variation due to the nanoparticles deposition/entrapment on/in the pores. The discrete-fracture model (DFM) is used to describe the flow and transport in fractured porous media. Moreover, multiscale time-splitting strategy has been employed to manage different time-step sizes for different physics, such as saturation, concentration, etc. Numerical examples are provided to demonstrate the efficiency of the proposed multi-scale time splitting approach.

  20. Including adverse drug events in economic evaluations of anti-tumour necrosis factor-α drugs for adult rheumatoid arthritis: a systematic review of economic decision analytic models.

    Science.gov (United States)

    Heather, Eleanor M; Payne, Katherine; Harrison, Mark; Symmons, Deborah P M

    2014-02-01

    Anti-tumour necrosis factor-α drugs (anti-TNFs) have revolutionised the treatment of rheumatoid arthritis (RA). More effective than standard non-biological disease-modifying anti-rheumatic drugs (nbDMARDs), anti-TNFs are also substantially more expensive. Consequently, a number of model-based economic evaluations have been conducted to establish the relative cost-effectiveness of anti-TNFs. However, anti-TNFs are associated with an increased risk of adverse drug events (ADEs) such as serious infections relative to nbDMARDs. Such ADEs will likely impact on both the costs and consequences of anti-TNFs, for example, through hospitalisations and forced withdrawal from treatment. The aim of this review was to identify and critically appraise if, and how, ADEs have been incorporated into model-based cost-effectiveness analyses of anti-TNFs for adult patients with RA. A systematic literature review was performed. Electronic databases (Ovid MEDLINE; Ovid EMBASE; Web of Science; NHS Economic Evaluations Database) were searched for literature published between January 1990 and October 2013 using electronic search strategies. The reference lists of retrieved studies were also hand searched. In addition, the National Institute for Health and Care Excellence technology appraisals were searched to identify economic models used to inform UK healthcare decision making. Only full economic evaluations that had used an economic model to evaluate biological DMARDs (bDMARDs) (including anti-TNFs) for adult patients with RA and had incorporated the direct costs and/or consequences of ADEs were critically appraised. To be included, studies also had to be available as a full text in English. Data extracted included general study characteristics and information concerning the methods used to incorporate ADEs and any associated assumptions made. The extracted data were synthesised using a tabular and narrative format. A total of 43 model-based economic evaluations of bDMARDs for adult RA

  1. Use of fatal real-life crashes to analyze a safe road transport system model, including the road user, the vehicle, and the road.

    Science.gov (United States)

    Stigson, Helena; Krafft, Maria; Tingvall, Claes

    2008-10-01

    To evaluate if the Swedish Road Administration (SRA) model for a safe road transport system, which includes the interaction between the road user, the vehicle, and the road, could be used to classify fatal car crashes according to some safety indicators. Also, to present a development of the model to better identify system weakness. Real-life crashes with a fatal outcome were classified according to the vehicle's safety rating by Euro NCAP (European Road Assessment Programme) and fitment of ESC (Electronic Stability Control). For each crash, the road was also classified according to EuroRAP (European Road Assessment Programme) criteria, and human behavior in terms of speeding, seat belt use, and driving under the influence of alcohol. Each crash was compared with the model criteria, to identify components that might have contributed to fatal outcome. All fatal crashes where a car occupant was killed that occurred in Sweden during 2004 were included: in all, 215 crashes with 248 fatalities. The data were collected from the in-depth fatal crash data of the Swedish Road Administration (SRA). It was possible to classify 93% of the fatal car crashes according to the SRA model. A number of shortcomings in the criteria were identified since the model did not address rear-end or animal collisions or collisions with stationary/parked vehicles or trailers (18 out of 248 cases). Using the further developed model, it was possible to identify that most of the crashes occurred when two or all three components interacted (in 85 of the total 230 cases). Noncompliance with safety criteria for the road user, the vehicle, and the road led to fatal outcome in 43, 27, and 75 cases, respectively. The SRA model was found to be useful for classifying fatal crashes but needs to be further developed to identify how the components interact and thereby identify weaknesses in the road traffic system. This developed model might be a tool to systematically identify which of the components are

  2. Applying the model of Goal-Directed Behavior, including descriptive norms, to physical activity intentions: A contribution to improving the Theory of Planned Behavior

    Science.gov (United States)

    The theory of planned behavior (TPB) has received its fair share of criticism lately, including calls for it to retire. We contributed to improving the theory by testing extensions such as the model of goal-directed behavior (MGDB, which adds desire and anticipated positive and negative emotions) ap...

  3. Porcine skin visible lesion thresholds for near-infrared lasers including modeling at two pulse durations and spot sizes.

    Science.gov (United States)

    Cain, C P; Polhamus, G D; Roach, W P; Stolarski, D J; Schuster, K J; Stockton, K L; Rockwell, B A; Chen, Bo; Welch, A J

    2006-01-01

    With the advent of such systems as the airborne laser and advanced tactical laser, high-energy lasers that use 1315-nm wavelengths in the near-infrared band will soon present a new laser safety challenge to armed forces and civilian populations. Experiments in nonhuman primates using this wavelength have demonstrated a range of ocular injuries, including corneal, lenticular, and retinal lesions as a function of pulse duration. American National Standards Institute (ANSI) laser safety standards have traditionally been based on experimental data, and there is scant data for this wavelength. We are reporting minimum visible lesion (MVL) threshold measurements using a porcine skin model for two different pulse durations and spot sizes for this wavelength. We also compare our measurements to results from our model based on the heat transfer equation and rate process equation, together with actual temperature measurements on the skin surface using a high-speed infrared camera. Our MVL-ED50 thresholds for long pulses (350 micros) at 24-h postexposure are measured to be 99 and 83 J cm(-2) for spot sizes of 0.7 and 1.3 mm diam, respectively. Q-switched laser pulses of 50 ns have a lower threshold of 11 J cm(-2) for a 5-mm-diam top-hat laser pulse.

  4. A Novel Mean-Value Model of the Cardiovascular System Including a Left Ventricular Assist Device.

    Science.gov (United States)

    Ochsner, Gregor; Amacher, Raffael; Schmid Daners, Marianne

    2017-06-01

    Time-varying elastance models (TVEMs) are often used for simulation studies of the cardiovascular system with a left ventricular assist device (LVAD). Because these models are computationally expensive, they cannot be used for long-term simulation studies. In addition, their equilibria are periodic solutions, which prevent the extraction of a linear time-invariant model that could be used e.g. for the design of a physiological controller. In the current paper, we present a new type of model to overcome these problems: the mean-value model (MVM). The MVM captures the behavior of the cardiovascular system by representative mean values that do not change within the cardiac cycle. For this purpose, each time-varying element is manually converted to its mean-value counterpart. We compare the derived MVM to a similar TVEM in two simulation experiments. In both cases, the MVM is able to fully capture the inter-cycle dynamics of the TVEM. We hope that the new MVM will become a useful tool for researchers working on physiological control algorithms. This paper provides a plant model that enables for the first time the use of tools from classical control theory in the field of physiological LVAD control.

  5. F1 occurrence including L condition in TUCUMAN and BUENOS AIRES

    International Nuclear Information System (INIS)

    Mosert Gonzalez, M. de; Ezquer, R.G.; Oviedo, R.V. del

    1997-01-01

    An analysis of the occurrence of the F1 layer including the L condition has been done, using data from two Argentine stations: TUCUMAN and BUENOS AIRES, at different seasons and solar activity conditions. The comparisons between observations and the F1 occurrence predicted by the IRI-90 model show the need of reviewing the use of the DuCharme et al. (1973) formula adopted by the model to predict the occurrence of the intermediate F1 layer including the L condition. (author). 6 refs, 6 figs, 2 tabs

  6. Advanced Modeling of Ramp Operations including Departure Status at Secondary Airports, Phase I

    Data.gov (United States)

    National Aeronautics and Space Administration — This project addresses three modeling elements relevant to NASA's IADS research and ATD-2 project, two related to ramp operations at primary airports and one related...

  7. INCLUDING RISK IN ECONOMIC FEASIBILITY ANALYSIS:A STOCHASTIC SIMULATION MODEL FOR BLUEBERRY INVESTMENT DECISIONS IN CHILE

    Directory of Open Access Journals (Sweden)

    GERMÁN LOBOS

    2015-12-01

    Full Text Available ABSTRACT The traditional method of net present value (NPV to analyze the economic profitability of an investment (based on a deterministic approach does not adequately represent the implicit risk associated with different but correlated input variables. Using a stochastic simulation approach for evaluating the profitability of blueberry (Vaccinium corymbosum L. production in Chile, the objective of this study is to illustrate the complexity of including risk in economic feasibility analysis when the project is subject to several but correlated risks. The results of the simulation analysis suggest that the non-inclusion of the intratemporal correlation between input variables underestimate the risk associated with investment decisions. The methodological contribution of this study illustrates the complexity of the interrelationships between uncertain variables and their impact on the convenience of carrying out this type of business in Chile. The steps for the analysis of economic viability were: First, adjusted probability distributions for stochastic input variables (SIV were simulated and validated. Second, the random values of SIV were used to calculate random values of variables such as production, revenues, costs, depreciation, taxes and net cash flows. Third, the complete stochastic model was simulated with 10,000 iterations using random values for SIV. This result gave information to estimate the probability distributions of the stochastic output variables (SOV such as the net present value, internal rate of return, value at risk, average cost of production, contribution margin and return on capital. Fourth, the complete stochastic model simulation results were used to analyze alternative scenarios and provide the results to decision makers in the form of probabilities, probability distributions, and for the SOV probabilistic forecasts. The main conclusion shown that this project is a profitable alternative investment in fruit trees in

  8. The Description Of 1 Liter Of Tears Written By Aya Kito

    OpenAIRE

    Yendie, Rizka

    2011-01-01

    Tulisan ini membahas arti dari judul novel 1 liter of Tears. Novel ini dibuat berdasarkan diary sang penulis Aya Kito dalam berjuang melawan penyakit yang tidak ada obatnya yaitu Spinocerebellar Ataxia (SCA), penyakit yang menyerang sel-sel penopang sel saraf. Awalnya penderita merasakan tubuhnya melemah. Berangsur-angsur kemampuan motorik penderita menurun. Sampai-sampai Aya-chan mengalami kesulitan menelan makanan, berbicara dan bahkan tersenyum. Aya-chan mulai merasakan gejala penyakit...

  9. A generalized model for optimal transport of images including dissipation and density modulation

    KAUST Repository

    Maas, Jan; Rumpf, Martin; Schö nlieb, Carola; Simon, Stefan

    2015-01-01

    transport to strongly dissipative dynamics. For this model a robust and effective variational time discretization of geodesic paths is proposed. This requires to minimize a discrete path energy consisting of a sum of consecutive image matching functionals

  10. The clinical obesity maintenance model: an integration of psychological constructs including mood, emotional regulation, disordered overeating, habitual cluster behaviours, health literacy and cognitive function.

    Science.gov (United States)

    Raman, Jayanthi; Smith, Evelyn; Hay, Phillipa

    2013-01-01

    Psychological distress and deficits in executive functioning are likely to be important barriers to effective weight loss maintenance. The purpose of this paper is twofold. First, in the light of recent evidence in the fields of neuropsychology and obesity, particularly on the deficits in the executive function in overweight and obese individuals, a conceptual and theoretical framework of obesity maintenance is introduced by way of a clinical obesity maintenance model (COMM). It is argued that psychological variables, that of habitual cluster Behaviors, emotional dysregulation, mood, and health literacy, interact with executive functioning and impact on the overeating/binge eating behaviors of obese individuals. Second, cognizant of this model, it is argued that the focus of obesity management should be extended to include a broader range of maintaining mechanisms, including but not limited to cognitive deficits. Finally, a discussion on potential future directions in research and practice using the COMM is provided.

  11. Modeling a space-based quantum link that includes an adaptive optics system

    Science.gov (United States)

    Duchane, Alexander W.; Hodson, Douglas D.; Mailloux, Logan O.

    2017-10-01

    Quantum Key Distribution uses optical pulses to generate shared random bit strings between two locations. If a high percentage of the optical pulses are comprised of single photons, then the statistical nature of light and information theory can be used to generate secure shared random bit strings which can then be converted to keys for encryption systems. When these keys are incorporated along with symmetric encryption techniques such as a one-time pad, then this method of key generation and encryption is resistant to future advances in quantum computing which will significantly degrade the effectiveness of current asymmetric key sharing techniques. This research first reviews the transition of Quantum Key Distribution free-space experiments from the laboratory environment to field experiments, and finally, ongoing space experiments. Next, a propagation model for an optical pulse from low-earth orbit to ground and the effects of turbulence on the transmitted optical pulse is described. An Adaptive Optics system is modeled to correct for the aberrations caused by the atmosphere. The long-term point spread function of the completed low-earth orbit to ground optical system is explored in the results section. Finally, the impact of this optical system and its point spread function on an overall quantum key distribution system as well as the future work necessary to show this impact is described.

  12. Femtosecond-laser induced dynamics of CO on Ru(0001): Deep insights from a hot-electron friction model including surface motion

    Science.gov (United States)

    Scholz, Robert; Floß, Gereon; Saalfrank, Peter; Füchsel, Gernot; Lončarić, Ivor; Juaristi, J. I.

    2016-10-01

    A Langevin model accounting for all six molecular degrees of freedom is applied to femtosecond-laser induced, hot-electron driven dynamics of Ru(0001)(2 ×2 ):CO. In our molecular dynamics with electronic friction approach, a recently developed potential energy surface based on gradient-corrected density functional theory accounting for van der Waals interactions is adopted. Electronic friction due to the coupling of molecular degrees of freedom to electron-hole pairs in the metal are included via a local density friction approximation, and surface phonons by a generalized Langevin oscillator model. The action of ultrashort laser pulses enters through a substrate-mediated, hot-electron mechanism via a time-dependent electronic temperature (derived from a two-temperature model), causing random forces acting on the molecule. The model is applied to laser induced lateral diffusion of CO on the surface, "hot adsorbate" formation, and laser induced desorption. Reaction probabilities are strongly enhanced compared to purely thermal processes, both for diffusion and desorption. Reaction yields depend in a characteristic (nonlinear) fashion on the applied laser fluence, as well as branching ratios for various reaction channels. Computed two-pulse correlation traces for desorption and other indicators suggest that aside from electron-hole pairs, phonons play a non-negligible role for laser induced dynamics in this system, acting on a surprisingly short time scale. Our simulations on precomputed potentials allow for good statistics and the treatment of long-time dynamics (300 ps), giving insight into this system which hitherto has not been reached. We find generally good agreement with experimental data where available and make predictions in addition. A recently proposed laser induced population of physisorbed precursor states could not be observed with the present low-coverage model.

  13. OBLIMAP 2.0: a fast climate model-ice sheet model coupler including online embeddable mapping routines

    Science.gov (United States)

    Reerink, Thomas J.; van de Berg, Willem Jan; van de Wal, Roderik S. W.

    2016-11-01

    This paper accompanies the second OBLIMAP open-source release. The package is developed to map climate fields between a general circulation model (GCM) and an ice sheet model (ISM) in both directions by using optimal aligned oblique projections, which minimize distortions. The curvature of the surfaces of the GCM and ISM grid differ, both grids may be irregularly spaced and the ratio of the grids is allowed to differ largely. OBLIMAP's stand-alone version is able to map data sets that differ in various aspects on the same ISM grid. Each grid may either coincide with the surface of a sphere, an ellipsoid or a flat plane, while the grid types might differ. Re-projection of, for example, ISM data sets is also facilitated. This is demonstrated by relevant applications concerning the major ice caps. As the stand-alone version also applies to the reverse mapping direction, it can be used as an offline coupler. Furthermore, OBLIMAP 2.0 is an embeddable GCM-ISM coupler, suited for high-frequency online coupled experiments. A new fast scan method is presented for structured grids as an alternative for the former time-consuming grid search strategy, realising a performance gain of several orders of magnitude and enabling the mapping of high-resolution data sets with a much larger number of grid nodes. Further, a highly flexible masked mapping option is added. The limitation of the fast scan method with respect to unstructured and adaptive grids is discussed together with a possible future parallel Message Passing Interface (MPI) implementation.

  14. Overexpression of mutant ataxin-3 in mouse cerebellum induces ataxia and cerebellar neuropathology.

    Science.gov (United States)

    Nóbrega, Clévio; Nascimento-Ferreira, Isabel; Onofre, Isabel; Albuquerque, David; Conceição, Mariana; Déglon, Nicole; de Almeida, Luís Pereira

    2013-08-01

    Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is a fatal, dominant neurodegenerative disorder caused by the polyglutamine-expanded protein ataxin-3. Clinical manifestations include cerebellar ataxia and pyramidal signs culminating in severe neuronal degeneration. Currently, there is no therapy able to modify disease progression. In the present study, we aimed at investigating one of the most severely affected brain regions in the disorder--the cerebellum--and the behavioral defects associated with the neuropathology in this region. For this purpose, we injected lentiviral vectors encoding full-length human mutant ataxin-3 in the mouse cerebellum of 3-week-old C57/BL6 mice. We show that circumscribed expression of human mutant ataxin-3 in the cerebellum mediates within a short time frame--6 weeks, the development of a behavioral phenotype including reduced motor coordination, wide-based ataxic gait, and hyperactivity. Furthermore, the expression of mutant ataxin-3 resulted in the accumulation of intranuclear inclusions, neuropathological abnormalities, and neuronal death. These data show that lentiviral-based expression of mutant ataxin-3 in the mouse cerebellum induces localized neuropathology, which is sufficient to generate a behavioral ataxic phenotype. Moreover, this approach provides a physiologically relevant, cost-effective and time-effective animal model to gain further insights into the pathogenesis of MJD and for the evaluation of experimental therapeutics of MJD.

  15. DART model for irradiation-induced swelling of dispersion fuel elements including aluminum-fuel interaction

    International Nuclear Information System (INIS)

    Rest, J.; Hofman, G.L.

    1997-01-01

    The Dispersion Analysis Research Tool (DART) contains models for fission-gas-induced fuel swelling, interaction of fuel with the matrix aluminum, for the resultant reaction-product swelling, and for the calculation of the stress gradient within the fuel particle. The effects of an aluminide shell on fuel particle swelling are evaluated. Validation of the model is demonstrated by a comparison of DART calculations of fuel swelling of U 3 SiAl-Al and U 3 Si 2 -Al for various dispersion fuel element designs with the data

  16. Diffusion Tensor Imaging of Human Cerebellar Pathways and their Interplay with Cerebral Macrostructure

    Directory of Open Access Journals (Sweden)

    Zafer eKeser

    2015-04-01

    Full Text Available Cerebellar white matter connections to the central nervous system are classified functionally into the spinocerebellar, vestibulocerebellar, and cerebrocerebellar subdivisions. The Spinocerebellar (SC pathways project from spinal cord to cerebellum, whereas the vestibulocerebellar (VC pathways project from vestibular organs of the inner ear. Cerebrocerebellar connections are composed of feed forward and feedback connections between cerebrum and cerebellum including the cortico-ponto-cerebellar (CPC pathways being of cortical origin and the dentate-rubro-thalamo-cortical (DRTC pathway being of cerebellar origin. In this study we systematically quantified the whole cerebellar system connections using diffusion tensor magnetic resonance imaging (DT-MRI. Ten right-handed healthy subjects (7 males and 3 females, age range 20-51 years were studied. DT-MRI data were acquired with a voxel size = 2mm x 2mm x 2 mm at a 3.0 Tesla clinical MRI scanner. The DT-MRI data were prepared and analyzed using anatomically-guided deterministic tractography methods to reconstruct the SC, DRTC, fronto-ponto-cerebellar (FPC, parieto-ponto-cerebellar (PPC, temporo-ponto-cerebellar (TPC and occipito-ponto-cerebellar (OPC. The DTI-attributes or the cerebellar tracts along with their cortical representation (Brodmann areas were presented in standard Montréal Neurological Institute space. All cerebellar tract volumes were quantified and correlated with volumes of cerebral cortical, subcortical gray matter (GM, cerebral white matter (WM and cerebellar GM, and cerebellar WM. On our healthy cohort, the ratio of total cerebellar GM-to-WM was ~ 3.29 ± 0.24, whereas the ratio of cerebral GM-to-WM was approximately 1.10 ± 0.11. The sum of all cerebellar tract volumes is ~ 25.8 ± 7.3 mL, or a percentage of 1.52 ± 0.43 of the total intracranial volume.

  17. Structural and Functional Magnetic Resonance Imaging of the Cerebellum: Considerations for Assessing Cerebellar Ataxias.

    Science.gov (United States)

    Deistung, Andreas; Stefanescu, Maria R; Ernst, Thomas M; Schlamann, Marc; Ladd, Mark E; Reichenbach, Jürgen R; Timmann, Dagmar

    2016-02-01

    Magnetic resonance imaging (MRI) of the brain is of high interest for diagnosing and understanding degenerative ataxias. Here, we present state-of-the-art MRI methods to characterize structural alterations of the cerebellum and introduce initial experiments to show abnormalities in the cerebellar nuclei. Clinically, T1-weighted MR images are used to assess atrophy of the cerebellar cortex, the brainstem, and the spinal cord, whereas T2-weighted and PD-weighted images are typically employed to depict potential white matter lesions that may be associated with certain types of ataxias. More recently, attention has also focused on the characterization of the cerebellar nuclei, which are discernible on spatially highly resolved iron-sensitive MR images due to their relatively high iron content, including T2 (*)-weighted images, susceptibility-weighted images (SWI), effective transverse relaxation rate (R2 (*)) maps, and quantitative susceptibility maps (QSM). Among these iron-sensitive techniques, QSM reveals the best contrast between cerebellar nuclei and their surroundings. In particular, the gyrification of the dentate nuclei is prominently depicted, even at the clinically widely available field strength of 3 T. The linear relationship between magnetic susceptibility and local iron content allows for determination of iron deposition in cerebellar nuclei non-invasively. The increased signal-to-noise ratio of ultrahigh-field MRI (B0 ≥ 7 T) and advances in spatial normalization methods enable functional MRI (fMRI) at the level of the cerebellar cortex and cerebellar nuclei. Data from initial fMRI studies are presented in three common forms of hereditary ataxias (Friedreich's ataxia, spinocerebellar ataxia type 3, and spinocerebellar ataxia type 6). Characteristic changes in the fMRI signal are discussed in the light of histopathological data and current knowledge of the underlying physiology of the fMRI signal in the cerebellum.

  18. Styrene-spaced copolymers including anthraquinone and β-O-4 lignin model units: synthesis, characterization and reactivity under alkaline pulping conditions.

    Science.gov (United States)

    Megiatto, Jackson D; Cazeils, Emmanuel; Ham-Pichavant, Frédérique; Grelier, Stéphane; Gardrat, Christian; Castellan, Alain

    2012-05-14

    A series of random copoly(styrene)s has been synthesized via radical polymerization of functionalized anthraquinone (AQ) and β-O-4 lignin model monomers. The copolymers were designed to have a different number of styrene spacer groups between the AQ and β-O-4 lignin side chains aiming at investigating the distance effects on AQ/β-O-4 electron transfer mechanisms. A detailed molecular characterization, including techniques such as size exclusion chromatography, MALDI-TOF mass spectrometry, and (1)H, (13)C, (31)P NMR and UV-vis spectroscopies, afforded quantitative information about the composition of the copolymers as well as the average distribution of the AQ and β-O-4 groups in the macromolecular structures. TGA and DSC thermal analysis have indicated that the copolymers were thermally stable under regular pulping conditions, revealing the inertness of the styrene polymer backbone in the investigation of electron transfer mechanisms. Alkaline pulping experiments showed that close contact between the redox active side chains in the copolymers was fundamental for an efficient degradation of the β-O-4 lignin model units, highlighting the importance of electron transfer reactions in the lignin degradation mechanisms catalyzed by AQ. In the absence of glucose, AQ units oxidized phenolic β-O-4 lignin model parts, mainly by electron transfer leading to vanillin as major product. By contrast, in presence of glucose, anthrahydroquinone units (formed by reduction of AQ) reduced the quinone-methide units (issued by dehydration of phenolic β-O-4 lignin model part) mainly by electron transfer leading to guaiacol as major product. Both processes were distance dependent.

  19. Inline CBET Model Including SRS Backscatter

    Energy Technology Data Exchange (ETDEWEB)

    Bailey, David S. [Lawrence Livermore National Lab. (LLNL), Livermore, CA (United States)

    2015-06-26

    Cross-beam energy transfer (CBET) has been used as a tool on the National Ignition Facility (NIF) since the first energetics experiments in 2009 to control the energy deposition in ignition hohlraums and tune the implosion symmetry. As large amounts of power are transferred between laser beams at the entrance holes of NIF hohlraums, the presence of many overlapping beat waves can lead to stochastic ion heating in the regions where laser beams overlap [P. Michel et al., Phys. Rev. Lett. 109, 195004 (2012)]. Using the CBET gains derived in this paper, we show how to implement these equations in a ray-based laser source for a rad-hydro code.

  20. FOX-2 Dependent Splicing of Ataxin-2 Transcript Is Affected by Ataxin-1 Overexpression

    Science.gov (United States)

    Welzel, Franziska; Kaehler, Christian; Isau, Melanie; Hallen, Linda; Lehrach, Hans; Krobitsch, Sylvia

    2012-01-01

    Alternative splicing is a fundamental posttranscriptional mechanism for controlling gene expression, and splicing defects have been linked to various human disorders. The splicing factor FOX-2 is part of a main protein interaction hub in a network related to human inherited ataxias, however, its impact remains to be elucidated. Here, we focused on the reported interaction between FOX-2 and ataxin-1, the disease-causing protein in spinocerebellar ataxia type 1. In this line, we further evaluated this interaction by yeast-2-hybrid analyses and co-immunoprecipitation experiments in mammalian cells. Interestingly, we discovered that FOX-2 localization and splicing activity is affected in the presence of nuclear ataxin-1 inclusions. Moreover, we observed that FOX-2 directly interacts with ataxin-2, a protein modulating spinocerebellar ataxia type 1 pathogenesis. Finally, we provide evidence that splicing of pre-mRNA of ataxin-2 depends on FOX-2 activity, since reduction of FOX-2 levels led to increased skipping of exon 18 in ataxin-2 transcripts. Most striking, we observed that ataxin-1 overexpression has an effect on this splicing event as well. Thus, our results demonstrate that FOX-2 is involved in splicing of ataxin-2 transcripts and that this splicing event is altered by overexpression of ataxin-1. PMID:22666429

  1. The Clinical Obesity Maintenance Model: An Integration of Psychological Constructs including Mood, Emotional Regulation, Disordered Overeating, Habitual Cluster Behaviours, Health Literacy and Cognitive Function

    Directory of Open Access Journals (Sweden)

    Jayanthi Raman

    2013-01-01

    Full Text Available Psychological distress and deficits in executive functioning are likely to be important barriers to effective weight loss maintenance. The purpose of this paper is twofold. First, in the light of recent evidence in the fields of neuropsychology and obesity, particularly on the deficits in the executive function in overweight and obese individuals, a conceptual and theoretical framework of obesity maintenance is introduced by way of a clinical obesity maintenance model (COMM. It is argued that psychological variables, that of habitual cluster Behaviors, emotional dysregulation, mood, and health literacy, interact with executive functioning and impact on the overeating/binge eating behaviors of obese individuals. Second, cognizant of this model, it is argued that the focus of obesity management should be extended to include a broader range of maintaining mechanisms, including but not limited to cognitive deficits. Finally, a discussion on potential future directions in research and practice using the COMM is provided.

  2. An extended TRANSCAR model including ionospheric convection: simulation of EISCAT observations using inputs from AMIE

    Directory of Open Access Journals (Sweden)

    P.-L. Blelly

    2005-02-01

    Full Text Available The TRANSCAR ionospheric model was extended to account for the convection of the magnetic field lines in the auroral and polar ionosphere. A mixed Eulerian-Lagrangian 13-moment approach was used to describe the dynamics of an ionospheric plasma tube. In the present study, one focuses on large scale transports in the polar ionosphere. The model was used to simulate a 35-h period of EISCAT-UHF observations on 16-17 February 1993. The first day was magnetically quiet, and characterized by elevated electron concentrations: the diurnal F2 layer reached as much as 1012m-3, which is unusual for a winter and moderate solar activity (F10.7=130 period. An intense geomagnetic event occurred on the second day, seen in the data as a strong intensification of the ionosphere convection velocities in the early afternoon (with the northward electric field reaching 150mVm-1 and corresponding frictional heating of the ions up to 2500K. The simulation used time-dependent AMIE outputs to infer flux-tube transports in the polar region, and to provide magnetospheric particle and energy inputs to the ionosphere. The overall very good agreement, obtained between the model and the observations, demonstrates the high ability of the extended TRANSCAR model for quantitative modelling of the high-latitude ionosphere; however, some differences are found which are attributed to the precipitation of electrons with very low energy. All these results are finally discussed in the frame of modelling the auroral ionosphere with space weather applications in mind.

  3. Potential transformation of trace species including aircraft exhaust in a cloud environment. The `Chedrom model`

    Energy Technology Data Exchange (ETDEWEB)

    Ozolin, Y.E.; Karol, I.L. [Main Geophysical Observatory, St. Petersburg (Russian Federation); Ramaroson, R. [Office National d`Etudes et de Recherches Aerospatiales (ONERA), 92 - Chatillon (France)

    1997-12-31

    Box model for coupled gaseous and aqueous phases is used for sensitivity study of potential transformation of trace gases in a cloud environment. The rate of this transformation decreases with decreasing of pH in droplets, with decreasing of photodissociation rates inside the cloud and with increasing of the droplet size. Model calculations show the potential formation of H{sub 2}O{sub 2} in aqueous phase and transformation of gaseous HNO{sub 3} into NO{sub x} in a cloud. This model is applied for exploration of aircraft exhausts evolution in plume inside a cloud. (author) 10 refs.

  4. Potential transformation of trace species including aircraft exhaust in a cloud environment. The `Chedrom model`

    Energy Technology Data Exchange (ETDEWEB)

    Ozolin, Y E; Karol, I L [Main Geophysical Observatory, St. Petersburg (Russian Federation); Ramaroson, R [Office National d` Etudes et de Recherches Aerospatiales (ONERA), 92 - Chatillon (France)

    1998-12-31

    Box model for coupled gaseous and aqueous phases is used for sensitivity study of potential transformation of trace gases in a cloud environment. The rate of this transformation decreases with decreasing of pH in droplets, with decreasing of photodissociation rates inside the cloud and with increasing of the droplet size. Model calculations show the potential formation of H{sub 2}O{sub 2} in aqueous phase and transformation of gaseous HNO{sub 3} into NO{sub x} in a cloud. This model is applied for exploration of aircraft exhausts evolution in plume inside a cloud. (author) 10 refs.

  5. WE-H-BRA-03: Development of a Model to Include the Evolution of Resistant Tumor Subpopulations Into the Treatment Optimization Process for Schedules Involving Targeted Agents in Chemoradiation Therapy

    International Nuclear Information System (INIS)

    Grassberger, C; Paganetti, H

    2016-01-01

    Purpose: To develop a model that includes the process of resistance development into the treatment optimization process for schedules that include targeted therapies. Further, to validate the approach using clinical data and to apply the model to assess the optimal induction period with targeted agents before curative treatment with chemo-radiation in stage III lung cancer. Methods: Growth of the tumor and its subpopulations is modeled by Gompertzian growth dynamics, resistance induction as a stochastic process. Chemotherapy induced cell kill is modeled by log-cell kill dynamics, targeted agents similarly but restricted to the sensitive population. Radiation induced cell kill is assumed to follow the linear-quadratic model. The validation patient data consist of a cohort of lung cancer patients treated with tyrosine kinase inhibitors that had longitudinal imaging data available. Results: The resistance induction model was successfully validated using clinical trial data from 49 patients treated with targeted agents. The observed recurrence kinetics, with tumors progressing from 1.4–63 months, result in tumor growth equaling a median volume doubling time of 92 days [34–248] and a median fraction of pre-existing resistance of 0.035 [0–0.22], in agreement with previous clinical studies. The model revealed widely varying optimal time points for the use of curative therapy, reaching from ∼1m to >6m depending on the patient’s growth rate and amount of pre-existing resistance. This demonstrates the importance of patient-specific treatment schedules when targeted agents are incorporated into the treatment. Conclusion: We developed a model including evolutionary dynamics of resistant sub-populations with traditional chemotherapy and radiation cell kill models. Fitting to clinical data yielded patient specific growth rates and resistant fraction in agreement with previous studies. Further application of the model demonstrated how proper timing of chemo

  6. WE-H-BRA-03: Development of a Model to Include the Evolution of Resistant Tumor Subpopulations Into the Treatment Optimization Process for Schedules Involving Targeted Agents in Chemoradiation Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Grassberger, C; Paganetti, H [Massachusetts General Hospital, Boston, MA (United States)

    2016-06-15

    Purpose: To develop a model that includes the process of resistance development into the treatment optimization process for schedules that include targeted therapies. Further, to validate the approach using clinical data and to apply the model to assess the optimal induction period with targeted agents before curative treatment with chemo-radiation in stage III lung cancer. Methods: Growth of the tumor and its subpopulations is modeled by Gompertzian growth dynamics, resistance induction as a stochastic process. Chemotherapy induced cell kill is modeled by log-cell kill dynamics, targeted agents similarly but restricted to the sensitive population. Radiation induced cell kill is assumed to follow the linear-quadratic model. The validation patient data consist of a cohort of lung cancer patients treated with tyrosine kinase inhibitors that had longitudinal imaging data available. Results: The resistance induction model was successfully validated using clinical trial data from 49 patients treated with targeted agents. The observed recurrence kinetics, with tumors progressing from 1.4–63 months, result in tumor growth equaling a median volume doubling time of 92 days [34–248] and a median fraction of pre-existing resistance of 0.035 [0–0.22], in agreement with previous clinical studies. The model revealed widely varying optimal time points for the use of curative therapy, reaching from ∼1m to >6m depending on the patient’s growth rate and amount of pre-existing resistance. This demonstrates the importance of patient-specific treatment schedules when targeted agents are incorporated into the treatment. Conclusion: We developed a model including evolutionary dynamics of resistant sub-populations with traditional chemotherapy and radiation cell kill models. Fitting to clinical data yielded patient specific growth rates and resistant fraction in agreement with previous studies. Further application of the model demonstrated how proper timing of chemo

  7. The 4-parameter Compressible Packing Model (CPM) including a critical cavity size ratio

    Science.gov (United States)

    Roquier, Gerard

    2017-06-01

    The 4-parameter Compressible Packing Model (CPM) has been developed to predict the packing density of mixtures constituted by bidisperse spherical particles. The four parameters are: the wall effect and the loosening effect coefficients, the compaction index and a critical cavity size ratio. The two geometrical interactions have been studied theoretically on the basis of a spherical cell centered on a secondary class bead. For the loosening effect, a critical cavity size ratio, below which a fine particle can be inserted into a small cavity created by touching coarser particles, is introduced. This is the only parameter which requires adaptation to extend the model to other types of particles. The 4-parameter CPM demonstrates its efficiency on frictionless glass beads (300 values), spherical particles numerically simulated (20 values), round natural particles (125 values) and crushed particles (335 values) with correlation coefficients equal to respectively 99.0%, 98.7%, 97.8%, 96.4% and mean deviations equal to respectively 0.007, 0.006, 0.007, 0.010.

  8. Hydraulic Model for Drinking Water Networks, Including Household Connections; Modelo hidraulico para redes de agua potable con tomas domiciliarias

    Energy Technology Data Exchange (ETDEWEB)

    Guerrero Angulo, Jose Oscar [Universidad Autonoma de Sinaloa (Mexico); Arreguin Cortes, Felipe [Instituto Mexicano de Tecnologia del Agua, Jiutepec, Morelos (Mexico)

    2002-03-01

    This paper presents a hydraulic simulation model for drinking water networks, including elements that are currently not considered household connections, spatially variable flowrate distribution pipelines, and tee secondary network. This model is determined by solving the equations needed for a conventional model following an indirect procedure for the solution of large equations systems. Household connection performance is considered as dependent of water pressure and the way in which users operate the taps of such intakes. This approach allows a better a acquaintance with the drinking water supply networks performance as well as solving problems that demand a more precise hydraulic simulation, such as water quality variations, leaks in networks, and the influence of home water tanks as regulating devices. [Spanish] Se presenta un modelo de simulacion hidraulica para redes de agua potable en el cual se incluyen elementos que no se toman en cuenta actualmente, como las tomas domiciliarias, los tubos de distribucion con gastos espacialmente variado y la red secundaria, resolviendo el numero de ecuaciones que seria necesario plantear en un modelo convencional mediante un procedimiento indirecto para la solucion de grandes sistemas de ecuaciones. En las tomas domiciliarias se considera que su funcionamiento depende de las presiones y la forma en que los usuarios operan las llaves de las mismas. Este planteamiento permite conocer mejor el funcionamiento de las redes de abastecimiento de agua potable y solucionar problemas que requieren de una simulacion hidraulica mas precisa, como el comportamiento de la calidad del agua, las fugas en las redes y la influencia reguladora de los tinacos de las casas.

  9. Energy-based fatigue model for shape memory alloys including thermomechanical coupling

    Science.gov (United States)

    Zhang, Yahui; Zhu, Jihong; Moumni, Ziad; Van Herpen, Alain; Zhang, Weihong

    2016-03-01

    This paper is aimed at developing a low cycle fatigue criterion for pseudoelastic shape memory alloys to take into account thermomechanical coupling. To this end, fatigue tests are carried out at different loading rates under strain control at room temperature using NiTi wires. Temperature distribution on the specimen is measured using a high speed thermal camera. Specimens are tested to failure and fatigue lifetimes of specimens are measured. Test results show that the fatigue lifetime is greatly influenced by the loading rate: as the strain rate increases, the fatigue lifetime decreases. Furthermore, it is shown that the fatigue cracks initiate when the stored energy inside the material reaches a critical value. An energy-based fatigue criterion is thus proposed as a function of the irreversible hysteresis energy of the stabilized cycle and the loading rate. Fatigue life is calculated using the proposed model. The experimental and computational results compare well.

  10. An Evacuation Model for Passenger Ships That Includes the Influence of Obstacles in Cabins

    Directory of Open Access Journals (Sweden)

    Baocheng Ni

    2017-01-01

    Full Text Available Passenger behavior and ship environment are the key factors affecting evacuation efficiency. However, current studies ignore the interior layout of passenger ship cabins and treat the cabins as empty rooms. To investigate the influence of obstacles (e.g., tables and stools on cabin evacuation, we propose an agent-based social force model for advanced evacuation analysis of passenger ships; this model uses a goal-driven submodel to determine a plan and an extended social force submodel to govern the movement of passengers. The extended social force submodel considers the interaction forces between the passengers, crew, and obstacles and minimises the range of these forces to improve computational efficiency. We drew the following conclusions based on a series of evacuation simulations conducted in this study: (1 the proposed model endows the passenger with the behaviors of bypassing and crossing obstacles, (2 funnel-shaped exits from cabins can improve evacuation efficiency, and (3 as the exit angle increases, the evacuation time also increases. These findings offer ship designers some insight towards increasing the safety of large passenger ships.

  11. Food Insecurity in U.S. Households That Include Children with Disabilities

    Science.gov (United States)

    Sonik, Rajan; Parish, Susan L.; Ghosh, Subharati; Igdalsky, Leah

    2016-01-01

    The authors examined food insecurity in households including children with disabilities, analyzing data from the 2004 and 2008 panels of the Survey of Income and Program Participation, which included 24,729 households with children, 3,948 of which had children with disabilities. Logistic regression models were used to estimate the likelihood of…

  12. An extended TRANSCAR model including ionospheric convection: simulation of EISCAT observations using inputs from AMIE

    Directory of Open Access Journals (Sweden)

    P.-L. Blelly

    2005-02-01

    Full Text Available The TRANSCAR ionospheric model was extended to account for the convection of the magnetic field lines in the auroral and polar ionosphere. A mixed Eulerian-Lagrangian 13-moment approach was used to describe the dynamics of an ionospheric plasma tube. In the present study, one focuses on large scale transports in the polar ionosphere. The model was used to simulate a 35-h period of EISCAT-UHF observations on 16-17 February 1993. The first day was magnetically quiet, and characterized by elevated electron concentrations: the diurnal F2 layer reached as much as 1012m-3, which is unusual for a winter and moderate solar activity (F10.7=130 period. An intense geomagnetic event occurred on the second day, seen in the data as a strong intensification of the ionosphere convection velocities in the early afternoon (with the northward electric field reaching 150mVm-1 and corresponding frictional heating of the ions up to 2500K. The simulation used time-dependent AMIE outputs to infer flux-tube transports in the polar region, and to provide magnetospheric particle and energy inputs to the ionosphere. The overall very good agreement, obtained between the model and the observations, demonstrates the high ability of the extended TRANSCAR model for quantitative modelling of the high-latitude ionosphere; however, some differences are found which are attributed to the precipitation of electrons with very low energy. All these results are finally discussed in the frame of modelling the auroral ionosphere with space weather applications in mind.

  13. Global anthropogenic emissions of particulate matter including black carbon

    Science.gov (United States)

    Klimont, Zbigniew; Kupiainen, Kaarle; Heyes, Chris; Purohit, Pallav; Cofala, Janusz; Rafaj, Peter; Borken-Kleefeld, Jens; Schöpp, Wolfgang

    2017-07-01

    This paper presents a comprehensive assessment of historical (1990-2010) global anthropogenic particulate matter (PM) emissions including the consistent and harmonized calculation of mass-based size distribution (PM1, PM2. 5, PM10), as well as primary carbonaceous aerosols including black carbon (BC) and organic carbon (OC). The estimates were developed with the integrated assessment model GAINS, where source- and region-specific technology characteristics are explicitly included. This assessment includes a number of previously unaccounted or often misallocated emission sources, i.e. kerosene lamps, gas flaring, diesel generators, refuse burning; some of them were reported in the past for selected regions or in the context of a particular pollutant or sector but not included as part of a total estimate. Spatially, emissions were calculated for 172 source regions (as well as international shipping), presented for 25 global regions, and allocated to 0.5° × 0.5° longitude-latitude grids. No independent estimates of emissions from forest fires and savannah burning are provided and neither windblown dust nor unpaved roads emissions are included. We estimate that global emissions of PM have not changed significantly between 1990 and 2010, showing a strong decoupling from the global increase in energy consumption and, consequently, CO2 emissions, but there are significantly different regional trends, with a particularly strong increase in East Asia and Africa and a strong decline in Europe, North America, and the Pacific region. This in turn resulted in important changes in the spatial pattern of PM burden, e.g. European, North American, and Pacific contributions to global emissions dropped from nearly 30 % in 1990 to well below 15 % in 2010, while Asia's contribution grew from just over 50 % to nearly two-thirds of the global total in 2010. For all PM species considered, Asian sources represented over 60 % of the global anthropogenic total, and residential combustion

  14. Global anthropogenic emissions of particulate matter including black carbon

    Directory of Open Access Journals (Sweden)

    Z. Klimont

    2017-07-01

    Full Text Available This paper presents a comprehensive assessment of historical (1990–2010 global anthropogenic particulate matter (PM emissions including the consistent and harmonized calculation of mass-based size distribution (PM1, PM2. 5, PM10, as well as primary carbonaceous aerosols including black carbon (BC and organic carbon (OC. The estimates were developed with the integrated assessment model GAINS, where source- and region-specific technology characteristics are explicitly included. This assessment includes a number of previously unaccounted or often misallocated emission sources, i.e. kerosene lamps, gas flaring, diesel generators, refuse burning; some of them were reported in the past for selected regions or in the context of a particular pollutant or sector but not included as part of a total estimate. Spatially, emissions were calculated for 172 source regions (as well as international shipping, presented for 25 global regions, and allocated to 0.5°  ×  0.5° longitude–latitude grids. No independent estimates of emissions from forest fires and savannah burning are provided and neither windblown dust nor unpaved roads emissions are included. We estimate that global emissions of PM have not changed significantly between 1990 and 2010, showing a strong decoupling from the global increase in energy consumption and, consequently, CO2 emissions, but there are significantly different regional trends, with a particularly strong increase in East Asia and Africa and a strong decline in Europe, North America, and the Pacific region. This in turn resulted in important changes in the spatial pattern of PM burden, e.g. European, North American, and Pacific contributions to global emissions dropped from nearly 30 % in 1990 to well below 15 % in 2010, while Asia's contribution grew from just over 50 % to nearly two-thirds of the global total in 2010. For all PM species considered, Asian sources represented over 60 % of the global

  15. Defects in the CAPN1 Gene Result in Alterations in Cerebellar Development and Cerebellar Ataxia in Mice and Humans

    Directory of Open Access Journals (Sweden)

    Yubin Wang

    2016-06-01

    Full Text Available A CAPN1 missense mutation in Parson Russell Terrier dogs is associated with spinocerebellar ataxia. We now report that homozygous or heterozygous CAPN1-null mutations in humans result in cerebellar ataxia and limb spasticity in four independent pedigrees. Calpain-1 knockout (KO mice also exhibit a mild form of ataxia due to abnormal cerebellar development, including enhanced neuronal apoptosis, decreased number of cerebellar granule cells, and altered synaptic transmission. Enhanced apoptosis is due to absence of calpain-1-mediated cleavage of PH domain and leucine-rich repeat protein phosphatase 1 (PHLPP1, which results in inhibition of the Akt pro-survival pathway in developing granule cells. Injection of neonatal mice with the indirect Akt activator, bisperoxovanadium, or crossing calpain-1 KO mice with PHLPP1 KO mice prevented increased postnatal cerebellar granule cell apoptosis and restored granule cell density and motor coordination in adult mice. Thus, mutations in CAPN1 are an additional cause of ataxia in mammals, including humans.

  16. A speech production model including the nasal Cavity: A novel approach to articulatory analysis of speech signals

    DEFF Research Database (Denmark)

    Olesen, Morten

    In order to obtain articulatory analysis of speech production the model is improved. the standard model, as used in LPC analysis, to a large extent only models the acoustic properties of speech signal as opposed to articulatory modelling of the speech production. In spite of this the LPC model...... is by far the most widely used model in speech technology....

  17. Energy-based fatigue model for shape memory alloys including thermomechanical coupling

    International Nuclear Information System (INIS)

    Zhang, Yahui; Zhu, Jihong; Moumni, Ziad; Zhang, Weihong; Van Herpen, Alain

    2016-01-01

    This paper is aimed at developing a low cycle fatigue criterion for pseudoelastic shape memory alloys to take into account thermomechanical coupling. To this end, fatigue tests are carried out at different loading rates under strain control at room temperature using NiTi wires. Temperature distribution on the specimen is measured using a high speed thermal camera. Specimens are tested to failure and fatigue lifetimes of specimens are measured. Test results show that the fatigue lifetime is greatly influenced by the loading rate: as the strain rate increases, the fatigue lifetime decreases. Furthermore, it is shown that the fatigue cracks initiate when the stored energy inside the material reaches a critical value. An energy-based fatigue criterion is thus proposed as a function of the irreversible hysteresis energy of the stabilized cycle and the loading rate. Fatigue life is calculated using the proposed model. The experimental and computational results compare well. (paper)

  18. Reliability analysis of the auxiliary feedwater system of Angra-1 including common cause failures using the multiple greek letter model

    International Nuclear Information System (INIS)

    Lapa, Celso Marcelo Franklin.

    1996-05-01

    The use of redundancy to increase the reliability of industrial systems make them subject to the occurrence of common cause events. The industrial experience and the results of safety analysis studies have indicated that common cause failures are the main contributors to the unreliability of plants that have redundant systems, specially in nuclear power plants. In this Thesis procedures are developed in order to include the impact of common cause failures in the calculation of the top event occurrence probability of the Auxiliary Feedwater System in a typical two-loop Nuclear Power Plant (PWR). For this purpose the Multiple Greek Letter Model is used. (author). 14 refs., 10 figs., 11 tabs

  19. A coupling method for a cardiovascular simulation model which includes the Kalman filter.

    Science.gov (United States)

    Hasegawa, Yuki; Shimayoshi, Takao; Amano, Akira; Matsuda, Tetsuya

    2012-01-01

    Multi-scale models of the cardiovascular system provide new insight that was unavailable with in vivo and in vitro experiments. For the cardiovascular system, multi-scale simulations provide a valuable perspective in analyzing the interaction of three phenomenons occurring at different spatial scales: circulatory hemodynamics, ventricular structural dynamics, and myocardial excitation-contraction. In order to simulate these interactions, multiscale cardiovascular simulation systems couple models that simulate different phenomena. However, coupling methods require a significant amount of calculation, since a system of non-linear equations must be solved for each timestep. Therefore, we proposed a coupling method which decreases the amount of calculation by using the Kalman filter. In our method, the Kalman filter calculates approximations for the solution to the system of non-linear equations at each timestep. The approximations are then used as initial values for solving the system of non-linear equations. The proposed method decreases the number of iterations required by 94.0% compared to the conventional strong coupling method. When compared with a smoothing spline predictor, the proposed method required 49.4% fewer iterations.

  20. Logical diagnosis model turbojet engine including double-circuit intermittent flow of his injuries

    Directory of Open Access Journals (Sweden)

    О.П. Стьопушкіна

    2007-01-01

    Full Text Available  In this article is considered question of the change quantitative and qualitative factors of the technical condition constructive element running part of jet engine. As a result called on experimental studies diagnostic sign were definite sign with provision for intermittent damages and on base this is built expert model of the turbojet double-circuit engine.

  1. A Simple Model of Fields Including the Strong or Nuclear Force and a Cosmological Speculation

    Directory of Open Access Journals (Sweden)

    David L. Spencer

    2016-10-01

    Full Text Available Reexamining the assumptions underlying the General Theory of Relativity and calling an object's gravitational field its inertia, and acceleration simply resistance to that inertia, yields a simple field model where the potential (kinetic energy of a particle at rest is its capacity to move itself when its inertial field becomes imbalanced. The model then attributes electromagnetic and strong forces to the effects of changes in basic particle shape. Following up on the model's assumption that the relative intensity of a particle's gravitational field is always inversely related to its perceived volume and assuming that all black holes spin, may create the possibility of a cosmic rebound where a final spinning black hole ends with a new Big Bang.

  2. Towards ligand docking including explicit interface water molecules.

    Directory of Open Access Journals (Sweden)

    Gordon Lemmon

    Full Text Available Small molecule docking predicts the interaction of a small molecule ligand with a protein at atomic-detail accuracy including position and conformation the ligand but also conformational changes of the protein upon ligand binding. While successful in the majority of cases, docking algorithms including RosettaLigand fail in some cases to predict the correct protein/ligand complex structure. In this study we show that simultaneous docking of explicit interface water molecules greatly improves Rosetta's ability to distinguish correct from incorrect ligand poses. This result holds true for both protein-centric water docking wherein waters are located relative to the protein binding site and ligand-centric water docking wherein waters move with the ligand during docking. Protein-centric docking is used to model 99 HIV-1 protease/protease inhibitor structures. We find protease inhibitor placement improving at a ratio of 9:1 when one critical interface water molecule is included in the docking simulation. Ligand-centric docking is applied to 341 structures from the CSAR benchmark of diverse protein/ligand complexes [1]. Across this diverse dataset we see up to 56% recovery of failed docking studies, when waters are included in the docking simulation.

  3. A review of cell-scale multiphase flow modeling, including water management, in polymer electrolyte fuel cells

    International Nuclear Information System (INIS)

    Andersson, M.; Beale, S.B.; Espinoza, M.; Wu, Z.; Lehnert, W.

    2016-01-01

    on the transport processes inside the porous GDL are extensively discussed. The selection of a computational approach, for the two-phase flow within a GDL or GC, for example, should be based on the computational resources available, concerns about time and scale (microscale, cell scale, stack scale or system scale), as well as accuracy requirements. One important feature, included in some computational approaches, is the possibility to track the front between the liquid and the gas phases. To build a PEFC model, one must make a large number of assumptions. Some assumptions have a negligible effect on the results and reliability of the model. However, other assumptions may significantly affect the result. It is strongly recommended in any modeling paper to clearly state the assumptions being implemented, for others to be able to judge the work. It is important to note that a large fraction of the expressions that presently are used to describe the transport processes inside PEFC GDLs were originally developed to describe significantly different systems, such as sand or rocks. Moreover, the flow pattern maps and pressure drop correlations of two phase flow in micro channels may not be applicable for GCs due to one side wall being porous, with the resulting interaction between the GDL and GC.

  4. Multiscale modelling of solidification microstructures, including microsegregation and microporosity, in an Al-Si-Cu alloy

    International Nuclear Information System (INIS)

    Lee, P.D.; Chirazi, A.; Atwood, R.C.; Wang, W.

    2004-01-01

    Phase transition phenomena in metallic alloys involve complex physical processes occurring over a wide range of temporal, spatial and energy scales. Multiscale modelling is a powerful methodology for understanding these complex systems. In this paper, a multiscale model of grain and pore formation is presented during solidification. At the microscale, a combined stochastic-deterministic approach based on the cellular automata method is used to solve multicomponent diffusion in a three-phase system (liquid, solid and gas), simulating the nucleation and growth of both grains and pores. The impingement of the growing pores upon the developing solid is also solved to predict the tortuous shape of the porosity, a critical factor for fatigue properties. The micromodel is coupled with a finite element method (FEM) solution of the macroscale heat transfer and fluid flow in industrial castings through the temperature and pressure fields. The result model was used to investigate the influence of local solidification time, hydrogen content, local metallostatic pressure and alloy composition upon the predicted grain structure and pore morphology. Comparison of the model predictions to both laboratory and industrial scale castings are presented

  5. Mitochondrial recessive ataxia syndrome mimicking dominant spinocerebellar ataxia.

    Science.gov (United States)

    Palin, Eino J H; Hakonen, Anna H; Korpela, Mari; Paetau, Anders; Suomalainen, Anu

    2012-04-15

    We studied the genetic background of a family with SCA, showing dominant inheritance and anticipation. Muscle histology, POLG1 gene sequence, neuropathology and mitochondrial DNA analyses in a mother and a son showed typical findings for a mitochondrial disorder, and both were shown to be homozygous for a recessive POLG1 mutation, underlying mitochondrial recessive ataxia syndrome, MIRAS. The healthy father was a heterozygous carrier for the same mutation. Recessively inherited MIRAS mutations should be tested in dominantly inherited SCAs cases of unknown cause, as the high carrier frequency of MIRAS may result in two independent introductions of the mutant allele in the family and thereby mimic dominant inheritance. Copyright © 2011 Elsevier B.V. All rights reserved.

  6. Two-stage robust UC including a novel scenario-based uncertainty model for wind power applications

    International Nuclear Information System (INIS)

    Álvarez-Miranda, Eduardo; Campos-Valdés, Camilo; Rahmann, Claudia

    2015-01-01

    Highlights: • Methodological framework for obtaining Robust Unit Commitment (UC) policies. • Wind-power forecast using a revisited bootstrap predictive inference approach. • Novel scenario-based model for wind-power uncertainty. • Efficient modeling framework for obtaining nearly optimal UC policies in reasonable time. • Effective incorporation of wind-power uncertainty in the UC modeling. - Abstract: The complex processes involved in the determination of the availability of power from renewable energy sources, such as wind power, impose great challenges in the forecasting processes carried out by transmission system operators (TSOs). Nowadays, many of these TSOs use operation planning tools that take into account the uncertainty of the wind-power. However, most of these methods typically require strict assumptions about the probabilistic behavior of the forecast error, and usually ignore the dynamic nature of the forecasting process. In this paper a methodological framework to obtain Robust Unit Commitment (UC) policies is presented; such methodology considers a novel scenario-based uncertainty model for wind power applications. The proposed method is composed by three main phases. The first two phases generate a sound wind-power forecast using a bootstrap predictive inference approach. The third phase corresponds to modeling and solving a one-day ahead Robust UC considering the output of the first phase. The performance of proposed approach is evaluated using as case study a new wind farm to be incorporated into the Northern Interconnected System (NIS) of Chile. A projection of wind-based power installation, as well as different characteristic of the uncertain data, are considered in this study

  7. Including cetaceans in multi-species assessment models using strandings data: why, how and what can we do about it?

    Directory of Open Access Journals (Sweden)

    Camilo Saavedra

    2014-07-01

    Full Text Available Single-species models have been commonly used to assess fish stocks in the past. Since these models have relatively simple data requirements, they sometimes provide the only tool available to assess the status of a stock when data are not enough to develop more complex models. However, these models have been criticized for several reasons since they provide reference points independently for each species assessed ignoring their interactions. For example, several studies suggest that even more substantial reductions in fishing mortality may be necessary to ensure MSY is reached when taking into consideration multiespecies interactions. Therefore, and as Pauly et al. (1998 stated, single-species analysis may mislead researchers and managers into neglecting the gear and trophic interactions which ultimately determine stocks long-term yields and ecosystem health. Ecosystem or multispecies models offer a number of advantages over single-species models. As stated in the workshop “Incorporating ecosystem considerations into stock assessments and management advice” (Mace, 2000 two general improvements are: a better appreciation of the fishing on ecosystem structure and function, and a better appreciation of the need to consider de value of marine ecosystems for functions other than harvesting fish. As disadvantages, multispecies models are statistically complex and include trophic relationships requiring more information (e.g. good estimations of biological parameters of each species and generally a full quantification of the diet sometimes available though the analysis of stomach contents. To reduce the number of species and therefore the amount of information needed, Minimum Realistic Models (MRMs represent an intermediate level of complexity, where only the subset of the ecosystem, important for the issue under consideration, is modeled. This approach offers the advantage of allowing a refinement of our estimates and can help answer more targeted

  8. The impact of including spatially longitudinal heterogeneities of vessel oxygen content and vascular fraction in 3D tumor oxygenation models on predicted radiation sensitivity.

    Science.gov (United States)

    Lagerlöf, Jakob H; Kindblom, Jon; Bernhardt, Peter

    2014-04-01

    Oxygen distribution models have been used to analyze the influences of oxygen tensions on tissue response after radiotherapy. These distributions are often generated assuming constant oxygen tension in the blood vessels. However, as red blood cells progress through the vessels, oxygen is continuously released into the plasma and the surrounding tissue, resulting in longitudinally varying oxygen levels in the blood vessels. In the present study, the authors investigated whether a tumor oxygenation model that incorporated longitudinally varying oxygen levels would provide different predictions of necrotic fractions and radiosensitivity compared to commonly used models with a constant oxygen pressure. Our models simulated oxygen diffusion based on a Green's function approach and oxygen consumption according to the Michaelis-Menten equation. The authors constructed tumor models with different vascular fractions (VFs), from which they generated depth oxygenation curves and a look-up table of oxygen pressure gradients. The authors evaluated models of spherical tumors of various sizes, from 1 to 10(4) mg. The authors compared the results from a model with constant vessel oxygen (CVO) pressure to those from models with longitudinal variations in oxygen saturation and either a constant VF (CVF) or variable VF (VVF) within the tumor tissue. The authors monitored the necrotic fractions, defined as tumor regions with an oxygen pressure below 1 mmHg. Tumor radiation sensitivity was expressed as D99, the homogeneous radiation dose required for a tumor control probability of 0.99. In the CVO saturation model, no necrosis was observed, and decreasing the VF could only decrease the D99 by up to 10%. Furthermore, the D99 vs VF dependence was similar for different tumor masses. Compared to the CVO model, the extended CVF and VVF models provided clearly different results, including pronounced effects of VF and tumor size on the necrotic fraction and D99, necrotic fractions ranging

  9. The baryonic spectrum in a constituent quark model including a three-body force

    International Nuclear Information System (INIS)

    Desplanques, B.; Gignoux, C.; Silvestre-Brac, B.; Gonzalez, P.; Navarro, J.; Noguera, S.

    1992-01-01

    We analyze, within a non-relativistic quark model, the low energy part of the baryonic spectrum in the octet and decuplet flavour representations. The relevance of a strong Coulomb potential is emphasized in order to explain its general features. The addition of a three-body force allows to solve the 'Roper puzzle', giving a consistent explanation to its relative position in the spectrum. (orig.)

  10. Description of the new version 4.0 of the tritium model UFOTRI including user guide

    International Nuclear Information System (INIS)

    Raskob, W.

    1993-08-01

    In view of the future operation of fusion reactors the release of tritium may play a dominant role during normal operation as well as after accidents. Because of its physical and chemical properties which differ significantly from those of other radionuclides, the model UFOTRI for assessing the radiological consequences of accidental tritium releases has been developed. It describes the behaviour of tritium in the biosphere and calculates the radiological impact on individuals and the population due to the direct exposure and by the ingestion pathways. Processes such as the conversion of tritium gas into tritiated water (HTO) in the soil, re-emission after deposition and the conversion of HTO into organically bound tritium, are considered. The use of UFOTRI in its probabilistic mode shows the spectrum of the radiological impact together with the associated probability of occurrence. A first model version was established in 1991. As the ongoing work on investigating the main processes of the tritium behaviour in the environment shows up new results, the model has been improved in several points. The report describes the changes incorporated into the model since 1991. Additionally provides the up-dated user guide for handling the revised UFOTRI version which will be distributed to interested organizations. (orig.) [de

  11. Model of spur processes in aqueous radiation chemistry including spur overlap and a novel initial hydrated electron distribution

    International Nuclear Information System (INIS)

    Short, D.R.

    1980-01-01

    Results are presented from computer calculations based upon an improved diffusion-kinetic model of the spur which includes a novel initial distribution for the hydrated electron and an approximate mathematical treatment of the overlap of spurs in three dimensions. Experimental data for the decay of the hydrated electron and hydroxyl radical before one in electron-pulse-irradated, solute-free and air-free water are fit wihtin experimental uncertainty by adjustment of the initial spatial distributions of spur intermediates and the average energy deposited in the spur. Using the same values of these parameters, the hydrated electron decay is computed for times from 1 ps 10 μs after the radiatio pulse. The results of such calcuations for various conditions of pulse dose and concentrations of scavengers of individual primary chemical species in the spur are compared with corresponding experimental data obtained predominantly from water and aqueous solutions irradiated with 10 to 15 MeV electron pulses. Very good agreement between calculated and experimental hydrated electron decay in pure water is observed for the entire time range studied when a pulse dose of approximately 7900 rads is modeled, but the calcuated and experimental curves are observed to deviate for times greater than 10 ns nanoseconds when low pulse doses and low scavenger concentrations are considered. It is shown that this deviation is experimental and calculated hydrated electron decay cannot be explained by assuming the presence of a hydrated electron scavenging impurity nor by employing a distribution of nearest neighbor interspur distances to refine the overlap approximation

  12. 40 CFR 60.3053 - What must I include in the deviation report?

    Science.gov (United States)

    2010-07-01

    ... 40 Protection of Environment 6 2010-07-01 2010-07-01 false What must I include in the deviation... PROGRAMS (CONTINUED) STANDARDS OF PERFORMANCE FOR NEW STATIONARY SOURCES Emission Guidelines and Compliance... Model Rule-Recordkeeping and Reporting § 60.3053 What must I include in the deviation report? In each...

  13. Extending the formal model of a spatial data infrastructure to include volunteered geographical information

    CSIR Research Space (South Africa)

    Cooper, Antony K

    2011-07-01

    Full Text Available , Information and Computational Viewpoints of the Reference Model for Open Distributed Processing (RM-ODP). We identified six stakeholders: Policy Maker, Producer, Provider, Broker, Value-added Reseller and End User. The Internet has spawned the development...

  14. A Lumped Thermal Model Including Thermal Coupling and Thermal Boundary Conditions for High Power IGBT Modules

    DEFF Research Database (Denmark)

    Bahman, Amir Sajjad; Ma, Ke; Blaabjerg, Frede

    2018-01-01

    Detailed thermal dynamics of high power IGBT modules are important information for the reliability analysis and thermal design of power electronic systems. However, the existing thermal models have their limits to correctly predict these complicated thermal behavior in the IGBTs: The typically used...... thermal model based on one-dimensional RC lumps have limits to provide temperature distributions inside the device, moreover some variable factors in the real-field applications like the cooling and heating conditions of the converter cannot be adapted. On the other hand, the more advanced three......-dimensional thermal models based on Finite Element Method (FEM) need massive computations, which make the long-term thermal dynamics difficult to calculate. In this paper, a new lumped three-dimensional thermal model is proposed, which can be easily characterized from FEM simulations and can acquire the critical...

  15. Multi-Objective Dynamic Economic Dispatch of Microgrid Systems Including Vehicle-to-Grid

    Directory of Open Access Journals (Sweden)

    Haitao Liu

    2015-05-01

    Full Text Available Based on the characteristics of electric vehicles (EVs, this paper establishes the load models of EVs under the autonomous charging mode and the coordinated charging and discharging mode. Integrating the EVs into a microgrid system which includes wind turbines (WTs, photovoltaic arrays (PVs, diesel engines (DEs, fuel cells (FCs and a storage battery (BS, this paper establishes multi-objective economic dispatch models of a microgrid, including the lowest operating cost, the least carbon dioxide emissions, and the lowest pollutant treatment cost. After converting the multi-objective functions to a single objective function by using the judgment matrix method, we analyze the dynamic economic dispatch of the microgrid system including vehicle-to-grid (V2G with an improved particle swarm optimization algorithm under different operation control strategies. With the example system, the proposed models and strategies are verified and analyzed. Simulation results show that the microgrid system with EVs under the coordinated charging and discharging mode has better operation economics than the autonomous charging mode. Meanwhile, the greater the load fluctuation is, the higher the operating cost of the microgrid system is.

  16. 25 CFR 1000.162 - What is included in a self-governance compact?

    Science.gov (United States)

    2010-04-01

    ... 25 Indians 2 2010-04-01 2010-04-01 false What is included in a self-governance compact? 1000.162...-DETERMINATION AND EDUCATION ACT Negotiation Process for Annual Funding Agreements Negotiating A Self-Governance Compact § 1000.162 What is included in a self-governance compact? A model format for self-governance...

  17. Molten pool-lower head integrity. Heat transfer models including advanced numerical simulations (DNS)

    Energy Technology Data Exchange (ETDEWEB)

    Seiler, J.M.; Bonnet, J.M.; Bernaz, L. [CEA Grenoble (France)

    2001-07-01

    Extensive studies have been performed to investigate the heat transfer within a molten corium pool (homogeneous, stratified and with miscibility gap): Synthesis of heat transfer correlations in molten pool (homogeneous and stratified), Focusing effect in stratified metal layer, DNS analysis of Rayleigh Benard instabilities at the top boundary; interpretation of the different convection regimes and exponents affecting the Rayleigh number in the heat transfer correlations, Molten pool model for corium presenting a miscibility gap. Condition for de-stratification. (authors)

  18. Molten pool-lower head integrity. Heat transfer models including advanced numerical simulations (DNS)

    International Nuclear Information System (INIS)

    Seiler, J.M.; Bonnet, J.M.; Bernaz, L.

    2001-01-01

    Extensive studies have been performed to investigate the heat transfer within a molten corium pool (homogeneous, stratified and with miscibility gap): Synthesis of heat transfer correlations in molten pool (homogeneous and stratified), Focusing effect in stratified metal layer, DNS analysis of Rayleigh Benard instabilities at the top boundary; interpretation of the different convection regimes and exponents affecting the Rayleigh number in the heat transfer correlations, Molten pool model for corium presenting a miscibility gap. Condition for de-stratification. (authors)

  19. porewater chemistry experiment at Mont Terri rock laboratory. Reactive transport modelling including bacterial activity

    International Nuclear Information System (INIS)

    Tournassat, Christophe; Gaucher, Eric C.; Leupin, Olivier X.; Wersin, Paul

    2010-01-01

    Document available in extended abstract form only. An in-situ test in the Opalinus Clay formation, termed pore water Chemistry (PC) experiment, was run for a period of five years. It was based on the concept of diffusive equilibration whereby traced water with a composition close to that expected in the formation was continuously circulated and monitored in a packed off borehole. The main original focus was to obtain reliable data on the pH/pCO 2 of the pore water, but because of unexpected microbially- induced redox reactions, the objective was then changed to elucidate the biogeochemical processes happening in the borehole and to understand their impact on pH/pCO 2 and pH in the low permeability clay formation. The biologically perturbed chemical evolution of the PC experiment was simulated with reactive transport models. The aim of this modelling exercise was to develop a 'minimal-' model able to reproduce the chemical evolution of the PC experiment, i.e. the chemical evolution of solute inorganic and organic compounds (organic carbon, dissolved inorganic carbon etc...) that are coupled with each other through the simultaneous occurrence of biological transformation of solute or solid compounds, in-diffusion and out-diffusion of solute species and precipitation/dissolution of minerals (in the borehole and in the formation). An accurate description of the initial chemical conditions in the surrounding formation together with simplified kinetics rule mimicking the different phases of bacterial activities allowed reproducing the evolution of all main measured parameters (e.g. pH, TOC). Analyses from the overcoring and these simulations evidence the high buffer capacity of Opalinus clay regarding chemical perturbations due to bacterial activity. This pH buffering capacity is mainly attributed to the carbonate system as well as to the clay surfaces reactivity. Glycerol leaching from the pH-electrode might be the primary organic source responsible for

  20. Situational effects of the school factors included in the dynamic model of educational effectiveness

    NARCIS (Netherlands)

    Creerners, Bert; Kyriakides, Leonidas

    We present results of a longitudinal study in which 50 schools, 113 classes and 2,542 Cypriot primary students participated. We tested the validity of the dynamic model of educational effectiveness and especially its assumption that the impact of school factors depends on the current situation of

  1. Emission-line diagnostics of nearby H II regions including interacting binary populations

    Science.gov (United States)

    Xiao, Lin; Stanway, Elizabeth R.; Eldridge, J. J.

    2018-06-01

    We present numerical models of the nebular emission from H II regions around young stellar populations over a range of compositions and ages. The synthetic stellar populations include both single stars and interacting binary stars. We compare these models to the observed emission lines of 254 H II regions of 13 nearby spiral galaxies and 21 dwarf galaxies drawn from archival data. The models are created using the combination of the BPASS (Binary Population and Spectral Synthesis) code with the photoionization code CLOUDY to study the differences caused by the inclusion of interacting binary stars in the stellar population. We obtain agreement with the observed emission line ratios from the nearby star-forming regions and discuss the effect of binary-star evolution pathways on the nebular ionization of H II regions. We find that at population ages above 10 Myr, single-star models rapidly decrease in flux and ionization strength, while binary-star models still produce strong flux and high [O III]/H β ratios. Our models can reproduce the metallicity of H II regions from spiral galaxies, but we find higher metallicities than previously estimated for the H II regions from dwarf galaxies. Comparing the equivalent width of H β emission between models and observations, we find that accounting for ionizing photon leakage can affect age estimates for H II regions. When it is included, the typical age derived for H II regions is 5 Myr from single-star models, and up to 10 Myr with binary-star models. This is due to the existence of binary-star evolution pathways, which produce more hot Wolf-Rayet and helium stars at older ages. For future reference, we calculate new BPASS binary maximal starburst lines as a function of metallicity, and for the total model population, and present these in Appendix A.

  2. Modeling the elastic behavior of ductile cast iron including anisotropy in the graphite nodules

    DEFF Research Database (Denmark)

    Andriollo, Tito; Thorborg, Jesper; Hattel, Jesper Henri

    2016-01-01

    by means of a 3D periodic unit cell model. In this respect, an explicit procedure to enforce both periodic displacement and periodic traction boundary conditions in ABAQUS is presented, and the importance of fulfilling the traction continuity conditions at the unit cell boundaries is discussed. It is shown...

  3. Model for CO2 leakage including multiple geological layers and multiple leaky wells.

    Science.gov (United States)

    Nordbotten, Jan M; Kavetski, Dmitri; Celia, Michael A; Bachu, Stefan

    2009-02-01

    Geological storage of carbon dioxide (CO2) is likely to be an integral component of any realistic plan to reduce anthropogenic greenhouse gas emissions. In conjunction with large-scale deployment of carbon storage as a technology, there is an urgent need for tools which provide reliable and quick assessments of aquifer storage performance. Previously, abandoned wells from over a century of oil and gas exploration and production have been identified as critical potential leakage paths. The practical importance of abandoned wells is emphasized by the correlation of heavy CO2 emitters (typically associated with industrialized areas) to oil and gas producing regions in North America. Herein, we describe a novel framework for predicting the leakage from large numbers of abandoned wells, forming leakage paths connecting multiple subsurface permeable formations. The framework is designed to exploit analytical solutions to various components of the problem and, ultimately, leads to a grid-free approximation to CO2 and brine leakage rates, as well as fluid distributions. We apply our model in a comparison to an established numerical solverforthe underlying governing equations. Thereafter, we demonstrate the capabilities of the model on typical field data taken from the vicinity of Edmonton, Alberta. This data set consists of over 500 wells and 7 permeable formations. Results show the flexibility and utility of the solution methods, and highlight the role that analytical and semianalytical solutions can play in this important problem.

  4. Briefing package for the Yucca Flat pre-emptive review, including overview, UZ model, SZ volcanics model and summary and conclusions sections

    Energy Technology Data Exchange (ETDEWEB)

    Kwicklis, Edward Michael [Los Alamos National Laboratory; Keating, Elizabeth H [Los Alamos National Laboratory

    2010-12-02

    Much progress has been made in the last several years in modeling radionuclide transport from tests conducted both in the unsaturated zone and saturated volcanic rocks of Yucca Flat, Nevada. The presentations to the DOE NNSA pre-emptive review panel contained herein document the progress to date, and discuss preliminary conclusions regarding the present and future extents of contamination resulting from past nuclear tests. The presentations also discuss possible strategies for addressing uncertainty in the model results.

  5. Including fluid shear viscosity in a structural acoustic finite element model using a scalar fluid representation.

    Science.gov (United States)

    Cheng, Lei; Li, Yizeng; Grosh, Karl

    2013-08-15

    An approximate boundary condition is developed in this paper to model fluid shear viscosity at boundaries of coupled fluid-structure system. The effect of shear viscosity is approximated by a correction term to the inviscid boundary condition, written in terms of second order in-plane derivatives of pressure. Both thin and thick viscous boundary layer approximations are formulated; the latter subsumes the former. These approximations are used to develop a variational formation, upon which a viscous finite element method (FEM) model is based, requiring only minor modifications to the boundary integral contributions of an existing inviscid FEM model. Since this FEM formulation has only one degree of freedom for pressure, it holds a great computational advantage over the conventional viscous FEM formulation which requires discretization of the full set of linearized Navier-Stokes equations. The results from thick viscous boundary layer approximation are found to be in good agreement with the prediction from a Navier-Stokes model. When applicable, thin viscous boundary layer approximation also gives accurate results with computational simplicity compared to the thick boundary layer formulation. Direct comparison of simulation results using the boundary layer approximations and a full, linearized Navier-Stokes model are made and used to evaluate the accuracy of the approximate technique. Guidelines are given for the parameter ranges over which the accurate application of the thick and thin boundary approximations can be used for a fluid-structure interaction problem.

  6. Machado-Joseph Disease in Pedigrees of Azorean descent is Linked to Chromosome 14

    OpenAIRE

    George-Hyslop, P. St; Rogaeva, E.; Huterer, J.; Tsuda, T.; Santos, J.; Haines, J. L.; Schlumpf, K.; Rogaev, E. I.; Liang, Y.; McLachlan, D. R. Crapper; Kennedy, J.; Weissenbach, J.; Billingsley, G. D.; Cox, D. W.; Lang, A. E.

    1994-01-01

    A locus for Machado-Joseph disease (MJD) has recently been mapped to a 30-cM region of chromosome 14q in five pedigrees of Japanese descent. MJD is a clinically pleomorphic neurodegenerative disease that was originally described in subjects of Azorean descent. In light of the nonallelic heterogeneity in other inherited spinocere-bellar ataxias, we were interested to determine if the MJD phenotype in Japanese and Azorean pedigrees arose from mutations at the same locus. We provide evidence tha...

  7. Unification of all elementary-particle forces including gravity

    International Nuclear Information System (INIS)

    Terazawa, Hidezumi; Chikashige, Yuichi; Matsuki, Takayuki; Akama, Keiichi.

    1978-07-01

    A unified model of the Nambu-Jona-Lasinio type for all elementary-particle forces including gravity is reviewed in some detail. Starting with a nonlinear fermion Lagrangian of the Heisenberg type and imposing the massless conditions of Bjorken on vector auxiliary fields, on effective Lagrangian is constructed, which combines the unified SU (2) x U (1) gauge theory of Weinberg and Salam for the weak and electromagnetic interactions of leptons and quarks and the Yang-Mills gauge theory of color SU (3) for the strong interaction of quarks. The photon, the weak vector bosons, and the physical Higgs scalar appear as collective excitations of lepton-antilepton or quark-antiquark pairs while the color-octet gluons appear as those of quark-antiquark pairs. The most important results of this unified model are presented. The Weinberg angle and the gluon coupling constant are determined, and the masses of the weak vector bosons are predicted. (Yoshimori, M.)

  8. Probing the structural and dynamical properties of liquid water with models including non-local electron correlation

    International Nuclear Information System (INIS)

    Del Ben, Mauro; Hutter, Jürg; VandeVondele, Joost

    2015-01-01

    Water is a ubiquitous liquid that displays a wide range of anomalous properties and has a delicate structure that challenges experiment and simulation alike. The various intermolecular interactions that play an important role, such as repulsion, polarization, hydrogen bonding, and van der Waals interactions, are often difficult to reproduce faithfully in atomistic models. Here, electronic structure theories including all these interactions at equal footing, which requires the inclusion of non-local electron correlation, are used to describe structure and dynamics of bulk liquid water. Isobaric-isothermal (NpT) ensemble simulations based on the Random Phase Approximation (RPA) yield excellent density (0.994 g/ml) and fair radial distribution functions, while various other density functional approximations produce scattered results (0.8-1.2 g/ml). Molecular dynamics simulation in the microcanonical (NVE) ensemble based on Møller-Plesset perturbation theory (MP2) yields dynamical properties in the condensed phase, namely, the infrared spectrum and diffusion constant. At the MP2 and RPA levels of theory, ice is correctly predicted to float on water, resolving one of the anomalies as resulting from a delicate balance between van der Waals and hydrogen bonding interactions. For several properties, obtaining quantitative agreement with experiment requires correction for nuclear quantum effects (NQEs), highlighting their importance, for structure, dynamics, and electronic properties. A computed NQE shift of 0.6 eV for the band gap and absorption spectrum illustrates the latter. Giving access to both structure and dynamics of condensed phase systems, non-local electron correlation will increasingly be used to study systems where weak interactions are of paramount importance

  9. 40 CFR 60.3012 - What should I include in my waste management plan?

    Science.gov (United States)

    2010-07-01

    ..., 2004 Model Rule-Waste Management Plan § 60.3012 What should I include in my waste management plan? A waste management plan must include consideration of the reduction or separation of waste-stream elements.... The plan must identify any additional waste management measures and implement those measures the...

  10. 40 CFR 60.2630 - What should I include in my waste management plan?

    Science.gov (United States)

    2010-07-01

    ... or Before November 30, 1999 Model Rule-Waste Management Plan § 60.2630 What should I include in my waste management plan? A waste management plan must include consideration of the reduction or separation... of recyclable materials. The plan must identify any additional waste management measures, and the...

  11. [Analysis of 14 individuals who requested predictive genetic testing for hereditary neuromuscular diseases].

    Science.gov (United States)

    Yoshida, Kunihiro; Tamai, Mariko; Kubota, Takeo; Kawame, Hiroshi; Amano, Naoji; Ikeda, Shu-ichi; Fukushima, Yoshimitsu

    2002-02-01

    Predictive genetic testing for hereditary neuromuscular diseases is a delicate issue for individuals at risk and their families, as well as for medical staff because these diseases are often late-onset and intractable. Therefore careful pre- and post-test genetic counseling and psychosocial support should be provided along with such genetic testing. The Division of Clinical and Molecular Genetics was established at our hospital in May 1996 to provide skilled professional genetic counseling. Since its establishment, 14 individuals have visited our clinic to request predictive genetic testing for hereditary neuromuscular diseases (4 for myotonic dystrophy, 6 for spinocerebellar ataxia, 3 for Huntington's disease, and 1 for Alzheimer's disease). The main reasons for considering testing were to remove uncertainty about the genetic status and to plan for the future. Nine of 14 individuals requested testing for making decisions about a forthcoming marriage or pregnancy (family planning). Other reasons raised by the individuals included career or financial planning, planning for their own health care, and knowing the risk for their children. At the first genetic counseling session, all of the individuals expressed hopes of not being a gene carrier and of escaping from fear of disease, and seemed not to be mentally well prepared for an increased-risk result. To date, 7 of the 14 individuals have received genetic testing and only one, who underwent predictive genetic testing for spinocerebellar ataxia, was given an increased-risk result. The seven individuals including the one with an increased-risk result, have coped well with their new knowledge about their genetic status after the testing results were disclosed. None of them has expressed regret. In pre-test genetic counseling sessions, we consider it quite important not only to determine the psychological status of the individual, but also to make the individual try to anticipate the changes in his/her life upon

  12. Coupled storm-time magnetosphere-ionosphere-thermosphere simulations including microscopic ionospheric turbulence

    Science.gov (United States)

    Merkin, V. G.; Wiltberger, M. J.; Zhang, B.; Liu, J.; Wang, W.; Dimant, Y. S.; Oppenheim, M. M.; Lyon, J.

    2017-12-01

    During geomagnetic storms the magnetosphere-ionosphere-thermosphere system becomes activated in ways that are unique to disturbed conditions. This leads to emergence of physical feedback loops that provide tighter coupling between the system elements, often operating across disparate spatial and temporal scales. One such process that has recently received renewed interest is the generation of microscopic ionospheric turbulence in the electrojet regions (electrojet turbulence, ET) that results from strong convective electric fields imposed by the solar wind-magnetosphere interaction. ET leads to anomalous electron heating and generation of non-linear Pedersen current - both of which result in significant increases in effective ionospheric conductances. This, in turn, provides strong non-linear feedback on the magnetosphere. Recently, our group has published two studies aiming at a comprehensive analysis of the global effects of this microscopic process on the magnetosphere-ionosphere-thermosphere system. In one study, ET physics was incorporated in the TIEGCM model of the ionosphere-thermosphere. In the other study, ad hoc corrections to the ionospheric conductances based on ET theory were incorporated in the conductance module of the Lyon-Fedder-Mobarry (LFM) global magnetosphere model. In this presentation, we make the final step toward the full coupling of the microscopic ET physics within our global coupled model including LFM, the Rice Convection Model (RCM) and TIEGCM. To this end, ET effects are incorporated in the TIEGCM model and propagate throughout the system via thus modified TIEGCM conductances. The March 17, 2013 geomagnetic storm is used as a testbed for these fully coupled simulations, and the results of the model are compared with various ionospheric and magnetospheric observatories, including DMSP, AMPERE, and Van Allen Probes. Via these comparisons, we investigate, in particular, the ET effects on the global magnetosphere indicators such as the

  13. Four Models Including Fish, Seafood, Red Meat and Enriched Foods to Achieve Australian Dietary Recommendations for n-3 LCPUFA for All Life-Stages

    Directory of Open Access Journals (Sweden)

    Flavia Fayet-Moore

    2015-10-01

    Full Text Available Populations are not meeting recommended intakes of omega-3 long chain polyunsaturated fatty acids (n-3 LCPUFA. The aim was (i to develop a database on n-3 LCPUFA enriched products; (ii to undertake dietary modelling exercise using four dietary approaches to meet the recommendations and (iii to determine the cost of the models. Six n-3 LCPUFA enriched foods were identified. Fish was categorised by n-3 LCPUFA content (mg/100 g categories as “excellent” “good” and “moderate”. The four models to meet recommended n-3 LCPUFA intakes were (i fish only; (ii moderate fish (with red meat and enriched foods; (iii fish avoiders (red meat and enriched foods only; and (iv lacto-ovo vegetarian diet (enriched foods only. Diets were modelled using the NUTTAB2010 database and n-3 LCPUFA were calculated and compared to the Suggested Dietary Targets (SDT. The cost of meeting these recommendations was calculated per 100 mg n-3 LCPUFA. The SDT were achieved for all life-stages with all four models. The weekly food intake in number of serves to meet the n-3 LCPUFA SDT for all life-stages for each dietary model were: (i 2 “excellent” fish; (ii 1 “excellent” and 1 “good” fish, and depending on life-stage, 3–4 lean red meat, 0–2 eggs and 3–26 enriched foods; (iii 4 lean red meat, and 20–59 enriched foods; (iv 37–66 enriched foods. Recommended intakes of n-3 LCPUFA were easily met by the consumption of fish, which was the cheapest source of n-3 LCPUFA. Other strategies may be required to achieve the recommendations including modifying the current food supply through feeding practices, novel plant sources and more enriched foods.

  14. Aerodynamic analysis of the Darrieus rotor including secondary effects

    Science.gov (United States)

    Paraschivoiu, I.; Delclaux, F.; Fraunie, P.; Beguier, C.

    1983-10-01

    An aerodynamic analysis is made of two variants of the two-actuator-disk theory for modeling the Darrieus wind turbine. The double-multiple-streamtube model with constant and variable interference factors, including secondary effects, is examined for a Darrieus rotor. The influence of the secondary effects, namely, the blade geometry and profile type, the rotating tower, and the presence of struts and aerodynamic spoilers, is relatively significant, especially at high tip-speed ratios. Variation of the induced velocity as a function of the azimuthal angle allows a more accurate calculation of the aerodynamic loads on the downwind zone of the rotor with respect to the assumed constant interference factors. The theoretical results were compared with available experimental data for the Magdalen Islands wind turbine and Sandia-type machines (straight-line/circular-arc shape).

  15. Analysis of Smart Composite Structures Including Debonding

    Science.gov (United States)

    Chattopadhyay, Aditi; Seeley, Charles E.

    1997-01-01

    Smart composite structures with distributed sensors and actuators have the capability to actively respond to a changing environment while offering significant weight savings and additional passive controllability through ply tailoring. Piezoelectric sensing and actuation of composite laminates is the most promising concept due to the static and dynamic control capabilities. Essential to the implementation of these smart composites are the development of accurate and efficient modeling techniques and experimental validation. This research addresses each of these important topics. A refined higher order theory is developed to model composite structures with surface bonded or embedded piezoelectric transducers. These transducers are used as both sensors and actuators for closed loop control. The theory accurately captures the transverse shear deformation through the thickness of the smart composite laminate while satisfying stress free boundary conditions on the free surfaces. The theory is extended to include the effect of debonding at the actuator-laminate interface. The developed analytical model is implemented using the finite element method utilizing an induced strain approach for computational efficiency. This allows general laminate geometries and boundary conditions to be analyzed. The state space control equations are developed to allow flexibility in the design of the control system. Circuit concepts are also discussed. Static and dynamic results of smart composite structures, obtained using the higher order theory, are correlated with available analytical data. Comparisons, including debonded laminates, are also made with a general purpose finite element code and available experimental data. Overall, very good agreement is observed. Convergence of the finite element implementation of the higher order theory is shown with exact solutions. Additional results demonstrate the utility of the developed theory to study piezoelectric actuation of composite

  16. Model - including thermal creep effects - for the analysis of three-dimensional concrete structures

    International Nuclear Information System (INIS)

    Rodriguez, C.; Rebora, B.; Favrod, J.D.

    1979-01-01

    This article presents the most recent developments and results of research carried out by IPEN to establish a mathematical model for the non-linear rheological three-dimensional analysis of massive prestressed concrete structures. The main point of these latest developments is the simulation of the creep of concrete submitted to high temperatures over a long period of time. This research, financed by the Swiss National Science Foundation, has taken an increased importance with the advent of nuclear reactor vessels of the HHT type and new conceptions concerning the cooling of their concrete (replacement of the thermal insulation by a zone of hot concrete). (orig.)

  17. Able but unintelligent: including positively stereotyped black subgroups in the stereotype content model.

    Science.gov (United States)

    Walzer, Amy S; Czopp, Alexander M

    2011-01-01

    The stereotype content model (SCM) posits that warmth and competence are the key components underlying judgments about social groups. Because competence can encompass different components (e.g., intelligence, talent) different group members may be perceived to be competent for different reasons. Therefore, we believe it may be important to specify the type of competence being assessed when examining perceptions of groups that are positively stereotyped (i.e., Black athletes and musical Blacks). Consistent with the SCM, these subgroups were perceived as high in competence-talent but not in competence-intelligence and low in warmth. Both the intelligence and talent frame of competence fit in the SCM's social structural hypothesis.

  18. Unified model to the Tungsten inert Gas welding process including the cathode, the plasma and the anode

    International Nuclear Information System (INIS)

    Brochard, M.

    2009-06-01

    During this work, a 2D axially symmetric model of a TIG arc welding process had been developed in order to predict for given welding parameters, the needed variables for a designer of welded assembly: the heat input on the work piece, the weld pool geometry,... The developed model, using the Cast3M finite elements software, deals with the physical phenomena acting in each part of the process: the cathode, the plasma, the work piece with a weld pool, and the interfaces between these parts. To solve this model, the thermohydraulics equations are coupled with the electromagnetic equations that are calculated in part using the least squares finite element method. The beginning of the model validation consisted in comparing the results obtained with the ones available in the scientific literature. Thus, this step points out the action of each force in the weld pool, the contribution of each heat flux in the energy balance. Finally, to validate the model predictiveness, experimental and numerical sensitivity analyses were conducted using a design of experiments approach. The effects of the process current, the arc gap and the electrode tip angle on the weld pool geometry and the energy transferred to the work piece and the arc efficiency were studied. The good agreement obtained by the developed model for these outputs shows the good reproduction of the process physics. (author)

  19. Reliability evaluation of containments including soil-structure interaction

    International Nuclear Information System (INIS)

    Pires, J.; Hwang, H.; Reich, M.

    1985-12-01

    Soil-structure interaction effects on the reliability assessment of containment structures are examined. The probability-based method for reliability evaluation of nuclear structures developed at Brookhaven National Laboratory is extended to include soil-structure interaction effects. In this method, reliability of structures is expressed in terms of limit state probabilities. Furthermore, random vibration theory is utilized to calculate limit state probabilities under random seismic loads. Earthquake ground motion is modeled by a segment of a zero-mean, stationary, filtered Gaussian white noise random process, represented by its power spectrum. All possible seismic hazards at a site, represented by a hazard curve, are also included in the analysis. The soil-foundation system is represented by a rigid surface foundation on an elastic halfspace. Random and other uncertainties in the strength properties of the structure, in the stiffness and internal damping of the soil, are also included in the analysis. Finally, a realistic reinforced concrete containment is analyzed to demonstrate the application of the method. For this containment, the soil-structure interaction effects on; (1) limit state probabilities, (2) structural fragility curves, (3) floor response spectra with probabilistic content, and (4) correlation coefficients for total acceleration response at specified structural locations, are examined in detail. 25 refs., 21 figs., 12 tabs

  20. Structure validation of the Josephin domain of ataxin-3: Conclusive evidence for an open conformation

    International Nuclear Information System (INIS)

    Nicastro, Giuseppe; Habeck, Michael; Masino, Laura; Svergun, Dmitri I.; Pastore, Annalisa

    2006-01-01

    The availability of new and fast tools in structure determination has led to a more than exponential growth of the number of structures solved per year. It is therefore increasingly essential to assess the accuracy of the new structures by reliable approaches able to assist validation. Here, we discuss a specific example in which the use of different complementary techniques, which include Bayesian methods and small angle scattering, resulted essential for validating the two currently available structures of the Josephin domain of ataxin-3, a protein involved in the ubiquitin/proteasome pathway and responsible for neurodegenerative spinocerebellar ataxia of type 3. Taken together, our results demonstrate that only one of the two structures is compatible with the experimental information. Based on the high precision of our refined structure, we show that Josephin contains an open cleft which could be directly implicated in the interaction with polyubiquitin chains and other partners

  1. Model-Based Brake Control including Tyre Behaviour

    NARCIS (Netherlands)

    De Vries, E.J.H.

    2012-01-01

    The objective of the thesis is to develop a method for controlled braking of a vehicle. The brake pedal depression has been considered to be proportional to the intended deceleration. The brake controller is not aimed to replace a cruise control; it will have an anti-lock braking (ABS) function. The

  2. Three dimensional model for particle saltation close to stream beds, including a detailed description of the particle interaction with turbulence and inter-particle collisions

    KAUST Repository

    Moreno, Pablo M.

    2011-05-19

    We present in this paper a new three-dimensional (3-D) model for bed-load sediment transport, based on a Lagrangian description. We analyze generalized sub-models for the velocities after collision and the representation of the bed-roughness. The free-flight sub-model includes the effect of several forces, such as buoyancy, drag, virtual mass, lift, Basset and Magnus, and also addresses the particle rotation. A recent methodology for saving computational time in the Basset force is also employed. The sub-models for the post-collision velocity and rotation are based on the conservation of linear and angular momentum during the collision with the bed. We develop a new 3-D representation for the bed roughness by using geometric considerations. In order to address the interaction of particles with the turbulent flow, we tracked the particles through a computed turbulent velocity field for a smooth flat plate. This velocity field was used as a surrogate of the 3-D turbulent conditions close to the bed in streams. We first checked that the basic turbulence statistics for this velocity field could be used to approximate those in an open-channel flow. We then analyzed the interaction of the sediment and the turbulence for a single and multiple particles. We compared numerical results with experimental data obtained by Niño and García (1998b). We show that model predictions are in good agreement with existing data, in the sand size range. © 2011 ASCE.

  3. Three dimensional model for particle saltation close to stream beds, including a detailed description of the particle interaction with turbulence and inter-particle collisions

    KAUST Repository

    Moreno, Pablo M.; Bombardelli, Fabiá n A.; Gonzá lez, Andrea E.; Calo, Victor M.

    2011-01-01

    We present in this paper a new three-dimensional (3-D) model for bed-load sediment transport, based on a Lagrangian description. We analyze generalized sub-models for the velocities after collision and the representation of the bed-roughness. The free-flight sub-model includes the effect of several forces, such as buoyancy, drag, virtual mass, lift, Basset and Magnus, and also addresses the particle rotation. A recent methodology for saving computational time in the Basset force is also employed. The sub-models for the post-collision velocity and rotation are based on the conservation of linear and angular momentum during the collision with the bed. We develop a new 3-D representation for the bed roughness by using geometric considerations. In order to address the interaction of particles with the turbulent flow, we tracked the particles through a computed turbulent velocity field for a smooth flat plate. This velocity field was used as a surrogate of the 3-D turbulent conditions close to the bed in streams. We first checked that the basic turbulence statistics for this velocity field could be used to approximate those in an open-channel flow. We then analyzed the interaction of the sediment and the turbulence for a single and multiple particles. We compared numerical results with experimental data obtained by Niño and García (1998b). We show that model predictions are in good agreement with existing data, in the sand size range. © 2011 ASCE.

  4. A depth-averaged debris-flow model that includes the effects of evolving dilatancy. I. physical basis

    Science.gov (United States)

    Iverson, Richard M.; George, David L.

    2014-01-01

    To simulate debris-flow behaviour from initiation to deposition, we derive a depth-averaged, two-phase model that combines concepts of critical-state soil mechanics, grain-flow mechanics and fluid mechanics. The model's balance equations describe coupled evolution of the solid volume fraction, m, basal pore-fluid pressure, flow thickness and two components of flow velocity. Basal friction is evaluated using a generalized Coulomb rule, and fluid motion is evaluated in a frame of reference that translates with the velocity of the granular phase, vs. Source terms in each of the depth-averaged balance equations account for the influence of the granular dilation rate, defined as the depth integral of ∇⋅vs. Calculation of the dilation rate involves the effects of an elastic compressibility and an inelastic dilatancy angle proportional to m−meq, where meq is the value of m in equilibrium with the ambient stress state and flow rate. Normalization of the model equations shows that predicted debris-flow behaviour depends principally on the initial value of m−meq and on the ratio of two fundamental timescales. One of these timescales governs downslope debris-flow motion, and the other governs pore-pressure relaxation that modifies Coulomb friction and regulates evolution of m. A companion paper presents a suite of model predictions and tests.

  5. NLO QCD corrections to Higgs pair production including dimension-6 operators

    Energy Technology Data Exchange (ETDEWEB)

    Groeber, Ramona [INFN, Sezione di Roma Tre, Roma (Italy); Muehlleitner, Margarete; Streicher, Juraj [Karlsruher Institut fuer Technologie (KIT), Karlsruhe (Germany). Institut fuer Theoretische Physik; Spira, Michael [Paul Scherrer Institut, Villigen (Switzerland)

    2016-07-01

    The role of the Higgs boson has developed from the long-sought particle into a tool for exploring beyond Standard Model (BSM) physics. While the Higgs boson signal strengths are close to the values predicted in the Standard Model (SM), the trilinear Higgs-selfcoupling can still deviate significantly from the SM expectations in some BSM scenarios. The Effective Field Theory (EFT) framework provides a way to describe these deviations in a rather model independent way, by including higher-dimensional operators which modify the Higgs boson couplings and induce novel couplings not present in the SM. The trilinear Higgs-selfcoupling is accessible in Higgs pair production, for which the gluon fusion is the dominant production channel. The next-to-leading (NLO) QCD corrections to this process are important for a proper prediction of the cross section and are known in the limit of heavy top quark masses. In our work, we provide the NLO QCD corrections in the large top quark mass limit to Higgs pair production including dimension-6 operators. The various higher-dimensional contributions are affected differently by the QCD corrections, leading to deviations in the relative NLO QCD corrections of several per-cent, while modifying the cross section by up to an order of magnitude.

  6. Multibody Dynamics of a Fluid Power Radial Piston Motor Including Transient Hydrodynamic Pressure Models of Lubricating Gaps

    DEFF Research Database (Denmark)

    Johansen, Per; Rømer, Daniel; Andersen, Torben Ole

    2014-01-01

    The increasing interest in hydraulic transmissions in wind and wave energy applications has created an incentive for the development of high efficiency fluid power machinery. Modeling and analysis of fluid power machinery loss mechanisms are necessary in order to accommodate this demand. At present...... fully coupled thermo-elastic models has been used to simulate and study loss mechanisms in various tribological interfaces. Consequently, a reasonable focus of further development is to couple the interface models and the rigid body mechanics of fluid power machinery. The focus of the current paper...

  7. Modeling of damage in ductile cast iron – The effect of including plasticity in the graphite noduless

    DEFF Research Database (Denmark)

    Andriollo, Tito; Thorborg, Jesper; Tiedje, Niels Skat

    2015-01-01

    In the present paper a micro-mechanical model for investigating the stress-strain relation of ductile cast iron subjected to simple loading conditions is presented. The model is based on a unit cell containing a single spherical graphite nodule embedded in a uniform ferritic matrix, under...... the assumption of infinitesimal strains and plane-stress conditions. Despite the latter being a limitation with respect to full 3D models, it allows a direct comparison with experimental investigations of damage evolution on the surface of ductile cast iron components, where the stress state is biaxial in nature...

  8. Optimization of piezoelectric cantilever energy harvesters including non-linear effects

    International Nuclear Information System (INIS)

    Patel, R; McWilliam, S; Popov, A A

    2014-01-01

    This paper proposes a versatile non-linear model for predicting piezoelectric energy harvester performance. The presented model includes (i) material non-linearity, for both substrate and piezoelectric layers, and (ii) geometric non-linearity incorporated by assuming inextensibility and accurately representing beam curvature. The addition of a sub-model, which utilizes the transfer matrix method to predict eigenfrequencies and eigenvectors for segmented beams, allows for accurate optimization of piezoelectric layer coverage. A validation of the overall theoretical model is performed through experimental testing on both uniform and non-uniform samples manufactured in-house. For the harvester composition used in this work, the magnitude of material non-linearity exhibited by the piezoelectric layer is 35 times greater than that of the substrate layer. It is also observed that material non-linearity, responsible for reductions in resonant frequency with increases in base acceleration, is dominant over geometric non-linearity for standard piezoelectric harvesting devices. Finally, over the tested range, energy loss due to damping is found to increase in a quasi-linear fashion with base acceleration. During an optimization study on piezoelectric layer coverage, results from the developed model were compared with those from a linear model. Unbiased comparisons between harvesters were realized by using devices with identical natural frequencies—created by adjusting the device substrate thickness. Results from three studies, each with a different assumption on mechanical damping variations, are presented. Findings showed that, depending on damping variation, a non-linear model is essential for such optimization studies with each model predicting vastly differing optimum configurations. (paper)

  9. Tyrosyl-DNA Phosphodiesterase I a critical survival factor for neuronal development and homeostasis.

    Science.gov (United States)

    van Waardenburg, Robert C A M

    2016-01-01

    Tyrosyl-DNA phosphodiesterase I (TDP1), like most DNA repair associated proteins, is not essential for cell viability. However, dysfunctioning TDP1 or ATM (ataxia telangiectasia mutated) results in autosomal recessive neuropathology with similar phenotypes, including cerebellar atrophy. Dual inactivation of TDP1 and ATM causes synthetic lethality. A TDP1H 493 R catalytic mutant is associated with spinocerebellar ataxia with axonal neuropathy (SCAN1), and stabilizes the TDP1 catalytic obligatory enzyme-DNA covalent complex. The ATM kinase activates proteins early on in response to DNA damage. Tdp1-/- and Atm-/- mice exhibit accumulation of DNA topoisomerase I-DNA covalent complexes (TOPO1-cc) explicitly in neuronal tissue during development. TDP1 resolves 3'- and 5'-DNA adducts including trapped TOPO1-cc and TOPO1 protease resistant peptide-DNA complex. ATM appears to regulate the response to TOPO1-cc via a noncanonical function by regulating SUMO/ubiquitin-mediated TOPO1 degradation. In conclusion, TDP1 and ATM are critical factors for neuronal cell viability via two independent but cooperative pathways.

  10. Dynamic Analysis of Wind Turbines Including Soil-Structure Interaction

    DEFF Research Database (Denmark)

    Harte, M.; Basu, B.; Nielsen, Søren R.K.

    2012-01-01

    This paper investigates the along-wind forced vibration response of an onshore wind turbine. The study includes the dynamic interaction effects between the foundation and the underlying soil, as softer soils can influence the dynamic response of wind turbines. A Multi-Degree-of-Freedom (MDOF......) horizontal axes onshore wind turbine model is developed for dynamic analysis using an Euler–Lagrangian approach. The model is comprised of a rotor blade system, a nacelle and a flexible tower connected to a foundation system using a substructuring approach. The rotor blade system consists of three rotating...... for displacement of the turbine system are obtained and the modal frequencies of the combined turbine-foundation system are estimated. Simulations are presented for the MDOF turbine structure subjected to wind loading for different soil stiffness conditions. Steady state and turbulent wind loading, developed using...

  11. Digital Elevation Model (DEM), DEM data are useful for terrain analysis and modeling including slope and aspect calculations. They may be used to produced shaded relief maps and contour maps., Published in 2001, 1:24000 (1in=2000ft) scale, Louisiana State University (LSU).

    Data.gov (United States)

    NSGIC Education | GIS Inventory — Digital Elevation Model (DEM) dataset current as of 2001. DEM data are useful for terrain analysis and modeling including slope and aspect calculations. They may be...

  12. A model for optimal offspring size in fish, including live-bearing and parental effects.

    Science.gov (United States)

    Jørgensen, Christian; Auer, Sonya K; Reznick, David N

    2011-05-01

    Since Smith and Fretwell's seminal article in 1974 on the optimal offspring size, most theory has assumed a trade-off between offspring number and offspring fitness, where larger offspring have better survival or fitness, but with diminishing returns. In this article, we use two ubiquitous biological mechanisms to derive the shape of this trade-off: the offspring's growth rate combined with its size-dependent mortality (predation). For a large parameter region, we obtain the same sigmoid relationship between offspring size and offspring survival as Smith and Fretwell, but we also identify parameter regions where the optimal offspring size is as small or as large as possible. With increasing growth rate, the optimal offspring size is smaller. We then integrate our model with strategies of parental care. Egg guarding that reduces egg mortality favors smaller or larger offspring, depending on how mortality scales with size. For live-bearers, the survival of offspring to birth is a function of maternal survival; if the mother's survival increases with her size, then the model predicts that larger mothers should produce larger offspring. When using parameters for Trinidadian guppies Poecilia reticulata, differences in both growth and size-dependent predation are required to predict observed differences in offspring size between wild populations from high- and low-predation environments.

  13. The importance of including dynamic social networks when modeling epidemics of airborne infections: does increasing complexity increase accuracy?

    OpenAIRE

    Blower, Sally; Go, Myong-Hyun

    2011-01-01

    Abstract Mathematical models are useful tools for understanding and predicting epidemics. A recent innovative modeling study by Stehle and colleagues addressed the issue of how complex models need to be to ensure accuracy. The authors collected data on face-to-face contacts during a two-day conference. They then constructed a series of dynamic social contact networks, each of which was used to model an epidemic generated by a fast-spreading airborne pathogen. Intriguingly, Stehle and colleagu...

  14. [Neuroanatomy of Isolated Body Lateropulsion].

    Science.gov (United States)

    Nakazato, Yoshihiko; Tamura, Naotoshi; Ikeda, Kei; Tanaka, Ai; Yamamoto, Toshimasa

    2016-03-01

    Axial body lateropulsion, a phenomenon where the body is pulled toward the side of the lesion, with tendency of falling down, is the well-known transient feature of lateral medullary syndrome. In some cases, axial body lateropulsion occurs without vestibular and cerebellar symptoms (isolated body lateropulsion:[iBL]). Patients with iBL have a lesion located in the spinocerebellar tract, descending lateral vestibulospinal tract, vestibulo-thalamic pathway, dentatorubrothalamic pathway, or thalamocortical fascicle. This review deals with the anatomic basis and clinical significance of iBL.

  15. The importance of including dynamic social networks when modeling epidemics of airborne infections: does increasing complexity increase accuracy?

    Science.gov (United States)

    Blower, Sally; Go, Myong-Hyun

    2011-07-19

    Mathematical models are useful tools for understanding and predicting epidemics. A recent innovative modeling study by Stehle and colleagues addressed the issue of how complex models need to be to ensure accuracy. The authors collected data on face-to-face contacts during a two-day conference. They then constructed a series of dynamic social contact networks, each of which was used to model an epidemic generated by a fast-spreading airborne pathogen. Intriguingly, Stehle and colleagues found that increasing model complexity did not always increase accuracy. Specifically, the most detailed contact network and a simplified version of this network generated very similar results. These results are extremely interesting and require further exploration to determine their generalizability.

  16. Health and economic benefits of reducing sugar intake in the USA, including effects via non-alcoholic fatty liver disease: a microsimulation model

    Science.gov (United States)

    Vreman, Rick A; Goodell, Alex J; Rodriguez, Luis A; Porco, Travis C; Lustig, Robert H; Kahn, James G

    2017-01-01

    Objectives Excessive consumption of added sugars in the human diet has been associated with obesity, type 2 diabetes (T2D), coronary heart disease (CHD) and other elements of the metabolic syndrome. Recent studies have shown that non-alcoholic fatty liver disease (NAFLD) is a critical pathway to metabolic syndrome. This model assesses the health and economic benefits of interventions aimed at reducing intake of added sugars. Methods Using data from US National Health Surveys and current literature, we simulated an open cohort, for the period 2015–2035. We constructed a microsimulation model with Markov chains for NAFLD (including steatosis, non-alcoholic steatohepatitis (NASH), cirrhosis and hepatocellular carcinoma (HCC)), body mass index, T2D and CHD. We assessed reductions in population disease prevalence, disease-attributable disability-adjusted life years (DALYs) and costs, with interventions that reduce added sugars consumption by either 20% or 50%. Findings The model estimated that a 20% reduction in added sugars intake will reduce prevalence of hepatic steatosis, NASH, cirrhosis, HCC, obesity, T2D and CHD. Incidence of T2D and CHD would be expected to decrease by 19.9 (95% CI 12.8 to 27.0) and 9.4 (95% CI 3.1 to 15.8) cases per 100 000 people after 20 years, respectively. A 20% reduction in consumption is also projected to annually avert 0.767 million (M) DALYs (95% CI 0.757M to 0.777M) and a total of US$10.3 billion (B) (95% CI 10.2B to 10.4B) in discounted direct medical costs by 2035. These effects increased proportionally when added sugars intake were reduced by 50%. Conclusions The decrease in incidence and prevalence of disease is similar to results in other models, but averted costs and DALYs were higher, mainly due to inclusion of NAFLD and CHD. The model suggests that efforts to reduce consumption of added sugars may result in significant public health and economic benefits. PMID:28775179

  17. Studies on Molecular Mechanisms Underlying Spinocerebellar Ataxia Type 3

    DEFF Research Database (Denmark)

    Kristensen, Line Vildbrad

    . Even though a range of mechanisms contributing to polyQ diseases have been uncovered, there is still no treatment available. One of the more common polyQ diseases is SCA3, which is caused by a polyQ expansion in the ataxin-3 protein that normally functions as a deubiquitinating enzyme involved...... in protein quality control. In SCA3 patients polyQ expanded ataxin-3 forms intranuclear inclusions in various brain areas, but why the polyQ expansion of ataxin-3 leads to neuronal dysfunction is still not well understood. This thesis describes molecular biological investigations of ataxin-3 biology, aimed...... at furthering our understanding of SCA3 disease mechanisms. In manuscript I, we investigated if post-translational modifications of ataxin-3 were changed by the polyQ expansion. The ubiquitin chain topology and ubiquitination pattern of ataxin-3 were unaltered by the polyQ expansion. In contrast...

  18. Genotyping and prenatal diagnosis of a large spinocerebellar ataxia ...

    Indian Academy of Sciences (India)

    2011-08-19

    Aug 19, 2011 ... range of eye movements with horizontal nystagmus, dysdi- adochokinesia, and ... individuals; open symbols indicate normal individuals; grey sym- bols indicate .... prenatal diagnosis of a fetus at risk of MJD. In conclusion, we ...

  19. Multistate matrix population model to assess the contributions and impacts on population abundance of domestic cats in urban areas including owned cats, unowned cats, and cats in shelters.

    Science.gov (United States)

    Flockhart, D T Tyler; Coe, Jason B

    2018-01-01

    Concerns over cat homelessness, over-taxed animal shelters, public health risks, and environmental impacts has raised attention on urban-cat populations. To truly understand cat population dynamics, the collective population of owned cats, unowned cats, and cats in the shelter system must be considered simultaneously because each subpopulation contributes differently to the overall population of cats in a community (e.g., differences in neuter rates, differences in impacts on wildlife) and cats move among categories through human interventions (e.g., adoption, abandonment). To assess this complex socio-ecological system, we developed a multistate matrix model of cats in urban areas that include owned cats, unowned cats (free-roaming and feral), and cats that move through the shelter system. Our model requires three inputs-location, number of human dwellings, and urban area-to provide testable predictions of cat abundance for any city in North America. Model-predicted population size of unowned cats in seven Canadian cities were not significantly different than published estimates (p = 0.23). Model-predicted proportions of sterile feral cats did not match observed sterile cat proportions for six USA cities (p = 0.001). Using a case study from Guelph, Ontario, Canada, we compared model-predicted to empirical estimates of cat abundance in each subpopulation and used perturbation analysis to calculate relative sensitivity of vital rates to cat abundance to demonstrate how management or mismanagement in one portion of the population could have repercussions across all portions of the network. Our study provides a general framework to consider cat population abundance in urban areas and, with refinement that includes city-specific parameter estimates and modeling, could provide a better understanding of population dynamics of cats in our communities.

  20. Multistate matrix population model to assess the contributions and impacts on population abundance of domestic cats in urban areas including owned cats, unowned cats, and cats in shelters

    Science.gov (United States)

    Coe, Jason B.

    2018-01-01

    Concerns over cat homelessness, over-taxed animal shelters, public health risks, and environmental impacts has raised attention on urban-cat populations. To truly understand cat population dynamics, the collective population of owned cats, unowned cats, and cats in the shelter system must be considered simultaneously because each subpopulation contributes differently to the overall population of cats in a community (e.g., differences in neuter rates, differences in impacts on wildlife) and cats move among categories through human interventions (e.g., adoption, abandonment). To assess this complex socio-ecological system, we developed a multistate matrix model of cats in urban areas that include owned cats, unowned cats (free-roaming and feral), and cats that move through the shelter system. Our model requires three inputs—location, number of human dwellings, and urban area—to provide testable predictions of cat abundance for any city in North America. Model-predicted population size of unowned cats in seven Canadian cities were not significantly different than published estimates (p = 0.23). Model-predicted proportions of sterile feral cats did not match observed sterile cat proportions for six USA cities (p = 0.001). Using a case study from Guelph, Ontario, Canada, we compared model-predicted to empirical estimates of cat abundance in each subpopulation and used perturbation analysis to calculate relative sensitivity of vital rates to cat abundance to demonstrate how management or mismanagement in one portion of the population could have repercussions across all portions of the network. Our study provides a general framework to consider cat population abundance in urban areas and, with refinement that includes city-specific parameter estimates and modeling, could provide a better understanding of population dynamics of cats in our communities. PMID:29489854