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  1. Earliest functional declines in Huntington disease

    Science.gov (United States)

    Beglinger, Leigh J.; O'Rourke, Justin J.F.; Wang, Chiachi; Langbehn, Douglas R.; Duff, Kevin; Paulsen, Jane S.

    2013-01-01

    We examined the gold standard for Huntington disease (HD) functional assessment, the Unified Huntington's Disease Rating Scale (UHDRS), in a group of at-risk participants not yet diagnosed but who later phenoconverted to manifest HD. We also sought to determine which skill domains first weaken and the clinical correlates of declines. Using the UHDRS Total Functional Capacity (TFC) and Functional Assessment Scale (FAS), we examined participants from Huntington Study Group clinics who were not diagnosed at their baseline visit but were diagnosed at a later visit (N = 265). Occupational decline was the most common with 65.1% (TFC) and 55.6% (FAS) reporting some loss of ability to engage in their typical work. Inability to manage finances independently (TFC 49.2%, FAS 35.1%) and drive safely (FAS 33.5%) were also found. Functional decline was significantly predicted by motor, cognitive, and depressive symptoms. The UHDRS captured early functional losses in individuals with HD prior to formal diagnosis, however, fruitful areas for expanded assessment of early functional changes are performance at work, ability to manage finances, and driving. These are also important areas for clinical monitoring and treatment planning as up to 65% experienced loss in at least one area prior to diagnosis. PMID:20471695

  2. The role of tau in the pathological process and clinical expression of Huntington's disease

    DEFF Research Database (Denmark)

    Vuono, Romina; Winder-Rhodes, Sophie; de Silva, Rohan

    2015-01-01

    and progression of Huntington's disease, the exact molecular mechanisms driving its pathogenic cascade and clinical features, especially the dementia, are not fully understood. Recently the microtubule associated protein tau, MAPT, which is associated with several neurodegenerative disorders, has been implicated......-mortem brain samples from patients with Huntington's disease (n = 16) compared to cases with a known tauopathy and healthy controls. Next, we undertook a genotype-phenotype analysis of a large cohort of patients with Huntington's disease (n = 960) with a particular focus on cognitive decline. We report...... not only on the tau pathology in the Huntington's disease brain but also the association between genetic variation in tau gene and the clinical expression and progression of the disease. We found extensive pathological inclusions containing abnormally phosphorylated tau protein that co-localized in some...

  3. Huntington\\'s disease: Genetic heterogeneity in black African patients

    African Journals Online (AJOL)

    Objective. Huntington's disease (HD) has been reported to occur rarely in black patients. A new genetic variant– Huntington's disease-like 2 (HDL2) – occurring more frequently in blacks, has recently been described. The absence of an expanded trinucleotide repeat at the chromosome 4 HD locus was previously regarded ...

  4. Huntington's disease presenting as amyotrophic lateral sclerosis.

    LENUS (Irish Health Repository)

    Phukan, Julie

    2010-08-01

    We present the clinical, electrophysiological and molecular genetic findings of a 58-year-old male with genetically confirmed Huntington\\'s disease (HD) and concurrent clinically definite ALS by El Escorial criteria. The patient presented with asymmetric upper limb amyotrophy and weakness, and subsequently developed chorea and cognitive change. Genetic testing confirmed the presence of expanded trinucleotide repeats in huntingtin, consistent with a diagnosis of Huntington\\'s disease. This case confirms the rare coexistence of Huntington\\'s disease and motor neuron degeneration.

  5. Perceptions of genetic discrimination among people at risk for Huntington?s disease: a cross sectional survey

    OpenAIRE

    Bombard, Yvonne; Veenstra, Gerry; Friedman, Jan M; Creighton, Susan; Currie, Lauren; Paulsen, Jane S; Bottorff, Joan L; Hayden, Michael R

    2009-01-01

    Objective To assess the nature and prevalence of genetic discrimination experienced by people at risk for Huntington?s disease who had undergone genetic testing or remained untested. Design Cross sectional, self reported survey. Setting Seven genetics and movement disorders clinics servicing rural and urban communities in Canada. Participants 233 genetically tested and untested asymptomatic people at risk for Huntington?s disease (response rate 80%): 167 underwent testing (83 had the Huntingt...

  6. Exit and Voice: Organizational Loyalty and Dispute Resolution Strategies

    Science.gov (United States)

    Hoffmann, Elizabeth A.

    2006-01-01

    This study compares workplace dispute resolution strategies (exit, voice and toleration) in matched pairs of conventional and worker-owned cooperative organizations operating in three industries--coal mining, taxicab driving and organic food distribution. Building on Hirschman's classic exit, voice and loyalty thesis, this research demonstrates…

  7. Psychopathology in Huntington's disease

    NARCIS (Netherlands)

    Duijn, Erik van

    2010-01-01

    Dit proefschrift begint met een overzichtsartikel van oorspronkelijke onderzoek naar psychopathologie bij mutatiedragers voor de ziekte van Huntington. Aansluitend worden de resultaten van een cohortstudie naar de aanwezigheid en ernst van psychopathologie bij mensen met de ziekte van Huntington in

  8. Drivers' Visual Characteristics when Merging onto or Exiting an Urban Expressway.

    Directory of Open Access Journals (Sweden)

    Ying Cheng

    Full Text Available The aim of this study is to examine drivers' visual and driving behavior while merging onto or exiting an urban expressway with low and high traffic densities. The analysis was conducted according to three periods (approaching, merging or exiting, and accelerating or decelerating. A total of 10 subjects (8 males and 2 females with ages ranging from 25 to 52 years old (M = 30.0 years old participated in the study. The research was conducted in a natural driving situation, and the drivers' eye movements were monitored and recorded using an eye tracking system. The results show that the influence of traffic density on the glance duration and scan duration is more significant when merging than when exiting. The results also demonstrate that the number of glances and the mean glance duration are mainly related to the driving task (e.g., the merging period. Therefore, drivers' visual search strategies mainly depend on the current driving task. With regard to driving behavior, the variation tendencies of the duration and the velocity of each period are similar. These results support building an automated driving assistant system that can automatically identify gaps and accelerate or decelerate the car accordingly or provide suggestions to the driver to do so.

  9. VT Data - Zoning 20120709, Huntington

    Data.gov (United States)

    Vermont Center for Geographic Information — Zoning district data for the Town of Huntington, Vermont. For details regarding each zoning district refer to the current zoning regulations on town of Huntington's...

  10. High-order harmonics measured by the photon statistics of the infrared driving-field exiting the atomic medium.

    Science.gov (United States)

    Tsatrafyllis, N; Kominis, I K; Gonoskov, I A; Tzallas, P

    2017-04-27

    High-order harmonics in the extreme-ultraviolet spectral range, resulting from the strong-field laser-atom interaction, have been used in a broad range of fascinating applications in all states of matter. In the majority of these studies the harmonic generation process is described using semi-classical theories which treat the electromagnetic field of the driving laser pulse classically without taking into account its quantum nature. In addition, for the measurement of the generated harmonics, all the experiments require diagnostics in the extreme-ultraviolet spectral region. Here by treating the driving laser field quantum mechanically we reveal the quantum-optical nature of the high-order harmonic generation process by measuring the photon number distribution of the infrared light exiting the harmonic generation medium. It is found that the high-order harmonics are imprinted in the photon number distribution of the infrared light and can be recorded without the need of a spectrometer in the extreme-ultraviolet.

  11. Clinical presentation of juvenile Huntington disease

    Directory of Open Access Journals (Sweden)

    Ruocco Heloísa H.

    2006-01-01

    Full Text Available OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questioner. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS: We identified 4 patients with juvenile onset of disease among 50 patients with Huntington disease followed prospectively in our Neurogenetics clinic. Age at onset varied from 3 to 13 years, there were 2 boys, and 3 patients had a paternal inheritance of the disease. Expanded Huntington disease allele sizes varied from 41 to 69 trinucleotide repeats. The early onset patients presented with rigidity, bradykinesia, dystonia, dysarthria, seizures and ataxia. MRI showed severe volume loss of caudate and putamen nuclei (p=0.001 and reduced cerebral and cerebellum volumes (p=0.01. CONCLUSION: 8% of Huntington disease patients seen in our clinic had juvenile onset of the disease. They did not present with typical chorea as seen in adult onset Huntington disease. There was a predominance of rigidity and bradykinesia. Two other important clinical features were seizures and ataxia, which related with the imaging findings of early cortical atrophy and cerebellum volume loss.

  12. Huntington's disease: a perplexing neurological disease ...

    African Journals Online (AJOL)

    Huntington's disease is an inherited intricate brain illness. It is a neurodegenerative, insidious disorder; the onset of the disease is very late to diagnose. It is caused by an expanded CAG repeat in the Huntingtin gene, which encodes an abnormally long polyglutamine repeat in the Huntingtin protein. Huntington's disease ...

  13. Huntington's disease : Psychological aspects of predictive testing

    NARCIS (Netherlands)

    Timman, Reinier

    2005-01-01

    Predictive testing for Huntington's disease appears to have long lasting psychological effects. The predictive test for Huntington's disease (HD), a hereditary disease of the nervous system, was introduced in the Netherlands in the late eighties. As adverse consequences of the test were

  14. Identification of genetic variants associated with Huntington's disease progression

    DEFF Research Database (Denmark)

    Hensman Moss, Davina J; Pardiñas, Antonio F; Langbehn, Douglas

    2017-01-01

    indivduals in the TRACK-HD cohort of Huntington's disease gene mutation carriers (data collected 2008-11). We generated a parallel progression score using data from 1773 previously genotyped participants from the European Huntington's Disease Network REGISTRY study of Huntington's disease mutation carriers...... in TRACK-HD participants, justifying use of a single, cross-domain measure of disease progression in both studies. The TRACK-HD and REGISTRY progression measures were correlated with each other (r=0·674), and with age at onset (TRACK-HD, r=0·315; REGISTRY, r=0·234). The meta-analysis of progression......BACKGROUND: Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate...

  15. Huntington's disease

    DEFF Research Database (Denmark)

    Hjermind, Lena Elisabeth; Law, Ian; Jønch, Aia

    2011-01-01

    In this open-label pilot study, the authors evaluated the effect of memantine on the distribution of brain glucose metabolism in four Huntington's disease (HD) patients as determined by serial 18-fluoro-deoxyglucose [F(18)]FDG-PET scans over a period of 3-4 months (90-129 days, with one patient...

  16. Progressive Impairment of Lactate-based Gluconeogenesis in the Huntington?s Disease Mouse Model R6/2

    OpenAIRE

    Nielsen, Signe Marie Borch; Hasholt, Lis; N?rrem?lle, Anne; Josefsen, Knud

    2015-01-01

    Huntington?s disease (HD) is a neurodegenerative illness, where selective neuronal loss in the brain caused by expression of mutant huntingtin protein leads to motor dysfunction and cognitive decline in addition to peripheral metabolic changes. In this study we confirm our previous observation of impairment of lactate-based hepatic gluconeogenesis in the transgenic HD mouse model R6/2 and determine that the defect manifests very early and progresses in severity with disease development, indic...

  17. Learning about Huntington's Disease

    Science.gov (United States)

    Skip to main content Learning About Huntington's Disease Enter Search Term(s): Español Research Funding An Overview Bioinformatics Current Grants Education and Training Funding Extramural Research ...

  18. Factors influencing the stay-exit intention of small livestock farmers

    NARCIS (Netherlands)

    Carter-Leal, Luis M.; Oude-Lansink, Alfons; Saatkamp, Helmut

    2018-01-01

    This study analyses the factors driving the stay-exit intention of small livestock farmers located in southern Chile. Technical, economic, and social characteristics from 212 farmers were included in this study. Through an empirical probit model we identified the variables that should be considered

  19. Changes in mental state and behaviour in Huntington's disease.

    Science.gov (United States)

    Eddy, Clare M; Parkinson, Ellice G; Rickards, Hugh E

    2016-11-01

    Changes in mental state and behaviour have been acknowledged in Huntington's disease since the original monograph in 1872 provided evidence of disinhibition and impaired social cognition. Behavioural problems can manifest before obvious motor symptoms and are frequently the most disabling part of the illness. Although pharmacological treatments are used routinely for psychiatric difficulties in Huntington's disease, the scientific evidence base for their use is somewhat sparse. Moreover, effective treatments for apathy and cognitive decline do not currently exist. Understanding the social cognitive impairments associated with Huntington's disease can assist management, but related therapeutic interventions are needed. Future research should aim to design rating scales for behaviour and mental state in Huntington's disease that can detect change in clinical trials. Generally, communication and understanding of behaviour and mental state in Huntington's would be enhanced by a clear conceptual framework that unifies ideas around movement, cognition, emotion, behaviour, and mental state, reflecting both the experience of the patient and their underlying neuropathology. Copyright © 2016 Elsevier Ltd. All rights reserved.

  20. A Metabolic Study of Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Rajasree Nambron

    Full Text Available Huntington's disease patients have a number of peripheral manifestations suggestive of metabolic and endocrine abnormalities. We, therefore, investigated a number of metabolic factors in a 24-hour study of Huntington's disease gene carriers (premanifest and moderate stage II/III and controls.Control (n = 15, premanifest (n = 14 and stage II/III (n = 13 participants were studied with blood sampling over a 24-hour period. A battery of clinical tests including neurological rating and function scales were performed. Visceral and subcutaneous adipose distribution was measured using magnetic resonance imaging. We quantified fasting baseline concentrations of glucose, insulin, cholesterol, triglycerides, lipoprotein (a, fatty acids, amino acids, lactate and osteokines. Leptin and ghrelin were quantified in fasting samples and after a standardised meal. We assessed glucose, insulin, growth hormone and cortisol concentrations during a prolonged oral glucose tolerance test.We found no highly significant differences in carbohydrate, protein or lipid metabolism markers between healthy controls, premanifest and stage II/III Huntington's disease subjects. For some markers (osteoprotegerin, tyrosine, lysine, phenylalanine and arginine there is a suggestion (p values between 0.02 and 0.05 that levels are higher in patients with premanifest HD, but not moderate HD. However, given the large number of statistical tests performed interpretation of these findings must be cautious.Contrary to previous studies that showed altered levels of metabolic markers in patients with Huntington's disease, our study did not demonstrate convincing evidence of abnormalities in any of the markers examined. Our analyses were restricted to Huntington's disease patients not taking neuroleptics, anti-depressants or other medication affecting metabolic pathways. Even with the modest sample sizes studied, the lack of highly significant results, despite many being tested, suggests that

  1. O desenvolvimento político em Huntington e Fukuyama Huntington and Fukuyama on political development

    Directory of Open Access Journals (Sweden)

    Natália Nóbrega de Mello

    2010-01-01

    Full Text Available O artigo contrasta as teses de Huntington e Fukuyama sobre desenvolvimento político. As obras analisadas, Ordem política nas sociedades em mudança e O fim da história, inscrevem-se entre duas conjunturas decisivas - 1968 e 1989. Huntington desmontou a equivalência entre desenvolvimento político e modernização e Fukuyama reafirmou a democracia como o destino de todos os países e, desse modo, como o fim da história. Nesta comparação, dois eixos se sobressaem: o contexto de produção das obras e a alternância entre os polos teóricos da democracia e da estabilidade. Procura-se demonstrar como, apesar de reinserir a democracia no desenvolvimento político, a instabilidade continua a ser um foco privilegiado de análise no pensamento de Fukuyama.The article contrasts the theories of Huntington and Fukuyama on political development. The analyzed works, Political order in changing societies and The end of history, fall between two decisive historical moments - in 1968 and 1989. Huntington disassembled the equivalence between political development and modernization; Fukuyama reaffirmed democracy as the destiny of all countries and, as such, it is the end of history. In this comparison, two axes call our attention: the production context of these works and the alternation between the theoreticals poles of democracy and stability. The article shows how, although reenters democracy in the political development theory, instablility remains a prime focus of analysis in Fukuyama's thought.

  2. Contribution of Neuroepigenetics to Huntington's Disease.

    Science.gov (United States)

    Francelle, Laetitia; Lotz, Caroline; Outeiro, Tiago; Brouillet, Emmanuel; Merienne, Karine

    2017-01-01

    Unbalanced epigenetic regulation is thought to contribute to the progression of several neurodegenerative diseases, including Huntington's disease (HD), a genetic disorder considered as a paradigm of epigenetic dysregulation. In this review, we attempt to address open questions regarding the role of epigenetic changes in HD, in the light of recent advances in neuroepigenetics. We particularly discuss studies using genome-wide scale approaches that provide insights into the relationship between epigenetic regulations, gene expression and neuronal activity in normal and diseased neurons, including HD neurons. We propose that cell-type specific techniques and 3D-based methods will advance knowledge of epigenome in the context of brain region vulnerability in neurodegenerative diseases. A better understanding of the mechanisms underlying epigenetic changes and of their consequences in neurodegenerative diseases is required to design therapeutic strategies more effective than current strategies based on histone deacetylase (HDAC) inhibitors. Researches in HD may play a driving role in this process.

  3. Exit selection strategy in pedestrian evacuation simulation with multi-exits

    International Nuclear Information System (INIS)

    Yue Hao; Zhang Bin-Ya; Shao Chun-Fu; Xing Yan

    2014-01-01

    A mixed strategy of the exit selection in a pedestrian evacuation simulation with multi-exits is constructed by fusing the distance-based and time-based strategies through a cognitive coefficient, in order to reduce the evacuation imbalance caused by the asymmetry of exits or pedestrian layout, to find a critical density to distinguish whether the strategy of exit selection takes effect or not, and to analyze the exit selection results with different cognitive coefficients. The strategy of exit selection is embedded in the computation of the shortest estimated distance in a dynamic parameter model, in which the concept of a jam area layer and the procedure of step-by-step expending are introduced. Simulation results indicate the characteristics of evacuation time gradually varying against cognitive coefficient and the effectiveness of reducing evacuation imbalance caused by the asymmetry of pedestrian or exit layout. It is found that there is a critical density to distinguish whether a pedestrian jam occurs in the evacuation and whether an exit selection strategy is in effect. It is also shown that the strategy of exit selection has no effect on the evacuation process in the no-effect phase with a low density, and that evacuation time and exit selection are dependent on the cognitive coefficient and pedestrian initial density in the in-effect phase with a high density. (general)

  4. Mapping energy poverty in Huntington, West Virginia

    Science.gov (United States)

    Callicoat, Elizabeth Anne

    Energy poverty is a growing phenomenon culminating from the combination of low to mid household income, deteriorating housing structures and rising household energy costs. Energy prices are increasing for all households, but the burden is proportionally larger for those with low to mid income. These groups must sacrifice to afford energy, and are often unable or do not have the autonomy to make structural improvements, especially if they rent their home. Data on residential dwellings from the Cabell County Tax Assessor's Office was used within a geographic information system to map where energy poverty likely exists within the city limits of Huntington, WV. It was found that one fifth of Huntington households are at a high risk of energy poverty, primarily located across the northern section of the city and in the center, surrounding Marshall University, Downtown and Cabell Huntington Hospital.

  5. Disease stage, but not sex, predicts depression and psychological distress in Huntington's disease

    DEFF Research Database (Denmark)

    Dale, Maria; Maltby, John; Shimozaki, Steve

    2016-01-01

    OBJECTIVE: Depression and anxiety significantly affect morbidity in Huntington's disease. Mice. models of Huntington's disease have identified sex differences in mood-like behaviours that vary across disease lifespan, but this interaction has not previously been explored in humans with Huntington......'s disease. However, among certain medical populations, evidence of sex differences in mood across various disease stages has been found, reflecting trends among the general population that women tend to experience anxiety and depression 1.5 to 2 times more than men. The current study examined whether...... disease stage and sex, either separately or as an interaction term, predicted anxiety and depression in Huntington's disease. METHODS: A cross-sectional study of REGISTRY data involving 453 Huntington's disease participants from 12 European countries was undertaken using the Hospital Anxiety...

  6. Huntington disease: a case study of early onset presenting as depression.

    Science.gov (United States)

    Duesterhus, Pia; Schimmelmann, Benno Graf; Wittkugel, Oliver; Schulte-Markwort, Michael

    2004-10-01

    Huntington disease is a dominantly inherited, neurodegenerative disease characterized by choreiform movement disturbances and dementia, usually with adult onset. The rare juvenile-onset Huntington disease differs from the adult phenotype. A case presenting twice, at age 10 with all the signs of a major depression and age 14 with mutism and rigidity, is reported. Meanwhile, the father developed the adult variant of Huntington disease. The boy's diagnosis was confirmed by molecular genetic analysis and magnetic resonance imaging. It is important to be aware of hereditary conditions such as Huntington disease and to provide family counseling before genetic testing and after the diagnosis is confirmed.

  7. Factor analysis of the hospital anxiety and depression scale among a Huntington's disease population

    DEFF Research Database (Denmark)

    Dale, Maria; Maltby, John; Martucci, Rossana

    2015-01-01

    INTRODUCTION: Depression and anxiety are common in Huntington's disease, a genetic neurodegenerative disorder. There is a need for measurement tools of mood to be validated within a Huntington's disease population. The current study aimed to analyze the factor structure of the Hospital Anxiety...... and Depression Scale in Huntington's disease. METHODS: Data from the European Huntington's Disease Network study REGISTRY 3 were used to undertake a factor analysis of the scale among a sample of 492 Huntington's disease mutation carriers. The sample was randomly divided into two equal subsamples...... support for an eight-item version of the scale to be used as a measure of general distress within Huntington's disease populations. © 2015 International Parkinson and Movement Disorder Society....

  8. PSYCHIATRIC ASPECTS OF HUNTINGTON DISEASE – CASE REPORTS

    Directory of Open Access Journals (Sweden)

    Mirela Batta

    2004-04-01

    Full Text Available Background. Huntington disease occurrs rarely, it can be encountered not only by neurologists and psychiatrists but also by other medical practitioners. Its characteristic features are involuntary movements, cognitive disorders and gradual development of dementia. Diagnosis is given on the basis of these clinical features, positive familial anamnesis, with the laboratory exclusion of other neuropsychiatric diseases and with the help of neuroimaging methods (in particular NMR. The disease can be only confirmed by means of genetic analysis.Patients and methods. In this article, four cases of patients with Huntington disease and diverse psychiatric disorders that were hospitalised at the psychiatric department of the Maribor General Hospital between October 2002 and March 2003 are described. All the patients fulfilled the valid criteria for the diagnosis of Huntington disease. However, they differed according to their accompanying psychiatric psychopathology, age and social problems.Conclusions. The purpose of this article is to draw attention to different psychiatric symptoms and clinical manifestations of Huntington disease that are often misleading in the diagnostic process. In addition, exigency of early diagnostics, guidelines for referrals to genetic testing and psychiatric monitoring of these patients are emphasised.

  9. Semantic, phonologic, and verb fluency in Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mariana Jardim Azambuja

    Full Text Available Abstract Verbal fluency tasks have been identified as important indicators of executive functioning impairment in patients with frontal lobe dysfunction. Although the usual evaluation of this ability considers phonologic and semantic criteria, there is some evidence that fluency of verbs would be more sensitive in disclosing frontostriatal physiopathology since frontal regions primarily mediate retrieval of verbs. Huntington's disease usually affects these circuitries. Objective: To compare three types of verbal fluency task in the assessment of frontal-striatal dysfunction in HD subjects. Methods: We studied 26 Huntington's disease subjects, divided into two subgroups: mild (11 and moderate (15 along with 26 normal volunteers matched for age, gender and schooling, for three types of verbal fluency: phonologic fluency (F-A-S, semantic fluency and fluency of verbs. Results: Huntington's disease subjects showed a significant reduction in the number of words correctly generated in the three tasks when compared to the normal group. Both controls and Huntington's disease subjects showed a similar pattern of decreasing task performance with the greatest number of words being generated by semantic elicitation followed by verbs and lastly phonologic criteria. We did not find greater production of verbs compared with F-A-S and semantic conditions. Moreover, the fluency of verbs distinguished only the moderate group from controls. Conclusion: Our results indicated that phonologic and semantic fluency can be used to evaluate executive functioning, proving more sensitive than verb fluency. However, it is important to point out that the diverse presentations of Huntington's disease means that an extended sample is necessary for more consistent analysis of this issue.

  10. Major Superficial White Matter Abnormalities in Huntington's Disease

    Science.gov (United States)

    Phillips, Owen R.; Joshi, Shantanu H.; Squitieri, Ferdinando; Sanchez-Castaneda, Cristina; Narr, Katherine; Shattuck, David W.; Caltagirone, Carlo; Sabatini, Umberto; Di Paola, Margherita

    2016-01-01

    Background: The late myelinating superficial white matter at the juncture of the cortical gray and white matter comprising the intracortical myelin and short-range association fibers has not received attention in Huntington's disease. It is an area of the brain that is late myelinating and is sensitive to both normal aging and neurodegenerative disease effects. Therefore, it may be sensitive to Huntington's disease processes. Methods: Structural MRI data from 25 Pre-symptomatic subjects, 24 Huntington's disease patients and 49 healthy controls was run through a cortical pattern-matching program. The surface corresponding to the white matter directly below the cortical gray matter was then extracted. Individual subject's Diffusion Tensor Imaging (DTI) data was aligned to their structural MRI data. Diffusivity values along the white matter surface were then sampled at each vertex point. DTI measures with high spatial resolution across the superficial white matter surface were then analyzed with the General Linear Model to test for the effects of disease. Results: There was an overall increase in the axial and radial diffusivity across much of the superficial white matter (p < 0.001) in Pre-symptomatic subjects compared to controls. In Huntington's disease patients increased diffusivity covered essentially the whole brain (p < 0.001). Changes are correlated with genotype (CAG repeat number) and disease burden (p < 0.001). Conclusions: This study showed broad abnormalities in superficial white matter even before symptoms are present in Huntington's disease. Since, the superficial white matter has a unique microstructure and function these abnormalities suggest it plays an important role in the disease. PMID:27242403

  11. Did the "Woman in the Attic" in Jane Eyre Have Huntington Disease?

    Science.gov (United States)

    Coon, Elizabeth A; Hassan, Anhar

    2015-01-01

    References to neurologic disorders are frequently found in fictional literature and may precede description in the medical literature. Our aim was to compare Charlotte Brontë's depiction of Bertha Mason in Jane Eyre to the tenets set forth in George Huntington's original essay "On chorea" with the hypothesis that Mason was displaying features of Huntington disease. Charlotte Brontë's 1847 Victorian novel Jane Eyre features the character Bertha Mason, who is portrayed with a progressive psychiatric illness, violent movements, and possible cognitive decline. Similar to Huntington's tenets, Mason has a disorder with a strong family history suggestive of autosomal dominant inheritance with onset in adulthood, and culminating in suicide. Brontë's character had features of Huntington disease as originally described by Huntington. Brontë's keen characterization may have increased awareness of treatment of neuropsychiatric patients in the Victorian era.

  12. Discrepancies in reporting the CAG repeat lengths for Huntington's disease

    DEFF Research Database (Denmark)

    Quarrell, Oliver W; Handley, Olivia; O'Donovan, Kirsty

    2011-01-01

    Huntington's disease results from a CAG repeat expansion within the Huntingtin gene; this is measured routinely in diagnostic laboratories. The European Huntington's Disease Network REGISTRY project centrally measures CAG repeat lengths on fresh samples; these were compared with the original...

  13. Exit from contract

    Directory of Open Access Journals (Sweden)

    Oren Bar-Gill

    2016-01-01

    Full Text Available Objective to study the procedure of exiting the contract its costs and benefits. Methods statistical method comparative analysis. Results free exit from contract is one of the most powerful tools for the consumer rights protection. The procedure frees consumers from bad deals and keeps businesses honest. Yet consumers often choose transactions with lockin provisions trading off exit rights for other perks. This article examines the costs and benefits of free exit as compared to the lockin alternative. According to the authors the present regulation of exit penalties in the USA is poorly tailored to address concerns about lockin particularly in light of increasingly ubiquitous marketbased solutions. The article also calls regulatory attention to loyalty rewards which are shown to be as powerful as exit penalties and equally detrimental. Scientific novelty the article reveals a paradoxical state of the law exit regulations in the USA are used most where they are needed least. Termination penalties present an obvishyous target for regulatory intervention while loyalty programs seem benign not warranting any regulatory attention. Practical significance the article is of interest for the Russian juridical science and lawmaking authorities as in Russia the issue of exiting the contract is as topical as in the USA and requires solution which would impair neither the rights of consumers nor the rights of the sellers ofnbspproducts and services. nbsp

  14. Exit-strategies

    DEFF Research Database (Denmark)

    Mørck, Line Lerche; Palm, Anne-Mette; Sys Møller-Andersen, Camilla

    different empirical sources. To develop and extend an "exit-prototype" about conditions of importance for moving beyond a gang/criminal position, we have analyzed documents (from newspapers and books), involved ex-gang members, social workers and experts as co-researchers in interviews, "gang......This paper is about exit-strategies, constructing a theoretical and empirical informed analysis of current societal conditions that influence motor cycle gangs such as Hells Angels or Bandidos and other ‘wild' youth' possibilities and limitations for moving beyond criminal activities. We especially...... focus on the involved communities in the current Danish gang-conflict, which escalated with deadly killings in 2008, and thereby became a so called "gang-war". We will start out presenting different practice notions of exit, and we will extend and discuss understandings of "exit-strategies" by analyzing...

  15. aliving with Huntington´s disease in the Czech Republic

    Czech Academy of Sciences Publication Activity Database

    Baxa, Monika

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 6-6 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] Institutional support: RVO:67985904 Keywords : Czech Huntington Association * life with Huntington ´ disease Subject RIV: FH - Neurology

  16. Altered brain mechanisms of emotion processing in pre-manifest Huntington's disease.

    Science.gov (United States)

    Novak, Marianne J U; Warren, Jason D; Henley, Susie M D; Draganski, Bogdan; Frackowiak, Richard S; Tabrizi, Sarah J

    2012-04-01

    Huntington's disease is an inherited neurodegenerative disease that causes motor, cognitive and psychiatric impairment, including an early decline in ability to recognize emotional states in others. The pathophysiology underlying the earliest manifestations of the disease is not fully understood; the objective of our study was to clarify this. We used functional magnetic resonance imaging to investigate changes in brain mechanisms of emotion recognition in pre-manifest carriers of the abnormal Huntington's disease gene (subjects with pre-manifest Huntington's disease): 16 subjects with pre-manifest Huntington's disease and 14 control subjects underwent 1.5 tesla magnetic resonance scanning while viewing pictures of facial expressions from the Ekman and Friesen series. Disgust, anger and happiness were chosen as emotions of interest. Disgust is the emotion in which recognition deficits have most commonly been detected in Huntington's disease; anger is the emotion in which impaired recognition was detected in the largest behavioural study of emotion recognition in pre-manifest Huntington's disease to date; and happiness is a positive emotion to contrast with disgust and anger. Ekman facial expressions were also used to quantify emotion recognition accuracy outside the scanner and structural magnetic resonance imaging with voxel-based morphometry was used to assess the relationship between emotion recognition accuracy and regional grey matter volume. Emotion processing in pre-manifest Huntington's disease was associated with reduced neural activity for all three emotions in partially separable functional networks. Furthermore, the Huntington's disease-associated modulation of disgust and happiness processing was negatively correlated with genetic markers of pre-manifest disease progression in distributed, largely extrastriatal networks. The modulated disgust network included insulae, cingulate cortices, pre- and postcentral gyri, precunei, cunei, bilateral putamena

  17. Psychiatric symptoms and CAG expansion in Huntington`s disease

    Energy Technology Data Exchange (ETDEWEB)

    Weber, M.W.; Schmid, W.; Spiegel, R. [Univ. of Zuerich (Switzerland)

    1996-02-16

    The mutation responsible for Huntington`s disease (HD) is an elongated CAG repeat in the coding region of the IT15 gene. A PCR-based test with high sensitivity and accuracy is now available to identify asymptomatic gene carriers and patients. An inverse correlation between CAG copy number and age at disease onset has been found in a large number of affected individuals. The influence of the CAG repeat expansion on other phenotypic manifestations, especially specific psychiatric symptoms has not been studied intensively. In order to elucidate this situation we investigated the relation between CAG copy number and distinct psychiatric phenotypes found in 79 HD-patients. None of the four differentiated categories (personality change, psychosis, depression, and nonspecific alterations) showed significant differences in respect to size of the CAG expansion. In addition, no influence of individual sex on psychiatric presentation could be found. On the other hand in patients with personality changes maternal transmission was significantly more frequent compared with all other groups. Therefore we suggest that clinical severity of psychiatric features in HD is not directly dependent on the size of the dynamic mutation involved. The complex pathogenetic mechanisms leading to psychiatric alterations are still unknown and thus genotyping does not provide information about expected psychiatric symptoms in HD gene carriers. 40 refs., 1 fig., 2 tabs.

  18. Clinical and genetic data of Huntington disease in Moroccan patients

    African Journals Online (AJOL)

    Background: Huntington's disease (HD) occurs worldwide with prevalence varying from 0.1 to 10 /100,000 depending of the ethnic origin. Since no data is available in the Maghreb population, the aim of this study is to describe clinical and genetic characteristics of Huntington patients of Moroccan origin. Methods: Clinical ...

  19. 75 FR 13454 - Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY

    Science.gov (United States)

    2010-03-22

    ...-AA08 Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY AGENCY: Coast... navigable waters of Huntington Bay, New York due to the annual Fran Schnarr Open Water Championships. The..., ``Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY'' (Docket number USCG...

  20. 75 FR 38710 - Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY

    Science.gov (United States)

    2010-07-06

    ...-AA08 Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY AGENCY: Coast... Regulation on the navigable waters of Huntington Bay, New York due to the annual Fran Schnarr Open Water... ``Special Local Regulation, Fran Schnarr Open Water Championships, Huntington Bay, NY'' in the Federal...

  1. Exit Prostitution

    DEFF Research Database (Denmark)

    Henriksen, Theresa Dyrvig; Aslaug Kjær, Agnete; Christensen, Gunvor

    2015-01-01

    Dette midtvejsnotat omhandler projektet ”Exit prostitution”. Exit-projektet blev påbegyndt i april 2012 og løber til udgangen af 2015 og befinder sig i øjeblikket midtvejs i projektets afprøvningsfase. I projektet anvendes metoden Critical Time Intervention (CTI), der er en evidensbaseret...... til det. Exit-projektet er dermed en central socialpolitisk indsats overfor borgere i prostitution i det danske samfund. I dette notat belyser vi midtvejsresultater for, hvordan udviklingen er for de borgere, der er nået halvt igennem et CTI-forløb. I den afsluttende evaluering af projektet i 2015 vil...

  2. Exit prostitution

    DEFF Research Database (Denmark)

    Mehlsen, Line; Aslaug Kjær, Agnete; Amilon, Anna

    2016-01-01

    Dette statusnotat for projektet ”Exit Prostitution” belyser de foreløbige resultater og tendenser for projektet. Exit Prostitution løb oprindeligt fra april 2012 til udgangen af 2015, men med en nylig forlængelse løber projektet til udgangen af 2016. Projektet befinder sig således i slutningen af...... afprøvet med succes i forhold til hjemløshed både nationalt og internationalt. Målet med anvendelsen af metoden i forhold til målgruppen for Exit Prostitution er, at borgere med prostitutionserfaring, som ønsker at ophøre med salg af seksuelle ydelser eller ønsker at opleve en forbedring af deres...

  3. The Addenbrooke's Cognitive Examination-Revised accurately detects cognitive decline in Huntington's disease.

    Science.gov (United States)

    Begeti, Faye; Tan, Adrian Y K; Cummins, Gemma A; Collins, Lucy M; Guzman, Natalie Valle; Mason, Sarah L; Barker, Roger A

    2013-11-01

    Cognitive features, which begin before manifestation of the motor features, are an integral part of Huntington's disease and profoundly affect quality of life. A number of neuropsychological batteries have been used to assess this aspect of the condition, many of which are difficult to administer and time consuming, especially in advanced disease. We, therefore, investigated a simple and practical way to monitor cognition using the Addenbrooke's Cognitive Examination-Revised (ACE-R) in 126 manifest Huntington's disease patients, 28 premanifest gene carriers and 21 controls. Using this test, we demonstrated a selective decrease in phonemic, but not semantic, fluency in premanifest participants Cognitive decline in manifest Huntington's disease varied according to disease severity with extensive cognitive decline observed in early-stage Huntington's disease patients, indicating that this would be an optimal stage for interventions designed to halt cognitive decline, and lesser changes in the advanced cases. We next examined cognitive performance in patients prescribed antidopaminergic drugs as these drugs are known to decrease cognition when administered to healthy volunteers. We paradoxically found that these drugs may be beneficial, as early-stage Huntington's disease participants in receipt of them had improved attention and Mini-Mental State Examination scores. In conclusion, this is the first study to test the usefulness of the ACE-R in a Huntington's disease population and demonstrates that this is a brief, inexpensive and practical way to measure global cognitive performance in clinical practice with potential use in clinical trials.

  4. Xeroderma pigmentosum is a definite cause of Huntington's disease-like syndrome.

    Science.gov (United States)

    Garcia-Moreno, Hector; Fassihi, Hiva; Sarkany, Robert P E; Phukan, Julie; Warner, Thomas; Lehmann, Alan R; Giunti, Paola

    2018-01-01

    Xeroderma pigmentosum is characterized by cutaneous, ophthalmological, and neurological features. Although it is typical of childhood, late presentations can mimic different neurodegenerative conditions. We report two families presenting as Huntington's disease-like syndromes. The first case (group G) presented with neuropsychiatric features, cognitive decline and chorea. Typical lentigines were only noticed after the neurological disease started. The second case (group B) presented adult-onset chorea and neuropsychiatric symptoms after an aggressive ocular melanoma. Xeroderma pigmentosum can manifest as a Huntington's Disease-like syndrome. Classic dermatological and oncological features have to be investigated in choreic patients with negative genetic tests for Huntington's disease-like phenotypes.

  5. Music therapy in Huntington's disease

    NARCIS (Netherlands)

    Bruggen-Rufi, van C.H.M.

    2018-01-01

    The thesis reports about the effects of music therapy with patients in the late stage of Huntington's disease. A literature review, a focus group study, a randomized controlled trial, an evaluation for complex interventions and a case report study are integrated in the thesis. The beneficial

  6. The Wilson films--Huntington's chorea.

    Science.gov (United States)

    Klein, Christine

    2011-12-01

    Wilson's Queen Square Case 9 with Huntington's chorea shows a 68-year-old man with mild to moderate generalized chorea, impaired fixation, and probable cognitive decline in keeping with a diagnosis of Huntington's disease (HD). An age of onset in the late sixties and a negative family history suggest a relatively small expanded trinucleotide repeat in the HTT gene in the patient and reduced penetrance of an even shorter repeat allele in one of his parents. A highly sensitive and specific gene test has been offered worldwide for diagnostic testing of HD for almost two decades. This test, obviously unavailable at Wilson's times, became the historic frontrunner for guidelines of symptomatic, presymptomatic, and prenatal testing for an adult-onset neurodegenerative disorder. Regarding treatment of HD, however, we are still awaiting the successful translation of research results into the development of effective cause-directed, neuropreventive and neurorestaurative therapies. Copyright © 2011 Movement Disorder Society.

  7. Patterns of drinking and driving offenses among nightclub patrons in Brazil.

    Science.gov (United States)

    Wagner, Gabriela A; Sanchez, Zila M

    2017-05-01

    Brazil has a strict drinking and driving law known as the Brazilian "Dry Law". The aim of the present study was to investigate characteristics associated with the breaking of the Brazilian traffic law, on drinking and driving, at nightclub exit among a representative sample of nightclub patrons in the city of São Paulo, Brazil. Portal survey realized with a two-stage cluster sampling survey design to collect data from 2422 patrons at the entrance and 1822 patrons at the exit of 31 nightclubs in the city of São Paulo, Brazil. Patrons' breath alcohol concentrations (BrACs) at the entrance and exit of the nightclubs were categorized according to the law as either a "traffic offense" or a "traffic offense and crime". Weighted multinomial logistic regression was used to analyze factors associated with different patterns of drinking and driving offenses. Of the subjects, 16.5% (n=369) were identified as driving patrons at the entrance and exit of the nightclubs. At entry, 80.1% of the patrons had a zero BrAC, 14.9% had a BrAC meeting the traffic offense criteria and 5.0% had a BrAC meeting the traffic offense and crime criteria. Women were less likely to have BrACs meeting the traffic offense criteria. At nightclub exit, 63.4% of patrons had maintained a zero BrAC, 24.7% had a BrAC that had increased and now met the traffic offense and crime criteria, and 11.9% had a decreased or stable BrAC. An increased BrAC was more frequently identified in patrons who were men, were single, and had used illicit drugs inside the nightclub. Despite the existence of a strict law regarding drinking and driving, a significant proportion of nightclub patrons in the city of São Paulo had violated this law, suggesting a perception of impunity and need for law enforcement. Copyright © 2017 Elsevier B.V. All rights reserved.

  8. The use of olanzapine in Huntington disease accompanied by psychotic symptoms

    Directory of Open Access Journals (Sweden)

    Cafer Alhan

    2014-06-01

    Full Text Available Huntington's disease is an autosomal dominant neurodegenerative disease. The disease begins between the ages of 30-50, including motor symptoms, psychiatric symptoms and is characterized by progressive dementia. Common psychiatric disorders of Huntington’s disease include mood and anxiety disorders, behavior and personality changes. Psychosis is relatively rare. Here, a patient is present, who has Huntington’s disease, which is associated with psychotic symptoms. 61-year-old male patient who were followed for Huntington disease for 25 years was admitted for complaints of thinking of poisoning and refuse to eat something. Patient was started on olanzapine at dose of 5 mg/day. In follow up psychotic symptoms disappeared. Emerging psychotic symptoms in Huntington disease is created a need for antipsychotic treatment. Atypical antipsychotic agents should be preferred in the treatment and as in the case olanzapine may be used as a treatment option should be kept in mind to control both involuntary movements and psychotic symptoms in Huntington's disease with psychotic features. J Clin Exp Invest 2014; 5 (2: 326-328

  9. Kas Huntington oli prohvet? / Priit Simson

    Index Scriptorium Estoniae

    Simson Priit, 1977-

    2008-01-01

    Autor käsitleb Samuel Huntingtoni teese ning leiab, et tegelikult Huntington ei pakkunud õigustust islamiriikide ründamisele, vaid pigem hoiatas tsivilisatsioonide siseasjusse sekkumise, tekkida võiva ahelreaktsiooni eest, kus üks tsivilisatsiooni liige tõmbab sõtta ka teise

  10. The emotional experiences of family carers in Huntington disease.

    Science.gov (United States)

    Williams, Janet K; Skirton, Heather; Paulsen, Jane S; Tripp-Reimer, Toni; Jarmon, Lori; McGonigal Kenney, Meghan; Birrer, Emily; Hennig, Bonnie L; Honeyford, Joann

    2009-04-01

    This paper is a report of a study conducted to examine the emotional experience of caregiving by family carers of people with Huntington disease and to describe strategies they used to deal with that experience. Huntington disease, commonly diagnosed in young to middle adulthood, is an inherited single gene disorder involving loss of cognitive, motor and neuropsychiatric function. Many family members become caregivers as well as continuing as parents and wage earners. The emotional aspects of caregiving contribute to mental health risks for family members. Focus groups were conducted with 42 adult carers of people with Huntington disease in four United States and two Canadian Huntington disease centers between 2001 and 2005. Data were analyzed through descriptive coding and thematic analysis. All participants reported multiple aspects of emotional distress. Being a carer was described as experiencing disintegration of one's life. Carers attempted to cope by seeking comfort from selected family members, anticipating the time when the care recipient had died and/or using prescription medications. Spousal carers were distressed by the loss of their relationship with their spouse and dealt with this by no longer regarding the person as an intimate partner. Carers were concerned about the disease risk for children in their families and hoped for a cure. Emotional distress can compromise the well-being of family carers, who attempt to maintain multiple roles. Nurses should monitor carer mental health, identify sources of emotional distress and support effective strategies used by carers to mediate distress.

  11. Américo Negrette and Huntington's disease

    Directory of Open Access Journals (Sweden)

    Mariana Moscovich

    2011-08-01

    Full Text Available The authors present a historical review of the seminal clinical contribution of Professor Américo Negrette, a Venezuelan neurologist, to the evolution of scientific knowledge about Huntington's disease.

  12. Clinical and genetic study of a juvenile-onset Huntington disease

    Directory of Open Access Journals (Sweden)

    HAO Ying

    2012-06-01

    Full Text Available Background Huntington's disease (HD is an autosomal dominant hereditary progressive neurodegenerative disorder with a distinct phenotype characterized by chorea, dementia, cognitive and affective impairment. There are selective neural cell loss and atrophy in the caudate and putamen. Dr. George Huntington firstly described the disease accurately and insightfully, which led to a widespread recognition of the inherited chorea that now bears his name. Huntington disease gene (IT15 locus on chromosome 4p16.3, and encompasses 67 exons with a trinucleotide repeat (CAG in the first exon. The CAG repeat length is highly polymorphic in the population and expanded on at least one chromosome of individuals with HD. Clinically, patient with HD are often onset in adulthood. Juvenile-onset HD is relatively rare. Adult-onset HD patients usually have a CAG expansion from 40 to 55 whereas those with juvenile-onset greater than 60 which are often inherited from the father. We investigated the clinical features of a juvenile-onset case with Huntington disease and dynamic mutation of his family. Methods The CAG repeats of IT15 gene were detected using polymerase chain reaction and capillary electrophoresis in 115 individuals with preliminary diagnosis as Huntington disease. The repeat numbers of some samples carried expanded or intermediate alleles were verified by the pMD18-T vector clone sequencing. Results Fragment analysis showed that one juvenile-onset case presenting with cognitive dysfunction and hypokinesis carried 15/68 CAG repeats of IT15. His father carried 17/37 and mother carried 15/17. Conclusion 1 The juvenile-onset case of HD presented with different clinical features compared with adult-onset cases. The typical signs of adult-onset cases include progressive chorea, rigidity and dementia. The most common sign of juvenile-onset Huntington disease is cognitive decline. 2 The dynamic mutation of IT15 gene expansion of the CAG repeats in the

  13. On a mean field game optimal control approach modeling fast exit scenarios in human crowds

    KAUST Repository

    Burger, Martin; Di Francesco, Marco; Markowich, Peter A.; Wolfram, Marie Therese

    2013-01-01

    The understanding of fast exit and evacuation situations in crowd motion research has received a lot of scientific interest in the last decades. Security issues in larger facilities, like shopping malls, sports centers, or festivals necessitate a better understanding of the major driving forces in crowd dynamics. In this paper we present an optimal control approach modeling fast exit scenarios in pedestrian crowds. The model is formulated in the framework of mean field games and based on a parabolic optimal control problem. We consider the case of a large human crowd trying to exit a room as fast as possible. The motion of every pedestrian is determined by minimizing a cost functional, which depends on his/her position and velocity, the overall density of people, and the time to exit. This microscopic setup leads in a mean-field formulation to a nonlinear macroscopic optimal control problem, which raises challenging questions for the analysis and numerical simulations.We discuss different aspects of the mathematical modeling and illustrate them with various computational results. ©2013 IEEE.

  14. On a mean field game optimal control approach modeling fast exit scenarios in human crowds

    KAUST Repository

    Burger, Martin

    2013-12-01

    The understanding of fast exit and evacuation situations in crowd motion research has received a lot of scientific interest in the last decades. Security issues in larger facilities, like shopping malls, sports centers, or festivals necessitate a better understanding of the major driving forces in crowd dynamics. In this paper we present an optimal control approach modeling fast exit scenarios in pedestrian crowds. The model is formulated in the framework of mean field games and based on a parabolic optimal control problem. We consider the case of a large human crowd trying to exit a room as fast as possible. The motion of every pedestrian is determined by minimizing a cost functional, which depends on his/her position and velocity, the overall density of people, and the time to exit. This microscopic setup leads in a mean-field formulation to a nonlinear macroscopic optimal control problem, which raises challenging questions for the analysis and numerical simulations.We discuss different aspects of the mathematical modeling and illustrate them with various computational results. ©2013 IEEE.

  15. A 24-Hour Study of the Hypothalamo-Pituitary Axes in Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Eirini Kalliolia

    Full Text Available Huntington's disease is an inherited neurodegenerative disorder characterised by motor, cognitive and psychiatric disturbances. Patients exhibit other symptoms including sleep and mood disturbances, muscle atrophy and weight loss which may be linked to hypothalamic pathology and dysfunction of hypothalamo-pituitary axes.We studied neuroendocrine profiles of corticotropic, somatotropic and gonadotropic hypothalamo-pituitary axes hormones over a 24-hour period in controlled environment in 15 healthy controls, 14 premanifest and 13 stage II/III Huntington's disease subjects. We also quantified fasting levels of vasopressin, oestradiol, testosterone, dehydroepiandrosterone sulphate, thyroid stimulating hormone, free triiodothyronine, free total thyroxine, prolactin, adrenaline and noradrenaline. Somatotropic axis hormones, growth hormone releasing hormone, insulin-like growth factor-1 and insulin-like factor binding protein-3 were quantified at 06:00 (fasting, 15:00 and 23:00. A battery of clinical tests, including neurological rating and function scales were performed.24-hour concentrations of adrenocorticotropic hormone, cortisol, luteinizing hormone and follicle-stimulating hormone did not differ significantly between the Huntington's disease group and controls. Daytime growth hormone secretion was similar in control and Huntington's disease subjects. Stage II/III Huntington's disease subjects had lower concentration of post-sleep growth hormone pulse and higher insulin-like growth factor-1:growth hormone ratio which did not reach significance. In Huntington's disease subjects, baseline levels of hypothalamo-pituitary axis hormones measured did not significantly differ from those of healthy controls.The relatively small subject group means that the study may not detect subtle perturbations in hormone concentrations. A targeted study of the somatotropic axis in larger cohorts may be warranted. However, the lack of significant results despite many

  16. 29 CFR 1917.122 - Employee exits.

    Science.gov (United States)

    2010-07-01

    ... 29 Labor 7 2010-07-01 2010-07-01 false Employee exits. 1917.122 Section 1917.122 Labor Regulations...) MARINE TERMINALS Terminal Facilities § 1917.122 Employee exits. (a) Employee exits shall be clearly marked. (b) If an employee exit is not visible from employees' work stations, directional signs...

  17. The Huntington disease locus is most likely within 325 kilobases of the chromosome 4p telomere

    International Nuclear Information System (INIS)

    Doggett, N.A.; Cheng, J.F.; Smith, C.L.; Cantor, C.R.

    1989-01-01

    The genetic defect responsible for Huntington disease was originally localized near the tip of the short arm of chromosome 4 by genetic linkage to the locus D4S10. Several markers closer to Huntington disease have since been isolated, but these all appear to be proximal to the defect. A physical map that extends from the most distal of these loci, D4S90, to the telomere of chromosome 4 was constructed. This map identifies at least two CpG islands as markers for Huntington disease candidate genes and places the most likely location of the Huntington disease defect remarkably close (within 325 kilobases) to the telomere

  18. [The life as a caregiver of a person affected by Chorea Huntington: multiple case study].

    Science.gov (United States)

    Winkler, Evi; Ausserhofer, Dietmar; Mantovan, Franco

    2012-10-01

    Chorea Huntington is an autosomal dominantly inherited, neurodegenerative brain disorder that leads to involuntary hyperkinesia, psychotic symptoms and dementia. The illness not only changes the life of the person itself but also the world of the caregivers. The challenges in the care of a person which is affected by Chorea Huntington have an effect on the daily living as an assemblage of natural and social conditions. a multiple case study was conducted. It included semi-structured interviews with three caregivers of people with Chorea Huntington in South Tyrol. The qualitative data was analyzed using the qualitative structured analysis of Mayring (2007). The objective of this study was to describe the phenomenon of change of life from family members that care people affected by Chorea Huntington in a specific cultural setting (South Tyrol, Italy). The caregivers reported that the diagnosis of Chorea Huntington leads to negative changes in "relationship and family". Particularly, frustration, aggression, impatience and apathy were perceived as stressful. At the same time they highlight the positive changes through home care. They report that the relationship became more intimate and integral and it was characterized by more cohesion. Family caregivers get valuable support from the home care service, however, they complain that there is no facility in South Tyrol, which is specialized to care people with Chorea Huntington. Therefore, the caregivers have to "give up a lot" and don't have any personal desires, dreams and expectations for the future. The caregivers have learned independently to deal with their changed life step by step, and to see also the positive effects of the caring role. The life of family caregivers of a person which is affected by Chorea Huntington is characterized by abandonment. A continuous and professional care would be important for the affected and his caregiver. A continuous and professional care is important for both, addressing the

  19. An image-based model of brain volume biomarker changes in Huntington's disease.

    Science.gov (United States)

    Wijeratne, Peter A; Young, Alexandra L; Oxtoby, Neil P; Marinescu, Razvan V; Firth, Nicholas C; Johnson, Eileanoir B; Mohan, Amrita; Sampaio, Cristina; Scahill, Rachael I; Tabrizi, Sarah J; Alexander, Daniel C

    2018-05-01

    Determining the sequence in which Huntington's disease biomarkers become abnormal can provide important insights into the disease progression and a quantitative tool for patient stratification. Here, we construct and present a uniquely fine-grained model of temporal progression of Huntington's disease from premanifest through to manifest stages. We employ a probabilistic event-based model to determine the sequence of appearance of atrophy in brain volumes, learned from structural MRI in the Track-HD study, as well as to estimate the uncertainty in the ordering. We use longitudinal and phenotypic data to demonstrate the utility of the patient staging system that the resulting model provides. The model recovers the following order of detectable changes in brain region volumes: putamen, caudate, pallidum, insula white matter, nonventricular cerebrospinal fluid, amygdala, optic chiasm, third ventricle, posterior insula, and basal forebrain. This ordering is mostly preserved even under cross-validation of the uncertainty in the event sequence. Longitudinal analysis performed using 6 years of follow-up data from baseline confirms efficacy of the model, as subjects consistently move to later stages with time, and significant correlations are observed between the estimated stages and nonimaging phenotypic markers. We used a data-driven method to provide new insight into Huntington's disease progression as well as new power to stage and predict conversion. Our results highlight the potential of disease progression models, such as the event-based model, to provide new insight into Huntington's disease progression and to support fine-grained patient stratification for future precision medicine in Huntington's disease.

  20. Exit Polls and Voter Turnout

    DEFF Research Database (Denmark)

    Andersen, Asger Lau; Jensen, Thomas

    2014-01-01

    After the 2009 referendum on a proposed change to the Danish Law of Succession, it was widely claimed that the early publication of exit poll results changed the rate of turnout and eventually the outcome. We investigate this claim and contribute to the wider debate on the implications of exit...... polls by setting up and analyzing a formal model. We find that the introduction of an exit poll influences the incentive to vote both before and after the poll is published, but the signs of the effects are generally ambiguous. The observation that exit polls influence the incentive to vote even before...

  1. Destination and source memory in Huntington's disease

    NARCIS (Netherlands)

    El Haj, M.; Caillaud, M.; Verny, C.; Fasotti, L.; Allain, P.

    2016-01-01

    Destination memory refers to the recall of the destination of previously relayed information, and source memory refers to the recollection of the origin of received information. We compared both memory systems in Huntington's disease (HD) participants. For this, HD participants and healthy adults

  2. Perceptions of genetic discrimination among people at risk for Huntington's disease: a cross sectional survey.

    Science.gov (United States)

    Bombard, Yvonne; Veenstra, Gerry; Friedman, Jan M; Creighton, Susan; Currie, Lauren; Paulsen, Jane S; Bottorff, Joan L; Hayden, Michael R

    2009-06-09

    To assess the nature and prevalence of genetic discrimination experienced by people at risk for Huntington's disease who had undergone genetic testing or remained untested. Cross sectional, self reported survey. Seven genetics and movement disorders clinics servicing rural and urban communities in Canada. 233 genetically tested and untested asymptomatic people at risk for Huntington's disease (response rate 80%): 167 underwent testing (83 had the Huntington's disease mutation, 84 did not) and 66 chose not to be tested. Self reported experiences of genetic discrimination and related psychological distress based on family history or genetic test results. Discrimination was reported by 93 respondents (39.9%). Reported experiences occurred most often in insurance (29.2%), family (15.5%), and social (12.4%) settings. There were few reports of discrimination in employment (6.9%), health care (8.6%), or public sector settings (3.9%). Although respondents who were aware that they carried the Huntington's disease mutation reported the highest levels of discrimination, participation in genetic testing was not associated with increased levels of genetic discrimination. Family history of Huntington's disease, rather than the result of genetic testing, was the main reason given for experiences of genetic discrimination. Psychological distress was associated with genetic discrimination (PGenetic discrimination was commonly reported by people at risk for Huntington's disease and was a source of psychological distress. Family history, and not genetic testing, was the major reason for genetic discrimination.

  3. A randomized, double-blind, placebo-controlled study of latrepirdine in patients with mild to moderate Huntington disease

    DEFF Research Database (Denmark)

    Hjermind, Lena Elisabeth

    2013-01-01

    BACKGROUND Latrepirdine is an orally administered experimental small molecule that was initially developed as an antihistamine and subsequently was shown to stabilize mitochondrial membranes and function, which might be impaired in Huntington disease. OBJECTIVE To determine the effect of latrepir......BACKGROUND Latrepirdine is an orally administered experimental small molecule that was initially developed as an antihistamine and subsequently was shown to stabilize mitochondrial membranes and function, which might be impaired in Huntington disease. OBJECTIVE To determine the effect...... of latrepirdine on cognition and global function in patients with mild to moderate Huntington disease. DESIGN Randomized, double-blind, placebo-controlled study. SETTING Sixty-four research centers in Australia, Europe, and North America. PATIENTS Four hundred three patients with mild to moderate Huntington...... between those randomized to latrepirdine (68.5%) and placebo (68.0%). CONCLUSION In patients with mild to moderate Huntington disease and cognitive impairment, treatment with latrepirdine for 6 months was safe and well tolerated but did not improve cognition or global function relative to placebo. TRIAL...

  4. Firm Exit, Technological Progress and Trade

    DEFF Research Database (Denmark)

    Schröder, Philipp; Sørensen, Allan

    The dynamics of export market exit and firm closure have found limited attention in the new heterogeneous-firms trade literature. In fact, several of the predictions on firm survival and exit stemming from this new class of models are at odds with the stylized facts. Empirically, higher productiv......The dynamics of export market exit and firm closure have found limited attention in the new heterogeneous-firms trade literature. In fact, several of the predictions on firm survival and exit stemming from this new class of models are at odds with the stylized facts. Empirically, higher...... productivity firms survive longer, most firm closures are young firms, higher productivity exporters are more likely to continue to export compared to less productive exporters and market exits as well as firm closures are typically preceded by periods of contracting market shares. The present paper shows...... liberalization on export market exit and firm closure....

  5. An improved assay for the determination of Huntington`s disease allele size

    Energy Technology Data Exchange (ETDEWEB)

    Reeves, C.; Klinger, K.; Miller, G. [Intergrated Genetics, Framingham, MA (United States)

    1994-09-01

    The hallmark of Huntington`s disease (HD) is the expansion of a polymorphic (CAG)n repeat. Several methods have been published describing PCR amplification of this region. Most of these assays require a complex PCR reaction mixture to amplify this GC-rich region. A consistent problem with trinucleotide repeat PCR amplification is the presence of a number of {open_quotes}stutter bands{close_quotes} which may be caused by primer or amplicon slippage during amplification or insufficient polymerase processivity. Most assays for HD arbitrarily select a particular band for diagnostic purposes. Without a clear choice for band selection such an arbitrary selection may result in inconsistent intra- or inter-laboratory findings. We present an improved protocol for the amplification of the HD trinucleotide repeat region. This method simplifies the PCR reaction buffer and results in a set of easily identifiable bands from which to determine allele size. HD alleles were identified by selecting bands of clearly greater signal intensity. Stutter banding was much reduced thus permitting easy identification of the most relevant PCR product. A second set of primers internal to the CCG polymorphism was used in selected samples to confirm allele size. The mechanism of action of N,N,N trimethylglycine in the PCR reaction is not clear. It may be possible that the minimal isostabilizing effect of N,N,N trimethylglycine at 2.5 M is significant enough to affect primer specificity. The use of N,N,N trimethylglycine in the PCR reaction facilitated identification of HD alleles and may be appropriate for use in other assays of this type.

  6. Music therapy in Huntington's disease: a protocol for a multi-center randomized controlled trial.

    Science.gov (United States)

    van Bruggen-Rufi, Monique; Vink, Annemieke; Achterberg, Wilco; Roos, Raymund

    2016-07-26

    Huntington's disease is a progressive, neurodegenerative disease with autosomal dominant inheritance, characterized by motor disturbances, cognitive decline and behavioral and psychological symptoms. Since there is no cure, all treatment is aimed at improving quality of life. Music therapy is a non-pharmacological intervention, aiming to improve the quality of life, but its use and efficacy in patients with Huntington's disease has hardly been studied. In this article, a protocol is described to study the effects of music therapy in comparison with a control intervention to improve quality of life through stimulating expressive and communicative skills. By targeting these skills we assume that the social-cognitive functioning will improve, leading to a reduction in behavioral problems, resulting in an overall improvement of the quality of life in patients with Huntington's disease. The study is designed as a multi-center single-blind randomised controlled intervention trial. Sixty patients will be randomised using centre-stratified block-permuted randomisation. Patients will be recruited from four long-term care facilities specialized in Huntington's disease-care in The Netherlands. The outcome measure to assess changes in expressive and communication skills is the Behaviour Observation Scale Huntington and changes in behavior will be assessed by the Problem Behaviour Assesment-short version and by the BOSH. Measurements take place at baseline, then 8, 16 (end of intervention) and 12 weeks after the last intervention (follow-up). This randomized controlled study will provide greater insight into the effectiveness of music therapy on activities of daily living, social-cognitive functioning and behavior problems by improving expressive and communication skills, thus leading to a better quality of life for patients with Huntington's disease. Netherlands Trial Register: NTR4904 , registration date Nov. 15, 2014.

  7. Caregiver roles in families affected by Huntington's disease

    DEFF Research Database (Denmark)

    Røthing, Merete; Malterud, Kirsti; Frich, Jan C

    2013-01-01

    AIM: The objective of this study was to explore family caregivers' experiences with the impact of Huntington's disease (HD) on the family structure and roles in the family. METHODOLOGY: We interviewed 15 family caregivers in families affected by HD, based on a semi-structured interview guide...... for impairments by taking on adult responsibilities, and in some families, a child had the role as main caregiver. The increasing need for care could cause conflicts between the role as family member and family caregiver. The burden of care within the family could fragment and isolate the family. CONCLUSIONS......: Huntington's disease has a major impact on family systems. Caregiver roles are shaped by impairments in the affected family member and corresponding dynamic adoption and change in roles within the family. Making assessments of the family structure and roles, professionals may understand more about how...

  8. 77 FR 71636 - Huntington Foam LLC, Fort Smith, AR; Notice of Revised Determination on Reconsideration

    Science.gov (United States)

    2012-12-03

    ... Smith, AR; Notice of Revised Determination on Reconsideration On August 8, 2012, the Department of Labor... workers and former workers of Huntington Foam LLC, Fort Smith, Arkansas (subject firm). The workers are... reconsideration investigation, I determine that workers of Huntington Foam LLC, Fort Smith, Arkansas, who were...

  9. Promoting Exit from Violent Extremism

    DEFF Research Database (Denmark)

    Dalgaard-Nielsen, Anja

    2013-01-01

    A number of Western countries are currently adding exit programs targeting militant Islamists to their counterterrorism efforts. Drawing on research into voluntary exit from violent extremism, this article identifies themes and issues that seem to cause doubt, leading to exit. It then provides a ...... the influence attempt as subtle as possible, use narratives and self-affirmatory strategies to reduce resistance to persuasion, and consider the possibility to promote attitudinal change via behavioral change as an alternative to seek to influence beliefs directly....

  10. Unravelling and Exploiting Astrocyte Dysfunction in Huntington's Disease

    DEFF Research Database (Denmark)

    Khakh, Baljit S.; Beaumont, Vahri; Cachope, Roger

    2017-01-01

    Astrocytes are abundant within mature neural circuits and are involved in brain disorders. Here, we summarize our current understanding of astrocytes and Huntington's disease (HD), with a focus on correlative and causative dysfunctions of ion homeostasis, calcium signaling, and neurotransmitter...

  11. [Sporadic juvenile forms of Huntington's chorea].

    Science.gov (United States)

    Zinchenko, A P; Goncharov, V D; Burtianskii, D L; Zakhar'ev, Iu M

    1980-01-01

    Six patients with Huntington's chorea in the age of 15-24 years old, suffered from diffusive choreic hyperkynesis with slowly progressive dementia. The development of this disease in childhood and adolescence was atypical, as nobody in the family and in kin sufferred from it and it was difficult to diagnose the disease. Recognition of the disease was promoted by pneumoencephalography, electromyography and memory investigation.

  12. Did the "Woman in the Attic" in Jane Eyre Have Huntington Disease?

    Directory of Open Access Journals (Sweden)

    Elizabeth A. Coon

    2015-07-01

    Full Text Available Background: References to neurologic disorders are frequently found in fictional literature and may precede description in the medical literature. Aim: Our aim was to compare Charlotte Brontë’s depiction of Bertha Mason in Jane Eyre to the tenets set forth in George Huntington’s original essay “On chorea” with the hypothesis that Mason was displaying features of Huntington disease. Results: Charlotte Brontë’s 1847 Victorian novel Jane Eyre features the character Bertha Mason, who is portrayed with a progressive psychiatric illness, violent movements, and possible cognitive decline. Similar to Huntington’s tenets, Mason has a disorder with a strong family history suggestive of autosomal dominant inheritance with onset in adulthood, and culminating in suicide. Conclusion: Brontë’s character had features of Huntington disease as originally described by Huntington. Brontë’s keen characterization may have increased awareness of treatment of neuropsychiatric patients in the Victorian era.

  13. Motor cortex synchronization influences the rhythm of motor performance in premanifest huntington's disease.

    Science.gov (United States)

    Casula, Elias P; Mayer, Isabella M S; Desikan, Mahalekshmi; Tabrizi, Sarah J; Rothwell, John C; Orth, Michael

    2018-03-01

    In Huntington's disease there is evidence of structural damage in the motor system, but it is still unclear how to link this to the behavioral disorder of movement. One feature of choreic movement is variable timing and coordination between sequences of actions. We postulate this results from desynchronization of neural activity in cortical motor areas. The objective of this study was to explore the ability to synchronize activity in a motor network using transcranial magnetic stimulation and to relate this to timing of motor performance. We examined synchronization in oscillatory activity of cortical motor areas in response to an external input produced by a pulse of transcranial magnetic stimulation. We combined this with EEG to compare the response of 16 presymptomatic Huntington's disease participants with 16 age-matched healthy volunteers to test whether the strength of synchronization relates to the variability of motor performance at the following 2 tasks: a grip force task and a speeded-tapping task. Phase synchronization in response to M1 stimulation was lower in Huntington's disease than healthy volunteers (P synchronization (r = -0.356; P synchronization and desynchronization could be a physiological basis for some key clinical features of Huntington's disease. © 2018 International Parkinson and Movement Disorder Society. © 2018 International Parkinson and Movement Disorder Society.

  14. Network topology and functional connectivity disturbances precede the onset of Huntington's disease.

    Science.gov (United States)

    Harrington, Deborah L; Rubinov, Mikail; Durgerian, Sally; Mourany, Lyla; Reece, Christine; Koenig, Katherine; Bullmore, Ed; Long, Jeffrey D; Paulsen, Jane S; Rao, Stephen M

    2015-08-01

    Cognitive, motor and psychiatric changes in prodromal Huntington's disease have nurtured the emergent need for early interventions. Preventive clinical trials for Huntington's disease, however, are limited by a shortage of suitable measures that could serve as surrogate outcomes. Measures of intrinsic functional connectivity from resting-state functional magnetic resonance imaging are of keen interest. Yet recent studies suggest circumscribed abnormalities in resting-state functional magnetic resonance imaging connectivity in prodromal Huntington's disease, despite the spectrum of behavioural changes preceding a manifest diagnosis. The present study used two complementary analytical approaches to examine whole-brain resting-state functional magnetic resonance imaging connectivity in prodromal Huntington's disease. Network topology was studied using graph theory and simple functional connectivity amongst brain regions was explored using the network-based statistic. Participants consisted of gene-negative controls (n = 16) and prodromal Huntington's disease individuals (n = 48) with various stages of disease progression to examine the influence of disease burden on intrinsic connectivity. Graph theory analyses showed that global network interconnectivity approximated a random network topology as proximity to diagnosis neared and this was associated with decreased connectivity amongst highly-connected rich-club network hubs, which integrate processing from diverse brain regions. However, functional segregation within the global network (average clustering) was preserved. Functional segregation was also largely maintained at the local level, except for the notable decrease in the diversity of anterior insula intermodular-interconnections (participation coefficient), irrespective of disease burden. In contrast, network-based statistic analyses revealed patterns of weakened frontostriatal connections and strengthened frontal-posterior connections that evolved as disease

  15. Mental Symptoms in Huntington's Disease and a Possible Primary Aminergic Neuron Lesion

    Science.gov (United States)

    Mann, J. John; Stanley, Michael; Gershon, Samuel; Rossor, M.

    1980-12-01

    Monoamine oxidase activity was higher in the cerebral cortex and basal ganglia of patients dying from Huntington's disease than in controls. Enzyme kinetics and multiple substrate studies indicated that the increased activity was due to elevated concentrations of monoamine oxidase type B. Concentrations of homovanillic acid were increased in the cerebral cortex but not in the basal ganglia of brains of patients with Huntington's disease. These changes may represent a primary aminergic lesion that could underlie some of the mental symptoms of this disease.

  16. Timely Endocytosis of Cytokinetic Enzymes Prevents Premature Spindle Breakage during Mitotic Exit.

    Directory of Open Access Journals (Sweden)

    Cheen Fei Chin

    2016-07-01

    Full Text Available Cytokinesis requires the spatio-temporal coordination of membrane deposition and primary septum (PS formation at the division site to drive acto-myosin ring (AMR constriction. It has been demonstrated that AMR constriction invariably occurs only after the mitotic spindle disassembly. It has also been established that Chitin Synthase II (Chs2p neck localization precedes mitotic spindle disassembly during mitotic exit. As AMR constriction depends upon PS formation, the question arises as to how chitin deposition is regulated so as to prevent premature AMR constriction and mitotic spindle breakage. In this study, we propose that cells regulate the coordination between spindle disassembly and AMR constriction via timely endocytosis of cytokinetic enzymes, Chs2p, Chs3p, and Fks1p. Inhibition of endocytosis leads to over accumulation of cytokinetic enzymes during mitotic exit, which accelerates the constriction of the AMR, and causes spindle breakage that eventually could contribute to monopolar spindle formation in the subsequent round of cell division. Intriguingly, the mitotic spindle breakage observed in endocytosis mutants can be rescued either by deleting or inhibiting the activities of, CHS2, CHS3 and FKS1, which are involved in septum formation. The findings from our study highlight the importance of timely endocytosis of cytokinetic enzymes at the division site in safeguarding mitotic spindle integrity during mitotic exit.

  17. Beautiful Science: The Public and Private History of Astronomy at the Huntington Library

    Science.gov (United States)

    Lewis, Daniel

    2009-05-01

    The history of astronomy has a long tradition within research libraries. The rare collections at the Huntington Library (encompassing American and British history from around 1000 CE to the present, in many different subject areas) are among the most heavily-used in the United States, The history of astronomy holdings are a cornerstone within the library's history of science holdings. This talk will present the two faces of the history of astronomy holdings at the Huntington Library. The first of these is the research end of operations: what the collections consist of, how the scholarly public uses the collections, and what the implications are for modern astronomical practice. The second element concerns the public exhibit face of the history of astronomy holdings at The Huntington. Of the 600,000 people who visit the Huntington each year, the majority visit public displays and rare book and manuscript exhibits. "Beautiful Science: Ideas That Changed the World” is a new permanent history of science exhibit. One quarter of the exhibit relates to the history of astronomy. Public exhibits require a particular kind of planning and bring a specific set of values to the history of astronomy. Public exhibits also have their own concerns, and this talk will cover a number of those issues as well as the research issues.

  18. JUNCTOPHILIN 3 (JPH3) EXPANSION MUTATIONS CAUSING HUNTINGTON DISEASE LIKE 2 (HDL2) ARE COMMON IN SOUTH AFRICAN PATIENTS WITH AFRICAN ANCESTRY AND A HUNTINGTON DISEASE PHENOTYPE

    Science.gov (United States)

    Krause, A; Mitchell, CL; Essop, F; Tager, S; Temlett, J; Stevanin, G; Ross, CA; Rudnicki, DD; Margolis, RL

    2015-01-01

    Huntington disease (HD) is a progressive autosomal dominant neurodegenerative disorder, characterized by abnormal movements, cognitive decline and psychiatric symptoms, caused by a CAG repeat expansion in the huntingtin (HTT) gene on chromosome 4p. A CAG/CTG repeat expansion in the junctophilin-3 (JPH3) gene on chromosome 16q24.2 causes a Huntington disease-like phenotype (HDL2). All patients to date with HDL2 have some African ancestry. The present study aimed to characterize the genetic basis of the Huntington disease phenotype in South Africans and to investigate the possible origin of the JPH3 mutation. In a sample of unrelated South African individuals referred for diagnostic HD testing, 62% (106/171) of white patients compared to only 36% (47/130) of black patients had an expansion in HTT. However, 15% (20/130) of black South African patients and no white patients (0/171) had an expansion in JPH3, confirming the diagnosis of Huntington disease like 2 (HDL2). Individuals with HDL2 share many clinical features with individuals with HD and are clinically indistinguishable in many cases, although the average age of onset and diagnosis in HDL2 is 5 years later than HD and individual clinical features may be more prominent. HDL2 mutations contribute significantly to the HD phenotype in South Africans with African ancestry. JPH3 haplotype studies in 31 families, mainly from South Africa and North America, provide evidence for a founder mutation and support a common African origin for all HDL2 patients. Molecular testing in individuals with an HD phenotype and African ancestry should include testing routinely for JPH3 mutations. PMID:26079385

  19. Clinical neurogenetics: huntington disease.

    Science.gov (United States)

    Bordelon, Yvette M

    2013-11-01

    Huntington disease (HD) is an autosomal dominant, adult-onset, progressive neurodegenerative disease characterized by the triad of abnormal movements (typically chorea), cognitive impairment, and psychiatric problems. It is caused by an expanded CAG repeat in the gene encoding the protein huntingtin on chromosome 4 and causes progressive atrophy of the striatum as well as cortical and other extrastriatal structures. Genetic testing has been available since 1993 to confirm diagnosis in affected adults and for presymptomatic testing in at-risk individuals. This review covers HD signs, symptoms, and pathophysiology; current genetic testing issues; and current and future treatment strategies. Copyright © 2013 Elsevier Inc. All rights reserved.

  20. Spoiled Onions: Exposing Malicious Tor Exit Relays

    OpenAIRE

    Winter, Philipp; Lindskog, Stefan

    2014-01-01

    Several hundred Tor exit relays together push more than 1 GiB/s of network traffic. However, it is easy for exit relays to snoop and tamper with anonymised network traffic and as all relays are run by independent volunteers, not all of them are innocuous. In this paper, we seek to expose malicious exit relays and document their actions. First, we monitored the Tor network after developing a fast and modular exit relay scanner. We implemented several scanning modules for detecting common attac...

  1. Eternal extended inflation and graceful exit from old inflation without Jordan-Brans-Dicke

    International Nuclear Information System (INIS)

    Linde, A.

    1990-01-01

    Recently a possible solution to the graceful exit problem of the old inflation was proposed in the context of the Jordan-Brans-Dicke theory (extended inflation). In this paper we will argue that inflation in this theory occurs in a most natural way if it starts near the Planck density, as in the standard version of chaotic inflation. With most natural initial conditions, the inflationary universe in the JBD theory enters the stage of permanent reproduction of new inflationary domains (eternal extended inflation). In order to realize the extended inflation scenario at least two classical scalar fields driving inflation are necessary, as distinct from the simplest versions of new and chaotic inflation. It is shown that in the theory of two scalar fields one can solve the graceful exit problem even without modifying the Einstein gravity theory, due to the possibility that the decay rate of the false vacuum in old inflation depends on the value of the second scalar field and hence on time. (orig.)

  2. Understanding international exit from a non-economic and emotional perspective: the case of Taiwanese entrepreneurs exit China

    OpenAIRE

    Lin, Yangpei

    2015-01-01

    I investigate why Taiwanese entrepreneurs who have invested in China exit. Viewed from the non-economic perspective, there are three main themes in this thesis. Theme A focuses on the non-economic variables in international exit. Theme B examines how incident-generated emotions shape entrepreneur’s actions in internationalization. Theme C presents an overview of the decision-making of international exit, summarizing the finding in Theme A and Theme B and revisiting the theor...

  3. Reduction in mitochondrial DNA copy number in peripheral leukocytes after onset of Huntington's disease

    DEFF Research Database (Denmark)

    Petersen, Maria Hvidberg; Budtz-Jørgensen, Esben; Sørensen, Sven Asger

    2014-01-01

    Huntington's disease (HD) is an inherited neurodegenerative disorder characterised by movement disorder, cognitive symptoms and psychiatric symptoms with predominantly adult-onset. The mutant huntingtin protein leads to mitochondrial dysfunction in blood leukocytes. This discovery led to the inve......Huntington's disease (HD) is an inherited neurodegenerative disorder characterised by movement disorder, cognitive symptoms and psychiatric symptoms with predominantly adult-onset. The mutant huntingtin protein leads to mitochondrial dysfunction in blood leukocytes. This discovery led...

  4. Ethical issues and Huntington's disease | Kromberg | South African ...

    African Journals Online (AJOL)

    The practice of genetic counselling gives rise to many ethical dilemmas, and counsellors need to be familiar with the principles of biomedical ethics. The primary principles include respect for autonomy, beneficence, non-maleficence and justice. A case of identical twins at 50% risk for Huntington's disease, in which only one ...

  5. 14 CFR 25.809 - Emergency exit arrangement.

    Science.gov (United States)

    2010-01-01

    ... moveable door or hatch in the external walls of the fuselage, allowing an unobstructed opening to the... event of failure of the primary system. Manual operation of the exit (after failure of the primary... during flight. (i) Each emergency exit must have a means to retain the exit in the open position, once...

  6. Characterisation of aggression in Huntington's disease: rates, types and antecedents in an inpatient rehabilitation setting.

    Science.gov (United States)

    Brown, Anahita; Sewell, Katherine; Fisher, Caroline A

    2017-10-01

    To systematically review aggression in an inpatient Huntington's cohort examining rates, types and antecedents. Although the prevalence of aggression in Huntington's disease is high, research into this problematic behaviour has been limited. Few studies have investigated the nature of aggressive behaviour in Huntington's disease or antecedents that contribute to its occurrence. A systematic, double-coded, electronic medical file audit. The electronic hospital medical records of 10 people with Huntington's disease admitted to a brain disorders unit were audited for a 90-day period using the Overt Aggression Scale-Modified for Neurorehabilitation framework, yielding 900 days of clinical data. Nine of 10 clients exhibited aggression during the audit period. Both verbal (37·1%) aggression and physical aggression were common (33·8%), along with episodes of mixed verbal and physical aggression (15·2%), while aggression to objects/furniture was less prevalent (5·5%). The most common antecedent was physical guidance with personal care, far exceeding any other documented antecedents, and acting as the most common trigger for four of the nine clients who exhibited aggression. For the remaining five clients, there was intraindividual heterogeneity in susceptibility to specific antecedents. In Huntington's sufferers at mid- to late stages following disease onset, particular care should be made with personal care assistance due to the propensity for these procedures to elicit an episode of aggression. However, given the degree of intraindividual heterogeneity in susceptibility to specific antecedents observed in the present study, individualised behaviour support plans and sensory modulation interventions may be the most useful in identifying triggers and managing aggressive episodes. Rates of aggression in Huntington's disease inpatients can be high. Knowledge of potential triggers, such as personal care, is important for nursing and care staff, so that attempts can be

  7. Speeding driving behaviour: Age and gender experimental analysis

    Directory of Open Access Journals (Sweden)

    Teo Sir Hiang

    2016-01-01

    Full Text Available Road safety is a substantial issue worldwide. Speeding contributed to one-third of all the fatal crashes reported from year 2002 to 2011 with young drivers reported to have the highest fatality and injury rates. This paper studied on the speeding driving behavior of 10 teenagers and 10 adults, from both genders. The aim was to investigate the relationship between age and gender with speeding driving behavior. The drivers were required to drive within an enclosed compound by using a test car. Results showed young and male drivers averagely travelled at higher velocity before entering the roundabout and at the same time accelerate to higher velocity upon exiting the roundabout compared to old and female drivers.

  8. Family caregivers' views on coordination of care in Huntington's disease

    DEFF Research Database (Denmark)

    Røthing, Merete; Malterud, Kirsti; Frich, Jan C

    2015-01-01

    BACKGROUND: Collaboration between family caregivers and health professionals in specialised hospitals or community-based primary healthcare systems can be challenging. During the course of severe chronic disease, several health professionals might be involved at a given time, and the patient......'s illness may be unpredictable or not well understood by some of those involved in the treatment and care. AIM: The aim of this study was to explore the experiences and expectations of family caregivers for persons with Huntington's disease concerning collaboration with healthcare professionals. METHODS......: To shed light on collaboration from the perspectives of family caregivers, we conducted an explorative, qualitative interview study with 15 adult participants experienced from caring for family members in all stages of Huntington's disease. Data were analysed with systematic text condensation, a cross...

  9. Towards A Model of Identity and Role Exit

    Directory of Open Access Journals (Sweden)

    Jason S. Milne

    2011-11-01

    Full Text Available Explanations of role exit often focus on how factors associated with a specific role that affect whether the individual will exit a role or not. Other research explains how identities affect our performance in a role. However, no one has yet to demonstrate the connection between role-set factors and identities, and role exit. Using data from a survey of 940 current and former soccer referees, this paper provides a model of role exit that involves a complex of processes that include role-set factors (structural and cultural factors associated with a specific role and identity processes. Specifically, this paper demonstrates that, other than role conflict, identity processes explain the relationship between role-set factors and role exit. The model provides a beginning method for understanding the connection between identities and role exit.

  10. Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study

    OpenAIRE

    Hensman Moss, Davina J; Pardinas, Antonio; Langbehn, Douglas; Lo, Kitty; Leavitt, Blair R; Roos, Raymund; Durr, Alexandra; Mead, Simon; Holmans, Peter; Jones, Lesley; Tabrizi, Sarah J; Coleman, A; Santos, R Dar; Decolongon, J; Sturrock, A

    2017-01-01

    Background\\ud \\ud Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate a novel measure of disease progression and to identify genetic markers associated with this progression measure.\\ud \\ud Methods\\ud \\ud We generated a progression score on the basis of principal ...

  11. Palliative Care in Huntington Disease: Personal Reflections and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Christopher G. Tarolli

    2017-04-01

    Full Text Available Background Huntington disease is a fatal, autosomal dominant, neurodegenerative disorder manifest by the triad of a movement disorder, behavioral disturbances, and dementia. At present, no curative or disease modifying therapies exist for the condition and current treatments are symptomatic. Palliative care is an approach to care that focuses on symptom relief, patient and caregiver support, and end of life care. There is increasing evidence of the benefit of palliative care throughout the course of neurodegenerative conditions including Parkinson disease and amyotrophic lateral sclerosis. However, beyond its application at the end of life, little is known about the role of palliative care in Huntington disease.Methods In this article, we discuss what is known about palliative care in Huntington disease, specifically related to early disease burden, caregiver burnout, advance care planning, and end of life care.Results We provide a review of the current literature and discuss our own care practices.Discussion We conclude by discussing questions that remain unanswered and positing ideas for future work in the field.

  12. Huntington Revisited: Is Conservative Realism Still Essential for the Military Ethic

    National Research Council Canada - National Science Library

    Mahoney-Norris, Kathleen A

    2001-01-01

    ...). Furthermore, Huntington has developed what appears to be a powerful argument as to why conservative realism should be considered a fundamental component of the professional ethic of the military officer...

  13. The use of stem cells in regenerative medicine for Parkinson's and Huntington's Diseases.

    Science.gov (United States)

    Lescaudron, L; Naveilhan, P; Neveu, I

    2012-01-01

    Cell transplantation has been proposed as a means of replacing specific cell populations lost through neurodegenerative processes such as that seen in Parkinson's or Huntington's diseases. Improvement of the clinical symptoms has been observed in a number of Parkinson and Huntington's patients transplanted with freshly isolated fetal brain tissue but such restorative approach is greatly hampered by logistic and ethical concerns relative to the use of fetal tissue, in addition to potential side effects that remain to be controlled. In this context, stem cells that are capable of self-renewal and can differentiate into neurons, have received a great deal of interest, as demonstrated by the numerous studies based on the transplantation of neural stem/progenitor cells, embryonic stem cells or mesenchymal stem cells into animal models of Parkinson's or Huntington's diseases. More recently, the induction of pluripotent stem cells from somatic adult cells has raised a new hope for the treatment of neurodegenerative diseases. In the present article, we review the main experimental approaches to assess the efficiency of cell-based therapy for Parkinson's or Huntington's diseases, and discuss the recent advances in using stem cells to replace lost dopaminergic mesencephalic or striatal neurons. Characteristics of the different stem cells are extensively examined with a special attention to their ability of producing neurotrophic or immunosuppressive factors, as these may provide a favourable environment for brain tissue repair and long-term survival of transplanted cells in the central nervous system. Thus, stem cell therapy can be a valuable tool in regenerative medicine.

  14. Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease

    DEFF Research Database (Denmark)

    Aziz, N A; Jurgens, C K; Landwehrmeyer, G B

    2009-01-01

    OBJECTIVE: Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG repeat expansion in the HD gene (HTT). We aimed to assess whether interaction between CAG repeat sizes in the mutant and normal allele could affect disease severity and progression. METHODS: Using...... with less severe symptoms and pathology. CONCLUSIONS: Increasing CAG repeat size in normal HTT diminishes the association between mutant CAG repeat size and disease severity and progression in Huntington disease. The underlying mechanism may involve interaction of the polyglutamine domains of normal...

  15. Exit or revival?

    International Nuclear Information System (INIS)

    Anon.

    2001-01-01

    The answer given by the international representative at the colloquium:'nuclear: exit or revival? ' was tending towards the revival. The international, democratic, ecological and of energy policy stakes are tackled. (N.C.)

  16. Hypocretin and melanin-concentrating hormone in patients with Huntington disease.

    NARCIS (Netherlands)

    Aziz, A.; Fronczek, R.; Maat-Schieman, M.L.; Unmehopa, U.A.; Roelandse, F.W.; Overeem, S.; Duinen, S.G. van; Lammers, G.J.; Swaab, D.F.; Roos, R.A.C.

    2008-01-01

    To evaluate whether hypocretin-1 (orexin-A) and melanin-concentrating hormone (MCH) neurotransmission are affected in patients with Huntington disease (HD), we immunohistochemically stained hypocretin and MCH neurons and estimated their total numbers in the lateral hypothalamus of both HD patients

  17. Presence of tau pathology within foetal neural allografts in patients with Huntington's and Parkinson's disease.

    Science.gov (United States)

    Cisbani, Giulia; Maxan, Alexander; Kordower, Jeffrey H; Planel, Emmanuel; Freeman, Thomas B; Cicchetti, Francesca

    2017-11-01

    Cell replacement has been explored as a therapeutic strategy to repair the brain in patients with Huntington's and Parkinson's disease. Post-mortem evaluations of healthy grafted tissue in such cases have revealed the development of Huntington- or Parkinson-like pathology including mutant huntingtin aggregates and Lewy bodies. An outstanding question remains if tau pathology can also be seen in patients with Huntington's and Parkinson's disease who had received foetal neural allografts. This was addressed by immunohistochemical/immunofluorescent stainings performed on grafted tissue of two Huntington's disease patients, who came to autopsy 9 and 12 years post-transplantation, and two patients with Parkinson's disease who came to autopsy 18 months and 16 years post-transplantation. We show that grafts also contain tau pathology in both types of transplanted patients. In two patients with Huntington's disease, the grafted tissue showed the presence of hyperphosphorylated tau [both AT8 (phospho-tau Ser202 and Thr205) and CP13 (pSer202) immunohistochemical stainings] pathological inclusions, neurofibrillary tangles and neuropil threads. In patients with Parkinson's disease, the grafted tissue was characterized by hyperphosphorylated tau (AT8; immunofluorescent staining) pathological inclusions, neurofibrillary tangles and neuropil threads but only in the patient who came to autopsy 16 years post-transplantation. Abundant tau-related pathology was observed in the cortex and striatum of all cases studied. While the striatum of the grafted Huntington's disease patient revealed an equal amount of 3-repeat and 4-repeat isoforms of tau, the grafted tissue showed elevated 4-repeat isoforms by western blot. This suggests that transplants may have acquired tau pathology from the host brain, although another possibility is that this was due to acceleration of ageing. This finding not only adds to the recent reports that tau pathology is a feature of these neurodegenerative

  18. Exit from Synchrony in Joint Improvised Motion.

    Directory of Open Access Journals (Sweden)

    Assi Dahan

    Full Text Available Motion synchrony correlates with effective and well-rated human interaction. However, people do not remain locked in synchrony; Instead, they repeatedly enter and exit synchrony. In many important interactions, such as therapy, marriage and parent-infant communication, it is the ability to exit and then re-enter synchrony that is thought to build strong relationship. The phenomenon of entry into zero-phase synchrony is well-studied experimentally and in terms of mathematical modeling. In contrast, exit-from-synchrony is under-studied. Here, we focus on human motion coordination, and examine the exit-from-synchrony phenomenon using experimental data from the mirror game paradigm, in which people perform joint improvised motion, and from human tracking of computer-generated stimuli. We present a mathematical mechanism that captures aspects of exit-from-synchrony in human motion. The mechanism adds a random motion component when the accumulated velocity error between the players is small. We introduce this mechanism to several models for human coordinated motion, including the widely studied HKB model, and the predictor-corrector model of Noy, Dekel and Alon. In all models, the new mechanism produces realistic simulated behavior when compared to experimental data from the mirror game and from tracking of computer generated stimuli, including repeated entry and exit from zero-phase synchrony that generates a complexity of motion similar to that of human players. We hope that these results can inform future research on exit-from-synchrony, to better understand the dynamics of coordinated action of people and to enhance human-computer and human-robot interaction.

  19. Randomized controlled trial of ethyl-eicosapentaenoic acid in Huntington disease: the TREND-HD study.

    Science.gov (United States)

    2008-12-01

    To determine whether ethyl-eicosapentaenoic acid (ethyl-EPA), an omega-3 fatty acid, improves the motor features of Huntington disease. Six-month multicenter, randomized, double-blind, placebo-controlled trial followed by a 6-month open-label phase without disclosing initial treatment assignments. Forty-one research sites in the United States and Canada. Three hundred sixteen adults with Huntington disease, enriched for a population with shorter trinucleotide (cytosine-adenine-guanine) repeat length expansions. Random assignment to placebo or ethyl-EPA, 1 g twice a day, followed by open-label treatment with ethyl-EPA. Six-month change in the Total Motor Score 4 component of the Unified Huntington's Disease Rating Scale analyzed for all research participants and those with shorter cytosine-adenine-guanine repeat length expansions (<45). At 6 months, the Total Motor Score 4 point change for patients receiving ethyl-EPA did not differ from that for those receiving placebo. No differences were found in measures of function, cognition, or global impression. Before public disclosure of the 6-month placebo-controlled results, 192 individuals completed the open-label phase. The Total Motor Score 4 change did not worsen for those who received active treatment for 12 continuous months compared with those who received active treatment for only 6 months (2.0-point worsening; P=.02). Ethyl-EPA was not beneficial in patients with Huntington disease during 6 months of placebo-controlled evaluation. Clinical Trial Registry clinicaltrials.gov Identifier: NCT00146211.

  20. Eternal extended inflation and graceful exit from old inflation without Jordan-Brans-Dicke

    Science.gov (United States)

    Linde, Andrei

    1990-10-01

    Recently a possible solution to the graceful exit problem of the old inflation was proposed in the context of the Jordan-Brans-Dicke theory (extended inflation). In this paper we will argue that inflation in this theory occurs in a most natural way if it starts near the Planck density, as in the standard version of chaotic inflation. With most natural initial conditions, the inflationary universe in the JBD theory enters the stage of permanent reproduction of new inflationary domains (eternal extended inflation). In order to realize the extended inflation scenario at least two classical scalar fields driving inflation are necessary, as distinct from the simplest versions of new and chaotic inflation. It is shown that in the theory of two scalar fields one can solve the graceful exit problem even without modifying the Einstein gravity theory, due to the possibility that the decay rate of the false rate vacuum in old inflation depends on the value of the second scalar field and hence on time. Address after 1 September 1990: Physics Department, Stanford University, Varian Building, Stanford, CA 94305, USA.

  1. Remifentanil in a patient with Huntington's chorea - case report ...

    African Journals Online (AJOL)

    Relatively few published case reports related to the anaesthetic management of Huntington's chorea (HC) exist. At the time of surgery no publications were found related to remifentanil's use in patients with HC. This case report describes the management of a confirmed HC patient requiring urgent decompression of a spinal ...

  2. Assessment of exit block following pulmonary vein isolation: far-field capture masquerading as entrance without exit block.

    Science.gov (United States)

    Vijayaraman, Pugazhendhi; Dandamudi, Gopi; Naperkowski, Angela; Oren, Jess; Storm, Randle; Ellenbogen, Kenneth A

    2012-10-01

    Complete electrical isolation of pulmonary veins (PVs) remains the cornerstone of ablation therapy for atrial fibrillation. Entrance block without exit block has been reported to occur in 40% of the patients. Far-field capture (FFC) can occur during pacing from the superior PVs to assess exit block, and this may appear as persistent conduction from PV to left atrium (LA). To facilitate accurate assessment of exit block. Twenty consecutive patients with symptomatic atrial fibrillation referred for ablation were included in the study. Once PV isolation (entrance block) was confirmed, pacing from all the bipoles on the Lasso catheter was used to assess exit block by using a pacing stimulus of 10 mA at 2 ms. Evidence for PV capture without conduction to LA was necessary to prove exit block. If conduction to LA was noticed, pacing output was decreased until there was PV capture without conduction to LA or no PV capture was noted to assess for far-field capture in both the upper PVs. All 20 patients underwent successful isolation (entrance block) of all 76 (4 left common PV) veins: mean age 58 ± 9 years; paroxysmal atrial fibrillation 40%; hypertension 70%, diabetes mellitus 30%, coronary artery disease 15%; left ventricular ejection fraction 55% ± 10%; LA size 42 ± 11 mm. Despite entrance block, exit block was absent in only 16% of the PVs, suggesting persistent PV to LA conduction. FFC of LA appendage was noted in 38% of the left superior PVs. FFC of the superior vena cava was noted in 30% of the right superior PVs. The mean pacing threshold for FFC was 7 ± 4 mA. Decreasing pacing output until only PV capture (loss of FFC) is noted was essential to confirm true exit block. FFC of LA appendage or superior vena cava can masquerade as persistent PV to LA conduction. A careful assessment for PV capture at decreasing pacing output is essential to exclude FFC. Copyright © 2012 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  3. Tritium in Exit Signs | RadTown USA | US EPA

    Science.gov (United States)

    2017-08-07

    Many exit signs contain tritium to light the sign without batteries or electricity. Using tritium in exit signs allows the sign to remain lit if the power goes out. Tritium is most dangerous when it is inhaled or swallowed. Never tamper with a tritium exit sign. If a tritium exit sign is broken, leave the area immediately and notify the building maintenance staff.

  4. High Protein Diet and Huntington's Disease.

    Directory of Open Access Journals (Sweden)

    Chiung-Mei Chen

    Full Text Available Huntington's disease (HD is a neurodegenerative disorder caused by the huntingtin (HTT gene with expanded CAG repeats. In addition to the apparent brain abnormalities, impairments also occur in peripheral tissues. We previously reported that mutant Huntingtin (mHTT exists in the liver and causes urea cycle deficiency. A low protein diet (17% restores urea cycle activity and ameliorates symptoms in HD model mice. It remains unknown whether the dietary protein content should be monitored closely in HD patients because the normal protein consumption is lower in humans (~15% of total calories than in mice (~22%. We assessed whether dietary protein content affects the urea cycle in HD patients. Thirty HD patients were hospitalized and received a standard protein diet (13.7% protein for 5 days, followed by a high protein diet (HPD, 26.3% protein for another 5 days. Urea cycle deficiency was monitored by the blood levels of citrulline and ammonia. HD progression was determined by the Unified Huntington's Disease Rating Scale (UHDRS. The HPD increased blood citrulline concentration from 15.19 μmol/l to 16.30 μmol/l (p = 0.0378 in HD patients but did not change blood ammonia concentration. A 2-year pilot study of 14 HD patients found no significant correlation between blood citrulline concentration and HD progression. Our results indicated a short period of the HPD did not markedly compromise urea cycle function. Blood citrulline concentration is not a reliable biomarker of HD progression.

  5. Entry and Exit Dynamics of Nascent Business Owners

    DEFF Research Database (Denmark)

    Rocha, Vera; Carneiro, Anabela; Varum, Celeste

    2015-01-01

    results suggest that different exit modes can be predicted by business owners’ entry route. Furthermore, different exit modes exhibit different duration dependence patterns according to the entry mode. Additionally, the paper shows that businesses started after a displacement episode are not necessarily......This paper reports a comprehensive study on the dynamics of nascent business owners using a unique longitudinal matched employer–employee dataset. We follow over 157,000 individuals who leave paid employment and become business owners during the period 1992–2007. The contributions of this paper...... are twofold. First, we analyze both entry and exit, identifying and characterizing different profiles of individuals leaving paid employment to become business owners, and distinguishing exits by dissolution from exits by ownership transfer. Second, we provide new evidence on how particular experiences...

  6. Psychodynamic theory and counseling in predictive testing for Huntington's disease.

    Science.gov (United States)

    Tassicker, Roslyn J

    2005-04-01

    This paper revisits psychodynamic theory, which can be applied in predictive testing counseling for Huntington's Disease (HD). Psychodynamic theory has developed from the work of Freud and places importance on early parent-child experiences. The nature of these relationships, or attachments are reflected in adult expectations and relationships. Two significant concepts, identification and fear of abandonment, have been developed and expounded by the psychodynamic theorist, Melanie Klein. The processes of identification and fear of abandonment can become evident in predictive testing counseling and are colored by the client's experience of growing up with a parent affected by Huntington's Disease. In reflecting on family-of-origin experiences, clients can also express implied expectations of the future, and future relationships. Case examples are given to illustrate the dynamic processes of identification and fear of abandonment which may present in the clinical setting. Counselor recognition of these processes can illuminate and inform counseling practice.

  7. Thermal IR exitance model of a plant canopy

    Science.gov (United States)

    Kimes, D. S.; Smith, J. A.; Link, L. E.

    1981-01-01

    A thermal IR exitance model of a plant canopy based on a mathematical abstraction of three horizontal layers of vegetation was developed. Canopy geometry within each layer is quantitatively described by the foliage and branch orientation distributions and number density. Given this geometric information for each layer and the driving meteorological variables, a system of energy budget equations was determined and solved for average layer temperatures. These estimated layer temperatures, together with the angular distributions of radiating elements, were used to calculate the emitted thermal IR radiation as a function of view angle above the canopy. The model was applied to a lodgepole pine (Pinus contorta) canopy over a diurnal cycle. Simulated vs measured radiometric average temperatures of the midcanopy layer corresponded with 2 C. Simulation results suggested that canopy geometry can significantly influence the effective radiant temperature recorded at varying sensor view angles.

  8. A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease.

    Directory of Open Access Journals (Sweden)

    John P Miller

    Full Text Available A genome-scale RNAi screen was performed in a mammalian cell-based assay to identify modifiers of mutant huntingtin toxicity. Ontology analysis of suppressor data identified processes previously implicated in Huntington's disease, including proteolysis, glutamate excitotoxicity, and mitochondrial dysfunction. In addition to established mechanisms, the screen identified multiple components of the RRAS signaling pathway as loss-of-function suppressors of mutant huntingtin toxicity in human and mouse cell models. Loss-of-function in orthologous RRAS pathway members also suppressed motor dysfunction in a Drosophila model of Huntington's disease. Abnormal activation of RRAS and a down-stream effector, RAF1, was observed in cellular models and a mouse model of Huntington's disease. We also observe co-localization of RRAS and mutant huntingtin in cells and in mouse striatum, suggesting that activation of R-Ras may occur through protein interaction. These data indicate that mutant huntingtin exerts a pathogenic effect on this pathway that can be corrected at multiple intervention points including RRAS, FNTA/B, PIN1, and PLK1. Consistent with these results, chemical inhibition of farnesyltransferase can also suppress mutant huntingtin toxicity. These data suggest that pharmacological inhibition of RRAS signaling may confer therapeutic benefit in Huntington's disease.

  9. Exploring the Reasons and Ways to Exit: The Entrepreneur Perspective

    NARCIS (Netherlands)

    Parastuty, Zulaicha; Breitenecker, Robert J.; Schwarz, Erich J.; Harms, Rainer; Bögenhold, Dieter; Bonnet, Jean; Dejardin, Marcus; Garcia Perez de Lema, Domingo

    2016-01-01

    Research on entrepreneurial exit has received growing attention recently, attributing to the importance of exit in the entrepreneurial process. Yet, the complex phenomena of exit render the research scattered in the field. This research is aimed at understanding entrepreneurial exit at the

  10. The Political Economy of Early Exit

    DEFF Research Database (Denmark)

    Schmitt, Carina; Starke, Peter

    2016-01-01

    Large-scale exit from the labour market began in the 1970s in many OECD countries. The literature indicates that individual early retirement decisions are facilitated by generous and accessible ‘pathways’ into retirement in the public pension system, unemployment insurance or disability benefits....... in the tradable sector, against a more traditional class-based logic of welfare state policy-making. Quantitative analysis of employment outcomes in 21 countries shows that the political economy of early exit clearly rests on the sectoral politics of cost-shifting.......Large-scale exit from the labour market began in the 1970s in many OECD countries. The literature indicates that individual early retirement decisions are facilitated by generous and accessible ‘pathways’ into retirement in the public pension system, unemployment insurance or disability benefits....... It is unclear, however, why early exit became so much more prevalent in some countries than in others and why such differences remain, despite a recent shift back towards higher employment rates and ‘active ageing’. We test a logic of sectoral cost-shifting politics involving cross-class alliances...

  11. Prevalence of Huntington's disease gene CAG trinucleotide repeat alleles in patients with bipolar disorder.

    Science.gov (United States)

    Ramos, Eliana Marisa; Gillis, Tammy; Mysore, Jayalakshmi S; Lee, Jong-Min; Alonso, Isabel; Gusella, James F; Smoller, Jordan W; Sklar, Pamela; MacDonald, Marcy E; Perlis, Roy H

    2015-06-01

    Huntington's disease is a neurodegenerative disorder characterized by motor, cognitive, and psychiatric symptoms that are caused by huntingtin gene (HTT) CAG trinucleotide repeat alleles of 36 or more units. A greater than expected prevalence of incompletely penetrant HTT CAG repeat alleles observed among individuals diagnosed with major depressive disorder raises the possibility that another mood disorder, bipolar disorder, could likewise be associated with Huntington's disease. We assessed the distribution of HTT CAG repeat alleles in a cohort of individuals with bipolar disorder. HTT CAG allele sizes from 2,229 Caucasian individuals diagnosed with DSM-IV bipolar disorder were compared to allele sizes in 1,828 control individuals from multiple cohorts. We found that HTT CAG repeat alleles > 35 units were observed in only one of 4,458 chromosomes from individuals with bipolar disorder, compared to three of 3,656 chromosomes from control subjects. These findings do not support an association between bipolar disorder and Huntington's disease. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  12. The Effect of Music Therapy in Patients with Huntington's Disease: A Randomized Controlled Trial.

    Science.gov (United States)

    van Bruggen-Rufi, Monique C H; Vink, Annemieke C; Wolterbeek, Ron; Achterberg, Wilco P; Roos, Raymund A C

    2017-01-01

    Music therapy may have beneficial effects on improving communication and expressive skills in patients with Huntington's disease (HD). Most studies are, however, small observational studies and methodologically limited. Therefore we conducted a multi-center randomized controlled trial. To determine the efficacy of music therapy in comparison with recreational therapy in improving quality of life of patients with advanced Huntington's disease by means of improving communication. Sixty-three HD-patients with a Total Functional Capacity (TFC) score of ≤7, admitted to four long-term care facilities in The Netherlands, were randomized to receive either group music therapy or group recreational therapy in 16 weekly sessions. They were assessed at baseline, after 8, 16 and 28 weeks using the Behaviour Observation Scale for Huntington (BOSH) and the Problem Behaviour Assessment-short version (PBA-s). A linear mixed model with repeated measures was used to compare the scores between the two groups. Group music therapy offered once weekly for 16 weeks to patients with Huntington's disease had no additional beneficial effect on communication or behavior compared to group recreational therapy. This was the first study to assess the effect of group music therapy on HD patients in the advanced stages of the disease. The beneficial effects of music therapy, recorded in many, mainly qualitative case reports and studies, could not be confirmed with the design (i.e. group therapy vs individual therapy) and outcome measures that have been used in the present study. A comprehensive process-evaluation alongside the present effect evaluation is therefore performed.

  13. Functional Compensation of Motor Function in Pre-Symptomatic Huntington's Disease

    Science.gov (United States)

    Kloppel, Stefan; Draganski, Bogdan; Siebner, Hartwig R.; Tabrizi, Sarah J.; Weiller, Cornelius; Frackowiak, Richard S. J.

    2009-01-01

    Involuntary choreiform movements are a clinical hallmark of Huntington's disease. Studies in clinically affected patients suggest a shift of motor activations to parietal cortices in response to progressive neurodegeneration. Here, we studied pre-symptomatic gene carriers to examine the compensatory mechanisms that underlie the phenomenon of…

  14. 29 CFR 1910.36 - Design and construction requirements for exit routes.

    Science.gov (United States)

    2010-07-01

    ... construction requirements for exit routes. (a) Basic requirements. Exit routes must meet the following design... your workplace, consult NFPA 101-2000, Life Safety Code. (c) Exit discharge. (1) Each exit discharge... route must be adequate. (1) Exit routes must support the maximum permitted occupant load for each floor...

  15. Challenges of Huntington's disease and quest for therapeutic biomarkers

    Czech Academy of Sciences Publication Activity Database

    Kotrčová, Eva; Jarkovská, Karla; Valeková, Ivona; Žižková, Martina; Motlík, Jan; Gadher, S. J.; Kovářová, Hana

    2015-01-01

    Roč. 9, 1-2 (2015), s. 147-158 ISSN 1862-8346 R&D Projects: GA MŠk ED2.1.00/03.0124; GA TA ČR(CZ) TA01011466 Institutional support: RVO:67985904 Keywords : HD biomarkers * Huntington´s disease * Huntingtin neurotoxicity * Huntingtin pathogenesis Subject RIV: FH - Neurology Impact factor: 2.959, year: 2015

  16. Vertraagde diagnose van de ziekte van Huntington in een psychiatrische setting

    NARCIS (Netherlands)

    Tak, L M; Sizoo, B; de Stegge, B M Aan; Adema, S; van Duijn, E; Kremer, B

    2017-01-01

    Huntington's disease (hd) is characterised by a triad of neuropsychiatric symptoms, motor disturbances and cognitive decline. If initial symptoms are of neuropsychiatric nature they maybe misinterpreted, which can lead to delayed diagnosis. Three examples of delayed hd diagnosis in a psychiatric

  17. Spatial signals link exit from mitosis to spindle position.

    Science.gov (United States)

    Falk, Jill Elaine; Tsuchiya, Dai; Verdaasdonk, Jolien; Lacefield, Soni; Bloom, Kerry; Amon, Angelika

    2016-05-11

    In budding yeast, if the spindle becomes mispositioned, cells prevent exit from mitosis by inhibiting the mitotic exit network (MEN). The MEN is a signaling cascade that localizes to spindle pole bodies (SPBs) and activates the phosphatase Cdc14. There are two competing models that explain MEN regulation by spindle position. In the 'zone model', exit from mitosis occurs when a MEN-bearing SPB enters the bud. The 'cMT-bud neck model' posits that cytoplasmic microtubule (cMT)-bud neck interactions prevent MEN activity. Here we find that 1) eliminating cMT- bud neck interactions does not trigger exit from mitosis and 2) loss of these interactions does not precede Cdc14 activation. Furthermore, using binucleate cells, we show that exit from mitosis occurs when one SPB enters the bud despite the presence of a mispositioned spindle. We conclude that exit from mitosis is triggered by a correctly positioned spindle rather than inhibited by improper spindle position.

  18. Survival End Points for Huntington Disease Trials Prior to a Motor Diagnosis.

    Science.gov (United States)

    Long, Jeffrey D; Mills, James A; Leavitt, Blair R; Durr, Alexandra; Roos, Raymund A; Stout, Julie C; Reilmann, Ralf; Landwehrmeyer, Bernhard; Gregory, Sarah; Scahill, Rachael I; Langbehn, Douglas R; Tabrizi, Sarah J

    2017-11-01

    Predictive genetic testing in Huntington disease (HD) enables therapeutic trials in HTT gene expansion mutation carriers prior to a motor diagnosis. Progression-free survival (PFS) is the composite of a motor diagnosis or a progression event, whichever comes first. To determine if PFS provides feasible sample sizes for trials with mutation carriers who have not yet received a motor diagnosis. This study uses data from the 2-phase, longitudinal cohort studies called Track and from a longitudinal cohort study called the Cooperative Huntington Observational Research Trial (COHORT). Track had 167 prediagnosis mutation carriers and 156 noncarriers, whereas COHORT had 366 prediagnosis mutation carriers and noncarriers. Track studies were conducted at 4 sites in 4 countries (Canada, France, England, and the Netherlands) from which data were collected from January 17, 2008, through November 17, 2014. The COHORT was conducted at 38 sites in 3 countries (Australia, Canada, and the United States) from which data were collected from February 14, 2006, through December 31, 2009. Results from the Track data were externally validated with data from the COHORT. The required sample size was estimated for a 2-arm prediagnosis clinical trial. Data analysis took place from May 1, 2016, to June 10, 2017. The primary end point is PFS. Huntington disease progression events are defined for the Unified Huntington's Disease Rating Scale total motor score, total functional capacity, symbol digit modalities test, and Stroop word test. Of Track's 167 prediagnosis mutation carriers, 93 (55.6%) were women, and the mean (SD) age was 40.06 (8.92) years; of the 156 noncarriers, 87 (55.7%) were women, and the mean (SD) age was 45.58 (10.30) years. Of the 366 COHORT participants, 229 (62.5%) were women and the mean (SD) age was 42.21 (12.48) years. The PFS curves of the Track mutation carriers showed good external validity with the COHORT mutation carriers after adjusting for initial progression. For

  19. Dissuasive exit signage for building fire evacuation.

    Science.gov (United States)

    Olander, Joakim; Ronchi, Enrico; Lovreglio, Ruggiero; Nilsson, Daniel

    2017-03-01

    This work presents the result of a questionnaire study which investigates the design of dissuasive emergency signage, i.e. signage conveying a message of not utilizing a specific exit door. The work analyses and tests a set of key features of dissuasive emergency signage using the Theory of Affordances. The variables having the largest impact on observer preference, interpretation and noticeability of the signage have been identified. Results show that features which clearly negate the exit-message of the original positive exit signage are most effective, for instance a red X-marking placed across the entirety of the exit signage conveys a clear dissuasive message. Other features of note are red flashing lights and alternation of colour. The sense of urgency conveyed by the sign is largely affected by sensory inputs such as red flashing lights or other features which cause the signs to break the tendencies of normalcy. Copyright © 2016 Elsevier Ltd. All rights reserved.

  20. First molecular modeling report on novel arylpyrimidine kynurenine monooxygenase inhibitors through multi-QSAR analysis against Huntington's disease: A proposal to chemists!

    Science.gov (United States)

    Amin, Sk Abdul; Adhikari, Nilanjan; Jha, Tarun; Gayen, Shovanlal

    2016-12-01

    Huntington's disease (HD) is caused by mutation of huntingtin protein (mHtt) leading to neuronal cell death. The mHtt induced toxicity can be rescued by inhibiting the kynurenine monooxygenase (KMO) enzyme. Therefore, KMO is a promising drug target to address the neurodegenerative disorders such as Huntington's diseases. Fiftysix arylpyrimidine KMO inhibitors are structurally explored through regression and classification based multi-QSAR modeling, pharmacophore mapping and molecular docking approaches. Moreover, ten new compounds are proposed and validated through the modeling that may be effective in accelerating Huntington's disease drug discovery efforts. Copyright © 2016 Elsevier Ltd. All rights reserved.

  1. Fast, controlled stepping drive for D2 filament ejection

    International Nuclear Information System (INIS)

    Amenda, W.; Lang, R.S.

    1985-01-01

    Centrifugal pellet injectors are required to refuel plasma machines. The pellet feed into the centrifuge should, if possible, be direct to keep the exit angle divergence small. The D 2 filaments used are first stored in a cryostat and then rapidly transported to the intake region of the centrifuge. An intermittent drive for fast, controlled ejection of D 2 filaments is described here. Mean filament speed of up to 0.6 m/s per step (1.2 mm) are achieved for the centrifugal pellet injector which refuels the ASDEX tokamak at Garching. The timing of the (81) step shifts can be synchronized with the rotor motion. The drive allows rates of up to 50 pellets per second. The drive method also seems to be suitable for direct feeding of other known centrifugal pellet injectors

  2. Huntington's Disease in a Patient Misdiagnosed as Conversion Disorder.

    Science.gov (United States)

    Nogueira, João Machado; Franco, Ana Margarida; Mendes, Susana; Valadas, Anabela; Semedo, Cristina; Jesus, Gustavo

    2018-01-01

    Huntington's disease (HD) is an inherited, progressive, and neurodegenerative neuropsychiatric disorder caused by the expansion of cytosine-adenine-guanine (CAG) trinucleotide in Interested Transcript (IT) 15 gene on chromosome 4. This pathology typically presents in individuals aged between 30 and 50 years and the age of onset is inversely correlated with the length of the CAG repeat expansion. It is characterized by chorea, cognitive deficits, and psychiatric symptoms. Usually the psychiatric disorders precede motor and cognitive impairment, Major Depressive Disorder and anxiety disorders being the most common presentations. We present a clinical case of a 65-year-old woman admitted to our Psychiatric Acute Unit. During the 6 years preceding the admission, the patient had clinical assessments made several times by different specialties that focused only on isolated symptoms, disregarding the syndrome as a whole. In the course of her last admission, the patient was referred to our Neuropsychiatric Team, which made the provisional diagnosis of late-onset Huntington's disease, later confirmed by genetic testing. This clinical vignette highlights the importance of a multidisciplinary approach to atypical clinical presentations and raises awareness for the relevance of investigating carefully motor symptoms in psychiatric patients.

  3. Exit interviews to reduce turnover amongst healthcare professionals.

    Science.gov (United States)

    Webster, Joan; Flint, Anndrea

    2014-08-19

    Exit interviews are widely used in healthcare organisations to identify reasons for staff attrition, yet their usefulness in limiting turnover is unclear. To determine the effectiveness of various exit interview strategies in decreasing turnover rates amongst healthcare professionals. We searched the Cochrane EPOC Group Specialised Register; Cochrane Central Register of Controlled Trials (CENTRAL), Issue 11, 2012; MEDLINE, Ovid (1950- ); EMBASE, Ovid (1947- ); CINAHL, EbscoHost (1980- ), and PsycINFO, OVID (1806-) between October 31 and November 6, 2012. We also screened the reference lists of included studies and relevant reviews; and searched trial registries for planned and on-going studies. We did not restrict searches by language or publication date. Randomised controlled trials, controlled clinical trials, controlled before-after studies and interrupted time series studies comparing turnover rates between healthcare professionals who had undergone one form of exit interview with another form of exit interview or with no interview. Two review authors independently assessed trial quality and extracted data. The original search identified 1560 citations, of which we considered 19 potentially relevant. The two authors independently reviewed the abstracts of these studies and retrieved the full texts of eight studies. We excluded all eight following independent assessment; they were either interviews, commentaries on how to do an exit interview or descriptive studies about reasons for leaving. We found no studies that matched our inclusion criteria. For this first update, we screened 2220 citations and identified no new studies. Evidence about the effectiveness of exit interviews to reduce turnover is currently not available. However, exit interviews may provide useful information about the work environment which, in turn, may be useful in the development of interventions to reduce turnover.

  4. Exit examinations, peer academic climate, and adolescents' developmental outcomes.

    Science.gov (United States)

    Benner, Aprile D

    2013-02-01

    Implications of high school exit examination performance were examined with a sample of 672 racial/ethnic minority students. Exit examination failure in the 10th grade was negatively linked to subsequent grade point average, school engagement, and school belonging one year later, controlling for outcomes prior to taking the examination. Academically incongruent students-those who failed the exit examination but were in schools where their same-race/ethnicity peers were performing well academically-seemed to be at particular risk for struggling grades and poorer socioemotional well-being (e.g., experiencing greater depressive symptoms and loneliness). Findings contribute to the limited research base on exit examinations and highlight the links between exit examination performance and developmental outcomes beyond the oft-studied academic domain. Copyright © 2012 Society for the Study of School Psychology. Published by Elsevier Ltd. All rights reserved.

  5. Personality Traits in Huntington's Disease

    DEFF Research Database (Denmark)

    Larsen, Ida Unmack; Mortensen, Erik Lykke; Vinther-Jensen, Tua

    2016-01-01

    Huntington's disease (HD) is associated with risk for developing psychiatric symptoms. Vulnerability or resilience to psychiatric symptoms may be associated with personality traits. This exploratory study, aimed to investigate personality traits in a large cohort of HD carriers and at risk gene......-expansion negative individuals (HD non-carriers), exploring whether carrying the HD gene or growing up in an HD family influences personality traits. Forty-seven HD carriers, Thirty-nine HD non-carriers, and 121 healthy controls answered the Danish version of the revised NEO personality inventory. Comparisons...... symptoms. Our findings suggest that, there is no direct effect of the HD gene on personality traits, but that personality assessment may be relevant to use when identifying individuals from HD families who are vulnerable to develop psychiatric symptoms....

  6. Current status of PET imaging in Huntington's disease

    International Nuclear Information System (INIS)

    Pagano, Gennaro; Niccolini, Flavia; Politis, Marios

    2016-01-01

    To review the developments of recent decades and the current status of PET molecular imaging in Huntington's disease (HD). A systematic review of PET studies in HD was performed. The MEDLINE, Web of Science, Cochrane and Scopus databases were searched for articles in all languages published up to 19 August 2015 using the major medical subject heading ''Huntington Disease'' combined with text and key words ''Huntington Disease'', ''Neuroimaging'' and ''PET''. Only peer-reviewed, primary research studies in HD patients and premanifest HD carriers, and studies in which clinical features were described in association with PET neuroimaging results, were included in this review. Reviews, case reports and nonhuman studies were excluded. A total of 54 PET studies were identified and analysed in this review. Brain metabolism ([ 18 F]FDG and [ 15 O]H 2 O), presynaptic ([ 18 F]fluorodopa, [ 11 C]β-CIT and [ 11 C]DTBZ) and postsynaptic ([ 11 C]SCH22390, [ 11 C]FLB457 and [ 11 C]raclopride) dopaminergic function, phosphodiesterases ([ 18 F]JNJ42259152, [ 18 F]MNI-659 and [ 11 C]IMA107), and adenosine ([ 18 F]CPFPX), cannabinoid ([ 18 F]MK-9470), opioid ([ 11 C]diprenorphine) and GABA ([ 11 C]flumazenil) receptors were evaluated as potential biomarkers for monitoring disease progression and for assessing the development and efficacy of novel disease-modifying drugs in premanifest HD carriers and HD patients. PET studies evaluating brain restoration and neuroprotection were also identified and described in detail. Brain metabolism, postsynaptic dopaminergic function and phosphodiesterase 10A levels were proven to be powerful in assessing disease progression. However, no single technique may be currently considered an optimal biomarker and an integrative multimodal imaging approach combining different techniques should be developed for monitoring potential neuroprotective and preventive treatment

  7. 3-NP-induced neurodegeneration studies in experimental models of Huntington's disease.

    NARCIS (Netherlands)

    Vis, J.C.

    2005-01-01

    This thesis investigates the possible role of apoptosis, or programmed cell death, in Huntington's disease (HD). HD is caused by an expanded CAG repeat in the N-terminal region of the huntingtin protein leading to specific neostriatal neurodegeneration. The sequence of events that leads to this

  8. Electronic Transport in Single-Stranded DNA Molecule Related to Huntington's Disease

    Science.gov (United States)

    Sarmento, R. G.; Silva, R. N. O.; Madeira, M. P.; Frazão, N. F.; Sousa, J. O.; Macedo-Filho, A.

    2018-04-01

    We report a numerical analysis of the electronic transport in single chain DNA molecule consisting of 182 nucleotides. The DNA chains studied were extracted from a segment of the human chromosome 4p16.3, which were modified by expansion of CAG (cytosine-adenine-guanine) triplet repeats to mimics Huntington's disease. The mutated DNA chains were connected between two platinum electrodes to analyze the relationship between charge propagation in the molecule and Huntington's disease. The computations were performed within a tight-binding model, together with a transfer matrix technique, to investigate the current-voltage (I-V) of 23 types of DNA sequence and compare them with the distributions of the related CAG repeat numbers with the disease. All DNA sequences studied have a characteristic behavior of a semiconductor. In addition, the results showed a direct correlation between the current-voltage curves and the distributions of the CAG repeat numbers, suggesting possible applications in the development of DNA-based biosensors for molecular diagnostics.

  9. Huntington's disease does not appear to increase the risk of diabetes mellitus

    DEFF Research Database (Denmark)

    Boesgaard, T W; Nielsen, Troels Tolstrup; Josefsen, Knud Elnegaard

    2009-01-01

    Huntington's disease (HD) is an autosomal, dominantly inherited, neurodegenerative disorder characterised by neurological, cognitive and psychiatric symptoms. HD has been associated with diabetes mellitus, which is, to some extent, supported by studies in transgenic HD mice. In transgenic mice...

  10. Is the publication of exit poll results morally permissible?

    DEFF Research Database (Denmark)

    Sønderholm, Jørn

    2016-01-01

    This article is about exit polls. It addresses the question of whether or not it is morally permissible to publish exit poll results. The conclusion of the article is that an affirmative answer should be given to this question. In section 2, the master argument in favor of the moral permissibility...... of the publication of exit poll results is introduced. This is a strong argument. It is, however, argued that it might be the case that the conclusion of this argument should be rejected if there are other, and weightier, arguments against the idea that the publication of exit poll results is morally permissible....... In section 3, the strongest arguments against the moral permissibility of the publication of exit poll results are outlined and discussed. The conclusion of this section is that all these arguments fail in their intended purpose. The conclusion of the article is therefore justified....

  11. IPO as an Exit startegy in Management Buyouts

    OpenAIRE

    Sheth, Vidhi Chetan

    2008-01-01

    The basic subject to this research project is,IPO as an exit strategy in management buyouts. The paper provides with an understanding of the various characteristics and factors that have an impact on a buyout exit through an IPO. Discussions on the issues like the IPO versus other exit strategies, performance of a company's pre-IPO as well as post-IPO, the trends in the buyout and the IPO market, etc are done. For a better understanding and an in-depth knowledge about the topic, we have analy...

  12. 77 FR 22616 - Huntington Asset Advisors, Inc., et al.; Notice of Application

    Science.gov (United States)

    2012-04-16

    ...''), Huntington Strategy Shares (``Trust''), and SEI Investments Distribution Co. Summary of Application.... \\14\\ If Shares are listed on The NASDAQ Stock Market LLC (``Nasdaq'') or a similar electronic Listing... SECURITIES AND EXCHANGE COMMISSION [Investment Company Act Release No. 30032; 812-13785...

  13. Selected CSF biomarkers indicate no evidence of early neuroinflammation in Huntington disease

    DEFF Research Database (Denmark)

    Vinther-Jensen, Tua; Börnsen, Lars Svend; Budtz-Jorgensen, Esben

    2016-01-01

    Objective: To investigate CSF biomarkers of neuroinflammation and neurodegeneration in Huntington disease (HD) gene-expansion carriers compared to controls and to investigate these biomarkers in association with clinical HD rating scales and disease burden score. Methods: We collected CSF from 32...

  14. Exit by Afghanisation

    DEFF Research Database (Denmark)

    Holmberg, Hasse

    USA’s exit-strategi fra Afghanistan har båret præg af et italesat hovedmål om overdragelse af ansvar til de nationale myndigheder i landet. Exit-strategien udmærker sig ved sin lighed med USA’s afvikling af sit engagement i Vietnam for snart et halvt århundrede siden, hvor begrebet Vietnamisation...... om national selvbestemmelse. Den amerikanske opinion spillede en afgørende rolle og afslutningen af engagementet i Vietnam kan i lige så høj grad ses som en reaktion på den massive folkelige modstand mod krigen. Omtrent 40 år efter kan Obama-administrationen notere sig en lignende negativ trend i...... Kissinger vidste, at krigen i Vietnam var tabt. Vietnamisation havde i høj grad til formål at slutte USA’s engagement på en måde, der tog hensyn til USA’s internationale renomme. Spørgsmålet er så om de mange strategiske ligheder kan overføres til samme konklusion: krigen er tabt. Briefet har til hensigt...

  15. Motor outcome measures in Huntington disease clinical trials.

    Science.gov (United States)

    Reilmann, Ralf; Schubert, Robin

    2017-01-01

    Deficits in motor function are a hallmark of Huntington disease (HD). The Unified Huntington's Disease Rating Scale Total Motor Score (UHDRS-TMS) is a categoric clinical rating scale assessing multiple domains of motor disability in HD. The UHDRS-TMS or subsets of its items have served as primary or secondary endpoints in numerous clinical trials. In spite of a well-established video-based annual online certification system, intra- and interrater variability, subjective error, and rater-induced placebo effects remain a concern. In addition, the UHDRS-TMS was designed to primarily assess motor symptoms in manifest HD. Recently, advancement of technology resulted in the introduction of the objective Q-Motor (i.e., Quantitative-Motor) assessments in biomarker studies and clinical trials in HD. Q-Motor measures detected motor signs in blinded cross-sectional and longitudinal analyses of manifest, prodromal, and premanifest HD cohorts up to two decades before clinical diagnosis. In a multicenter clinical trial in HD, Q-Motor measures were more sensitive than the UHDRS-TMS and exhibited no placebo effects. Thus, Q-Motor measures are currently explored in several multicenter trials targeting both symptomatic and disease-modifying mechanisms. They may supplement the UHDRS-TMS, increase the sensitivity and reliability in proof-of-concept studies, and open the door for phenotype assessments in clinical trials in prodromal and premanifest HD. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. Motivating signage prompts safety belt use among drivers exiting senior communities.

    Science.gov (United States)

    Cox, B S; Cox, A B; Cox, D J

    2000-01-01

    Senior drivers are vulnerable to automobile crashes and subsequent injury and death. Safety belts reduce health risks associated with auto crashes. Therefore, it is important to encourage senior drivers to wear safety belts while driving. Using an AB design, replicated five times, we evaluated the short- and long-term effects of a sign with the message "BUCKLE UP, STAY SAFE" attached to a stop sign at the exits of five different senior communities. Safety belt use was stable during two pretreatment assessments averaged across the five sites and 250 drivers (72% and 68% usage), but significantly increased following installation of these signs (94% usage). Six months after installation of the signs, the effect persisted (88% usage). Use of such signs may be a cost-effective way of promoting safety belt use.

  17. Entry and exit decisions under uncertainty

    DEFF Research Database (Denmark)

    Kongsted, Hans Christian

    1996-01-01

    This paper establishes the general deterministic limit that corresponds to Dixit's model of entry and exit decisions under uncertainty. The interlinked nature of decisions is shown to be essential also in the deterministic limit. A numerical example illustrates the result......This paper establishes the general deterministic limit that corresponds to Dixit's model of entry and exit decisions under uncertainty. The interlinked nature of decisions is shown to be essential also in the deterministic limit. A numerical example illustrates the result...

  18. Huntington's disease: a clinical review

    Directory of Open Access Journals (Sweden)

    Roos Raymund AC

    2010-12-01

    Full Text Available Abstract Huntington disease (HD is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia. Prevalence in the Caucasian population is estimated at 1/10,000-1/20,000. Mean age at onset of symptoms is 30-50 years. In some cases symptoms start before the age of 20 years with behavior disturbances and learning difficulties at school (Juvenile Huntington's disease; JHD. The classic sign is chorea that gradually spreads to all muscles. All psychomotor processes become severely retarded. Patients experience psychiatric symptoms and cognitive decline. HD is an autosomal dominant inherited disease caused by an elongated CAG repeat (36 repeats or more on the short arm of chromosome 4p16.3 in the Huntingtine gene. The longer the CAG repeat, the earlier the onset of disease. In cases of JHD the repeat often exceeds 55. Diagnosis is based on clinical symptoms and signs in an individual with a parent with proven HD, and is confirmed by DNA determination. Pre-manifest diagnosis should only be performed by multidisciplinary teams in healthy at-risk adult individuals who want to know whether they carry the mutation or not. Differential diagnoses include other causes of chorea including general internal disorders or iatrogenic disorders. Phenocopies (clinically diagnosed cases of HD without the genetic mutation are observed. Prenatal diagnosis is possible by chorionic villus sampling or amniocentesis. Preimplantation diagnosis with in vitro fertilization is offered in several countries. There is no cure. Management should be multidisciplinary and is based on treating symptoms with a view to improving quality of life. Chorea is treated with dopamine receptor blocking or depleting agents. Medication and non-medical care for depression and aggressive behavior may be required. The progression of the disease leads to a complete dependency in daily life, which

  19. Jet Exit Rig Six Component Force Balance

    Science.gov (United States)

    Castner, Raymond; Wolter, John; Woike, Mark; Booth, Dennis

    2012-01-01

    A new six axis air balance was delivered to the NASA Glenn Research Center. This air balance has an axial force capability of 800 pounds, primary airflow of 10 pounds per second, and a secondary airflow of 3 pounds per second. Its primary use was for the NASA Glenn Jet Exit Rig, a wind tunnel model used to test both low-speed, and high-speed nozzle concepts in a wind tunnel. This report outlines the installation of the balance in the Jet Exit Rig, and the results from an ASME calibration nozzle with an exit area of 8 square-inches. The results demonstrated the stability of the force balance for axial measurements and the repeatability of measurements better than 0.20 percent.

  20. Criticality safety analysis of a calciner exit chute

    International Nuclear Information System (INIS)

    Haught, C.F.; Basoglu, B.; Brewer, R.W.; Hollenback, D.F.; Wilkinson, A.D.; Dodds, H.L.

    1994-01-01

    Calcination of uranyl nitrate into uranium oxide is part of normal operations of some enrichment plants. Typically, a calciner discharges uranium oxide powder (U 3 O 8 ) into an exit chute that directs the powder into a receiving can located in a glove box. One possible scenario for a criticality accident is the exit chute becoming blocked with powder near its discharge. The blockage restricts the flow of powder causing the exit chute to become filled with the powder. If blockage does occur, the height of the powder could reach a level that would not be safe from a criticality point of view. In this analysis, the subcritical height limit is examined for 98% enriched U 3 O 8 in the exit chute with full water reflection and optimal water moderation. The height limit for ensuring criticality safety during such an accumulation is 28.2 cm above the top of the discharge pipe at the bottom of the chute. Chute design variations are also evaluated with full water reflection and optimal water moderation. Subcritical configurations for the exit chute variation are developed, but the configurations are not safe when combined with the calciner. To ensure criticality safety, modifications must be made to the calciner tube or safety measures must be implemented if these designs are to be utilized with 98% enriched material. A geometrically safe configuration for the exit chute is developed for a blockage of 20% enriched powder with full water reflection and optimal water moderation, and this configuration is safe when combined with the existing calciner

  1. Huntington disease: Experimental models and therapeutic perspectives

    International Nuclear Information System (INIS)

    Serrano Sanchez, Teresa; Blanco Lezcano, Lisette; Garcia Minet, Rocio; Alberti Amador, Esteban; Diaz Armesto, Ivan and others

    2011-01-01

    Huntington's disease (HD) is a degenerative dysfunction of hereditary origin. Up to date there is not, an effective treatment to the disease which having lapsed 15 or 20 years advances inexorably, in a slow form, toward the total inability or death. This paper reviews the clinical and morphological characteristics of Huntington's disease as well as the experimental models more commonly used to study this disease, having as source the articles indexed in Medline data base, published in the last 20 years. Advantages and disadvantages of all experimental models to reproduce the disease as well as the perspectives to therapeutic assay have been also considered. the consent of outline reported about the toxic models, those induced by neurotoxins such as quinolinic acid, appears to be the most appropriate to reproduce the neuropathologic characteristic of the disease, an genetic models contributing with more evidence to the knowledge of the disease etiology. Numerous treatments ameliorate clinical manifestations, but none of them has been able to stop or diminish the affectations derived from neuronal loss. At present time it is possible to reproduce, at least partially, the characteristics of the disease in experimentation animals that allow therapy evaluation in HD. from the treatment view point, the more promissory seems to be transplantation of no neuronal cells, taking into account ethical issues and factibility. On the other hand the new technology of interference RNA emerges as a potential therapeutic tool for treatment in HD, and to respond basic questions on the development of the disease.

  2. Apolipoprotein E and presenilin-1 genotypes in Huntington's disease.

    Science.gov (United States)

    Panas, M; Avramopoulos, D; Karadima, G; Petersen, M B; Vassilopoulos, D

    1999-07-01

    Huntington's disease (HD) is an autosomal dominant degenerative disease of the central nervous system manifested by involuntary movements (chorea), psychiatric manifestations, and cognitive impairment with a variable age at onset. This variability is mainly attributed to genetic factors. The so-called aging genes [e.g., those for apolipoprotein E (APOE) and presenilin-1 (PS-1) have been implicated in determining the age at onset of Alzheimer's disease, a disease sharing common clinical features with HD. In 60 unrelated patients suffering from HD (mean age at onset 40.1 years, range 20-65) we determined number of CAG repeats and the distribution of the APOE alleles (epsilon2, epsilon3, epsilon4) and PS-1 alleles. The results showed that: (a) The age at onset was higher in the group of patients with the epsilon4 allele (51.6 vs. 38.0 P<0.002), (b) The correlation between the age at onset and the number of CAG repeats was strong in patients with the epsilon3/epsilon3 genotype while it was not detected in patients with epsilon3/epsilon4 genotype. (c) No correlation was found between age at onset and PS-1 alleles. In conclusion, APOE seems to be a significant factor influencing the age at onset of Huntington's disease.

  3. Striatal dopamine D2 receptors, metabolism, and volume in preclinical Huntington disease

    NARCIS (Netherlands)

    van Oostrom, JCH; Maguire, RP; Verschuuren-Bemelmans, CC; van der Duin, LV; Pruim, J; Roos, RAC; Leenders, KL

    2005-01-01

    Among 27 preclinical carriers of the Huntington disease mutation (PMC), the authors found normal striatal values for MRI volumetry in 88% and for fluorodesoxyglucose PET metabolic index in 67%. Raclopride PET binding potential (RAC-BP) was decreased in 50% and correlated with increases in the

  4. 24 CFR 3280.106 - Exit facilities; egress windows and devices.

    Science.gov (United States)

    2010-04-01

    ... 24 Housing and Urban Development 5 2010-04-01 2010-04-01 false Exit facilities; egress windows and... § 3280.106 Exit facilities; egress windows and devices. (a) Every room designed expressly for sleeping purposes, unless it has an exit door (see § 3280.105), shall have at least one outside window or approved...

  5. An Identity Theory of Role Exit among Soccer Referees

    OpenAIRE

    Milne, Jason Syme

    2006-01-01

    This study examines how identity processes affect role exit. I test a model of role exit that situates the identity processes of cognitive processes (reflected appraisals and social comparisons), rewards and costs related to the role, commitment to the role, and identity centrality as mediating factors between role-set and social characteristic background factors, and role exit. Using a sample of 940 current and former soccer referees in Virginia and the District of Columbia, the results s...

  6. Short G-rich oligonucleotides as a potential therapeutic for Huntington's Disease

    Directory of Open Access Journals (Sweden)

    Parekh-Olmedo Hetal

    2006-10-01

    Full Text Available Abstract Background Huntington's Disease (HD is an inherited autosomal dominant genetic disorder in which neuronal tissue degenerates. The pathogenesis of the disease appears to center on the development of protein aggregates that arise initially from the misfolding of the mutant HD protein. Mutant huntingtin (Htt is produced by HD genes that contain an increased number of glutamine codons within the first exon and this expansion leads to the production of a protein that misfolds. Recent studies suggest that mutant Htt can nucleate protein aggregation and interfere with a multitude of normal cellular functions. Results As such, efforts to find a therapy for HD have focused on agents that disrupt or block the mutant Htt aggregation pathway. Here, we report that short guanosine monotonic oligonucleotides capable of adopting a G-quartet structure, are effective inhibitors of aggregation. By utilizing a biochemical/immunoblotting assay as an initial screen, we identified a 20-mer, all G-oligonucleotide (HDG as an active molecule. Subsequent testing in a cell-based assay revealed that HDG was an effective inhibitor of aggregation of a fusion protein, comprised of a mutant Htt fragment and green fluorescent protein (eGFP. Taken together, our results suggest that a monotonic G-oligonucleotide, capable of adopting a G-quartet conformation is an effective inhibitor of aggregation. This oligonucleotide can also enable cell survival in PC12 cells overexpressing a mutant Htt fragment fusion gene. Conclusion Single-stranded DNA oligonucleotides capable of forming stable G-quartets can inhibit aggregation of the mutant Htt fragment protein. This activity maybe an important part of the pathogenecity of Huntington's Disease. Our results reveal a new class of agents that could be developed as a therapeutic approach for Huntington's Disease.

  7. Continuous deep sedation, physician-assisted suicide, and euthanasia in Huntington's disorder.

    Science.gov (United States)

    Lindblad, Anna; Juth, Niklas; Fürst, Carl Johan; Lynöe, Niels

    2010-11-01

    To investigate the attitudes among Swedish physicians and the general public towards continuous deep sedation (CDS) as an alternative treatment for a competent, not imminently dying patient with Huntington's disorder requesting physician-assisted suicide (PAS) and euthanasia. A questionnaire was distributed to 1200 physicians in Sweden and 1201 individuals in Stockholm. It consisted of three parts: 1) A vignette about a competent patient with Huntington's disease requesting PAS. When no longer competent, relatives request euthanasia on behalf of the patient. Responders were asked about their attitudes towards these requests and whether CDS would be an acceptable alternative. 2) General questions about PAS and euthanasia. 3) Background variables. The response rate was 56% (physicians) and 52% (general public). The majority of the general public and a fairly large proportion of physicians reported more liberal views on CDS than are expressed in current Swedish and international recommendations. In light of the results, we suggest that there is a need for a broader discussion about the recommendations for CDS, with a special focus on the needs of patients with progressive neurodegenerative disorders.

  8. Adenyl cyclase activator forskolin protects against Huntington's disease-like neurodegenerative disorders

    Directory of Open Access Journals (Sweden)

    Sidharth Mehan

    2017-01-01

    Full Text Available Long term suppression of succinate dehydrogenase by selective inhibitor 3-nitropropionic acid has been used in rodents to model Huntington's disease where mitochondrial dysfunction and oxidative damages are primary pathological hallmarks for neuronal damage. Improvements in learning and memory abilities, recovery of energy levels, and reduction of excitotoxicity damage can be achieved through activation of Adenyl cyclase enzyme by a specific phytochemical forskolin. In this study, intraperitoneal administration of 10 mg/kg 3-nitropropionic acid for 15 days in rats notably reduced body weight, worsened motor cocordination (grip strength, beam crossing task, locomotor activity, resulted in learning and memory deficits, greatly increased acetylcholinesterase, lactate dehydrogenase, nitrite, and malondialdehyde levels, obviously decreased adenosine triphosphate, succinate dehydrogenase, superoxide dismutase, catalase, and reduced glutathione levels in the striatum, cortex and hippocampus. Intragastric administration of forskolin at 10, 20, 30 mg/kg dose-dependently reversed these behavioral, biochemical and pathological changes caused by 3-nitropropionic acid. These results suggest that forskolin exhibits neuroprotective effects on 3-nitropropionic acid-induced Huntington's disease-like neurodegeneration.

  9. Double strand DNA breaks response in Huntington´s disease

    Czech Academy of Sciences Publication Activity Database

    Šolc, Petr; Valášek, Jan; Rausová, Petra; Juhásová, Jana; Juhás, Štefan; Motlík, Jan

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 15-15 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) 7F14308 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * DNA damage * double strand DNA breaks Subject RIV: FH - Neurology

  10. The BACHD Rat Model of Huntington Disease Shows Signs of Fronto-Striatal Dysfunction in Two Operant Conditioning Tests of Short-Term Memory.

    Directory of Open Access Journals (Sweden)

    Erik Karl Håkan Clemensson

    Full Text Available The BACHD rat is a recently developed transgenic animal model of Huntington disease, a progressive neurodegenerative disorder characterized by extensive loss of striatal neurons. Cognitive impairments are common among patients, and characterization of similar deficits in animal models of the disease is therefore of interest. The present study assessed the BACHD rats' performance in the delayed alternation and the delayed non-matching to position test, two Skinner box-based tests of short-term memory function. The transgenic rats showed impaired performance in both tests, indicating general problems with handling basic aspects of the tests, while short-term memory appeared to be intact. Similar phenotypes have been found in rats with fronto-striatal lesions, suggesting that Huntington disease-related neuropathology might be present in the BACHD rats. Further analyses indicated that the performance deficit in the delayed alternation test might be due to impaired inhibitory control, which has also been implicated in Huntington disease patients. The study ultimately suggests that the BACHD rats might suffer from neuropathology and cognitive impairments reminiscent of those of Huntington disease patients.

  11. The BACHD Rat Model of Huntington Disease Shows Signs of Fronto-Striatal Dysfunction in Two Operant Conditioning Tests of Short-Term Memory.

    Science.gov (United States)

    Clemensson, Erik Karl Håkan; Clemensson, Laura Emily; Riess, Olaf; Nguyen, Huu Phuc

    2017-01-01

    The BACHD rat is a recently developed transgenic animal model of Huntington disease, a progressive neurodegenerative disorder characterized by extensive loss of striatal neurons. Cognitive impairments are common among patients, and characterization of similar deficits in animal models of the disease is therefore of interest. The present study assessed the BACHD rats' performance in the delayed alternation and the delayed non-matching to position test, two Skinner box-based tests of short-term memory function. The transgenic rats showed impaired performance in both tests, indicating general problems with handling basic aspects of the tests, while short-term memory appeared to be intact. Similar phenotypes have been found in rats with fronto-striatal lesions, suggesting that Huntington disease-related neuropathology might be present in the BACHD rats. Further analyses indicated that the performance deficit in the delayed alternation test might be due to impaired inhibitory control, which has also been implicated in Huntington disease patients. The study ultimately suggests that the BACHD rats might suffer from neuropathology and cognitive impairments reminiscent of those of Huntington disease patients.

  12. Current status of PET imaging in Huntington's disease

    Energy Technology Data Exchange (ETDEWEB)

    Pagano, Gennaro; Niccolini, Flavia; Politis, Marios [King' s College London, Neurodegeneration Imaging Group, Department of Basic and Clinical Neuroscience, Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), Camberwell, London (United Kingdom)

    2016-06-15

    To review the developments of recent decades and the current status of PET molecular imaging in Huntington's disease (HD). A systematic review of PET studies in HD was performed. The MEDLINE, Web of Science, Cochrane and Scopus databases were searched for articles in all languages published up to 19 August 2015 using the major medical subject heading ''Huntington Disease'' combined with text and key words ''Huntington Disease'', ''Neuroimaging'' and ''PET''. Only peer-reviewed, primary research studies in HD patients and premanifest HD carriers, and studies in which clinical features were described in association with PET neuroimaging results, were included in this review. Reviews, case reports and nonhuman studies were excluded. A total of 54 PET studies were identified and analysed in this review. Brain metabolism ([{sup 18}F]FDG and [{sup 15}O]H{sub 2}O), presynaptic ([{sup 18}F]fluorodopa, [{sup 11}C]β-CIT and [{sup 11}C]DTBZ) and postsynaptic ([{sup 11}C]SCH22390, [{sup 11}C]FLB457 and [{sup 11}C]raclopride) dopaminergic function, phosphodiesterases ([{sup 18}F]JNJ42259152, [{sup 18}F]MNI-659 and [{sup 11}C]IMA107), and adenosine ([{sup 18}F]CPFPX), cannabinoid ([{sup 18}F]MK-9470), opioid ([{sup 11}C]diprenorphine) and GABA ([{sup 11}C]flumazenil) receptors were evaluated as potential biomarkers for monitoring disease progression and for assessing the development and efficacy of novel disease-modifying drugs in premanifest HD carriers and HD patients. PET studies evaluating brain restoration and neuroprotection were also identified and described in detail. Brain metabolism, postsynaptic dopaminergic function and phosphodiesterase 10A levels were proven to be powerful in assessing disease progression. However, no single technique may be currently considered an optimal biomarker and an integrative multimodal imaging approach combining different techniques should be developed

  13. Exit channels of autoionization resonances in atoms

    International Nuclear Information System (INIS)

    Krause, M.O.

    1985-01-01

    In many-electron atoms with open shells strong autoionization resonances occur when an electron from an inner, weakly bound subshell is excited. Usually, the resonance state lies above several ionization thresholds and, hence, will decay into more than one exit or continuum channel. Several cases are discussed in which the resonance state is induced by synchrotron radiation, and the exit channels are differentiated and characterized by the analysis of the ejected electrons

  14. Transgenic miniature pig as a model for the study of Huntington´s Disease

    Czech Academy of Sciences Publication Activity Database

    Baxa, Monika

    2012-01-01

    Roč. 22, č. 2 (2012), s. 23-25 ISSN 1210-1737 Institutional support: RVO:67985904 Keywords : transgenic pig * Huntington ´s disease * large animal model * neurodegenerative disease Subject RIV: EB - Genetics ; Molecular Biology

  15. 4p16.3 haplotype modifying age at onset of Huntington disease

    DEFF Research Database (Denmark)

    Nørremølle, A; Budtz-Jørgensen, E; Fenger, K

    2009-01-01

    Huntington disease (HD) is caused by an expanded CAG repeat sequence in the HD gene. Although the age at onset is correlated to the CAG repeat length, this correlation only explains approximately half of the variation in onset age. Less variation between siblings indicates that the variation is, ...

  16. Control of the mitotic exit network during meiosis

    Science.gov (United States)

    Attner, Michelle A.; Amon, Angelika

    2012-01-01

    The mitotic exit network (MEN) is an essential GTPase signaling pathway that triggers exit from mitosis in budding yeast. We show here that during meiosis, the MEN is dispensable for exit from meiosis I but contributes to the timely exit from meiosis II. Consistent with a role for the MEN during meiosis II, we find that the signaling pathway is active only during meiosis II. Our analysis further shows that MEN signaling is modulated during meiosis in several key ways. Whereas binding of MEN components to spindle pole bodies (SPBs) is necessary for MEN signaling during mitosis, during meiosis MEN signaling occurs off SPBs and does not require the SPB recruitment factor Nud1. Furthermore, unlike during mitosis, MEN signaling is controlled through the regulated interaction between the MEN kinase Dbf20 and its activating subunit Mob1. Our data lead to the conclusion that a pathway essential for vegetative growth is largely dispensable for the specialized meiotic divisions and provide insights into how cell cycle regulatory pathways are modulated to accommodate different modes of cell division. PMID:22718910

  17. Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas

    Directory of Open Access Journals (Sweden)

    GILBERTO LEVY

    1999-09-01

    Full Text Available Early-onset Huntington's disease (HD occurs in approximately 10% of HD's cases. We report juvenile HD in phenotypically identical twins, evaluated by history, clinical and neurologic examination, mini-mental state examination, blood laboratory exams, cerebrospinal fluid examination, skull computed tomography, and genetic examination for HD. Patients had the akinetic-rigid variety (Westphal variant of the disease and paternal inheritance. The laboratory workup confirmed the clinical diagnosis of HD, which adds this report to the rare cases of HD in twins reported in the literature.Doença de Huntington (DH de início precoce ocorre em aproximadamente 10% dos casos de DH. Relatamos DH juvenil em gêmeas fenotipicamente idênticas, avaliadas por história, exames clínico e neurológico, mini-exame do estado mental, exames de sangue, exame do líquido cefalorraquidiano, tomografia computadorizada de crânio e exame genético para DH. As pacientes apresentavam a variedade rígido-acinética (variante de Westphal da doença e herança paterna. A avaliação laboratorial confirmou o diagnóstico clínico de DH, acrescentando-se este relato aos raros casos de DH em gêmeos relatados na literatura.

  18. Turbine-missile casing exit tests

    International Nuclear Information System (INIS)

    Yoshimura, H.R.; Sliter, G.E.

    1978-01-01

    Nuclear power plant designers are required to provide safety-related components with adequate protection against hypothetical turbine-missile impacts. In plants with a ''peninsula'' arrangement, protection is provided by installing the turbine axis radially from the reactor building, so that potential missile trajectories are not in line with the plant. In plants with a ''non-peninsula'' arrangement (turbine axis perpendicular to a radius), designers rely on the low probability of a missile strike and on the protection provided by reinforced concrete walls in order to demonstrate an adequate level of protection USNRC Regulatory Guide 1.115). One of the critical first steps in demonstrating adequacy is the determination of the energy and spin of the turbine segments as they exit the turbine casing. The spin increases the probability that a subsequent impact with a protective barrier will be off-normal and therefore less severe than the normal impact assumed in plant designs. Two full-scale turbine-missile casing exit tests which were conducted by Sandia Laboratories at their rocket-sled facility in Albuquerque, New Mexico, are described. Because of wide variations in turbine design details, postulated failure conditions, and missile exit scenarios, the conditions for the two tests were carefully selected to be as prototypical as possible, while still maintaining the well-controlled and well-characterized test conditions needed for generating benchmark data

  19. Entry and Exit.

    Science.gov (United States)

    1987-03-01

    1. Introduction R Analyses of industrial competition have attained a new vigor with the application of game -theoretic methods. The process of... competition is represented in models that reflect genuine struggles for entry, market power, and continuing survival. Dynamics and informational effects are...presents a few of the models developed recently to study competitive processes that affect a firm’s entry into a market , and the decision to exit. The

  20. Exiting and Returning to the Parental Home for Boomerang Kids.

    Science.gov (United States)

    Sandberg-Thoma, Sara E; Snyder, Anastasia R; Jang, Bohyun Joy

    2015-06-01

    Young adults commonly exit from and return to the parental home, yet few studies have examined the motivation behind these exits and returns using a life course framework. Using data from the National Longitudinal Survey of Youth 1997, the authors examined associations between mental health problems and economic characteristics and exits from (n = 8,162), and returns to (n = 6,530), the parental home during the transition to adulthood. The average age of the respondents was 24 years. The authors found evidence that mental health and economic characteristics were related to home leaving and returning. Emotional distress was associated with earlier exits from, and returns to, the parental home; alcohol problems were associated with earlier returns to the parental home. The findings regarding economic resources were unexpectedly mixed. Greater economic resources were linked to delayed exits from, and earlier returns to, the parental home. The implications of these findings for young adults are discussed.

  1. We can predict when driving is no longer safe for people who have HD using standard neuropsychological measures.

    Science.gov (United States)

    Hennig, Bonnie L; Kaplan, Richard F; Nowicki, Ariel E; Barclay, Jessy E; Gertsberg, Anna G

    2014-01-01

    Early cognitive dysfunction in Huntington's Disease (HD) is typically of a subcortical frontal executive type, with bradyphrenia, poor spatial and working memory, poor planning and organization, a lack of judgment, and poor mental flexibility. Although there is literature suggesting a correlation between deficits in speed of processing, working memory and executive function on driving competency, there is little direct evidence comparing these declines on tests to actual driving skills. The current study examines the utility of specific neuropsychological measures in predicting actual driving competency in patients with HD. Fifty-two patients at the UConn Health HD Program underwent yearly neuropsychological evaluations and were included in this study. Four scales were chosen a priori to predict driving impairment because of their reported relationship to driving ability. Within each test category, subjects who scored below the threshold suggestive of neurological impairment were found to have results within the impaired range (1.5 standard deviations below corrective normative data). A referral to the Connecticut Department of Motor Vehicles (DMV) for a driving evaluation was subsequently made on patients who were found impaired on any two of these tests. The authors found a strong relationship between scores on a simple battery of four neuropsychological tests and driving competency. This short battery may prove of pragmatic value for clinicians working with people with HD and their families.

  2. Shored gunshot wound of exit. A phenomenon with identity crisis.

    Science.gov (United States)

    Aguilar, J C

    1983-09-01

    Shored gunshot wound of exit is produced when the outstretched skin is impaled, sandwiched, and crushed between the outgoing bullet and the unyielding object over the exit site, thus leaving an abrasion collar on the wound margin. Proper coaptation of the wound margin is impossible because of the loss of skin just like those observed in entrance wounds. In contrast to the entrance wound, the supported exit wound shows a scalloped or punched-out abrasion collar and sharply contoured skin in between the radiating skin lacerations marginating the abrasion (Fig. 1). Should gunpowder be observed around the exit site, it is often unevenly distributed, and is not associated with searing, gunpowder stippled abrasion, tatooing, and deposition of soot.

  3. Striatal grafts in a rat model of Huntington's disease

    DEFF Research Database (Denmark)

    Guzman, R; Meyer, M; Lövblad, K O

    1999-01-01

    Survival and integration into the host brain of grafted tissue are crucial factors in neurotransplantation approaches. The present study explored the feasibility of using a clinical MR scanner to study striatal graft development in a rat model of Huntington's disease. Rat fetal lateral ganglionic...... time-points graft location could not be further verified. Measures for graft size and ventricle size obtained from MR images highly correlated with measures obtained from histologically processed sections (R = 0.8, P fetal rat lateral ganglionic...

  4. Lingering illness or sudden death? Pre-exit employment developments in German establishments

    OpenAIRE

    Fackler, Daniel; Schnabel, Claus; Wagner, Joachim

    2012-01-01

    Using a large administrative dataset for Germany, this paper compares employment developments in exiting and surviving establishments. For both West and East Germany we find a clear 'shadow of death' effect reflecting lingering illness: establishments shrink dramatically already several years before closure, employment growth rates differ strongly between exiting and surviving establishments, and this difference becomes stronger as exit approaches. We further show that prior to exit the workf...

  5. Radiation sensitivity of fibroblast strains from patients with Usher's syndrome, Duchenne muscular dystrophy, and Huntington's disease

    International Nuclear Information System (INIS)

    Nove, J.; Little, J.B.; Tarone, R.E.; Robbins, J.H.

    1987-01-01

    The colony-forming ability of 10 normal human fibroblast cell strains and of 10 strains representing 3 degenerative diseases of either nerve or muscle cells was determined after exposure of the cells to X-rays or β-particles from tritiated water. Both methods of irradiation yielded similar comparative results. The fibroblast strains from the 5 Usher's syndrome patients and from 1 of the 2 Huntington's disease patients were hypersensitive to radiation, while those from the 3 Duchenne muscular dystrophy patients and the second Huntington's disease patient had normal sensitivity to radiation. These results indicate both disease-specific and strain-specific differences in the survival of fibroblasts after exposure to ionizing radiation. 38 refs.; 2 figs.; 3 tabs

  6. Analysis of unexpected exits using the Fokker - Planck equation

    NARCIS (Netherlands)

    Herwaarden, van O.A.

    1996-01-01


    In this thesis exit problems are considered for stochastic dynamical systems with small random fluctuations. We study exit from a domain in the state space through a boundary, or a specified part of the boundary, that is unattainable in the underlying deterministic system. We analyze

  7. A new mutation for Huntington disease following maternal transmission of an intermediate allele

    NARCIS (Netherlands)

    Semaka, Alicia; Kay, Chris; Belfroid, René D. M.; Bijlsma, Emilia K.; Losekoot, Monique; van Langen, Irene M.; van Maarle, Merel C.; Oosterloo, Mayke; Hayden, Michael R.; van Belzen, Martine J.

    2015-01-01

    New mutations for Huntington disease (HD) originate from CAG repeat expansion of intermediate alleles (27-35 CAG). Expansions of such alleles into the pathological range (≥ 36 CAG) have been exclusively observed in paternal transmission. We report the occurrence of a new mutation that defies the

  8. Digital bedrock geologic map of parts of the Huntington, Richmond, Bolton and Waterbury quadrangles, Vermont

    Data.gov (United States)

    Vermont Center for Geographic Information — Digital Data from VG95-9A Thompson, PJ�and Thompson, TB, 1995, Digital bedrock geologic map of parts of the Huntington, Richmond, Bolton and Waterbury quadrangles,...

  9. 77 FR 33805 - Notice of Passenger Facility Charge (PFC) Approvals and Disapprovals

    Science.gov (United States)

    2012-06-07

    ... AGENCY: Tri State Airport Authority, Huntington, West Virginia. APPLICATION NUMBER: 12-07-C-00-HTS... equipment. Install perimeter fencing. Rehabilitate terminal building. Rehabilitate taxiway A (west). Access.... Passenger loading bridges. Security exit lane equipment. PFC consulting fees. Brief Description of...

  10. Going, Going, Gone. Innovation and Exit in Manufacturing Firms

    NARCIS (Netherlands)

    E. Cefis (Elena); O. Marsili (Orietta)

    2007-01-01

    textabstractThis paper examines the effect of innovation on the risk of exit of a firm, distinguishing between different modes of exits. Innovation represents a resource and a capability that helps a firm to build competitive advantage and remain in the market. At the same time, the resources and

  11. Comparison of sources of exit fluence variation for IMRT

    International Nuclear Information System (INIS)

    Gardner, Joseph K; Gordon, J James; Wang Song; Siebers, Jeffrey V; Clews, Luke; Greer, Peter B

    2009-01-01

    The fluence exiting a patient during beam delivery can be used as treatment delivery quality assurance, either by direct comparison with expected exit fluences or by backprojection to reconstruct the patient dose. Multiple possible sources of measured exit fluence deviations exist, including changes in the beam delivery and changes in the patient anatomy. The purpose of this work is to compare the deviations caused by these sources. Machine delivery-related variability is measured by acquiring multiple dosimetric portal images (DPIs) of several test fields without a patient/phantom in the field over a time period of 2 months. Patient anatomy-related sources of fluence variability are simulated by computing transmission DPIs for a prostate patient using the same incident fluence for 11 different computed tomography (CT) images of the patient anatomy. The standard deviation (SD) and maximum deviation of the exit fluence, averaged over 5 mm x 5 mm square areas, is calculated for each test set. Machine delivery fluence SDs as large as 1% are observed for a sample patient field and as large as 2.5% for a picket-fence dMLC test field. Simulations indicate that day-to-day patient anatomy variations induce exit fluence SDs as large as 3.5%. The largest observed machine delivery deviations are 4% for the sample patient field and 7% for the picket-fence field, while the largest difference for the patient anatomy-related source is 8.5%. Since daily changes in patient anatomy can result in substantial exit fluence deviations, care should be taken when applying fluence back-projection to ensure that such deviations are properly attributed to their source. (note)

  12. Magnetic biomineralisation in Huntington's disease transgenic mice

    International Nuclear Information System (INIS)

    Beyhum, W; Hautot, D; Dobson, J; Pankhurst, Q A

    2005-01-01

    The concentration levels of biogenic magnetite nanoparticles in transgenic R6/2 Huntington's disease (HD) mice have been investigated, using seven control and seven HD mice each from an 8 week-old litter and from a 12 week-old litter. Hysteresis and isothermal remnant magnetisation data were collected on a SQUID magnetometer, and analysed using a model comprising dia/paramagnetic, ferrimagnetic and superparamagnetic contributions, to extract the magnetite and ferritin concentrations present. It was found that magnetite was present in both superparamagnetic and blocked states. A larger spread and higher concentration of magnetite levels was found in the diseased mice for both the 8 week-old and 12 week-old batches, compared to the controls

  13. Using an electronic portal imaging device for exit dose measurements in radiotherapy

    International Nuclear Information System (INIS)

    Ganowicz, M.; Wozniak, B.; Bekman, A.; Maniakowski, Z.

    2003-01-01

    To present a method of determining the exit dose with the use of an electronic portal imaging device (EPID). The device used was the Portal Vision LC250 (Varian). The EPID signals on the central beam axis have been related to the exit dose. The exit dose measurements were performed with the ionisation chamber in the slab phantom at the distance of dose maximum from the exit surface of the phantom. EPID reading was investigated as a function of field size, phantom thickness and source-detector distance. The relation between dose rate and the EPID reading is described with empirical functions applicable to the obtained data. The exit dose is calculated from the EPID reading as a product of the calibration factor and appropriate correction factors. The determination of the exit dose rate from the EPID signal requires the knowledge of many parameters and earlier determination of essential characteristics. (author)

  14. Evidence for Deficits on Different Components of Theory of Mind in Huntington's Disease

    NARCIS (Netherlands)

    Allain, P.; Havel-Thomassin, V.; Verny, C.; Gohier, B.; Lancelot, C.; Besnard, J.; Fasotti, L.; Le Gall, D.

    2011-01-01

    Objective: The main aim of this study was to investigate the effects of Huntington's disease (HD) on cognitive and affective Theory of Mind (ToM) abilities. The relation of ToM performance and executive functions was also examined. Method: Eighteen HD patients, early in the course of the disease,

  15. The determinants of firm exit in the French food industries

    OpenAIRE

    Blanchard, Pierre; Mathieu, Claude

    2012-01-01

    A semi-parametric approach is used to estimate firm propensity to exit. The unobserved individual productivity of a firm is first estimated using the Ackerberg et al. (2006) approach and then introduced as a determinant of firm exit in conjunction with other variables that may serve as barriers to exit, including the firm’s level of sunk costs and the industry concentration. Using an unbalanced panel of data for 5,849 firms in French food industries from 1996 to 2002, we find a signifi...

  16. Behavioral testing of minipigs transgenic for the Huntington gene-A three-year observational study

    Czech Academy of Sciences Publication Activity Database

    Schuldenzucker, V.; Schubert, R.; Muratori, L. M.; Freisfeld, F.; Rieke, L.; Matheis, T.; Schramke, S.; Motlík, Jan; Kemper, N.; Radespiel, U.; Reilmann, R.

    2017-01-01

    Roč. 12, č. 10 (2017), č. článku e0185970. E-ISSN 1932-6203 Institutional support: RVO:67985904 Keywords : Huntington´s disease * minipigs Subject RIV: EG - Zoology OBOR OECD: Behavioral sciences biology Impact factor: 2.806, year: 2016

  17. Motor, emotional and cognitive deficits in adult BACHD mice : A model for Huntington's disease

    NARCIS (Netherlands)

    Abada, Yah-se K.; Schreiber, Rudy; Ellenbroek, Bart

    2013-01-01

    Rationale: Huntington's disease (HD) is characterized by progressive motor dysfunction, emotional disturbances and cognitive deficits. It is a genetic disease caused by an elongation of the polyglutamine repeats in the huntingtin gene. Whereas HD is a complex disorder, previous studies in mice

  18. Reversal learning and associative memory impairments in a BACHD rat model for Huntington disease

    NARCIS (Netherlands)

    Abada, Yah-se K.; Nguyen, Huu Phuc; Ellenbroek, Bart; Schreiber, Rudy

    2013-01-01

    Chorea and psychiatric symptoms are hallmarks of Huntington disease (HD), a neurodegenerative disorder, genetically characterized by the presence of expanded CAG repeats (>35) in the HUNTINGTIN (HTT) gene. HD patients present psychiatric symptoms prior to the onset of motor symptoms and we recently

  19. Congenital Cervical Teratoma: Anaesthetic Management (The EXIT Procedure

    Directory of Open Access Journals (Sweden)

    Ferruh Bilgin

    2009-01-01

    Full Text Available Ex utero intrapartum treatment (EXIT is a procedure performed during caesarean section with preservation of fetal-placental circulation, which allows the safe handling of fetal airways with risk of airways obstruction. This report aimed at describing a case of anaesthesia for EXIT in a fetus with cervical teratoma. A 30-year-old woman, 70 kg, 160 cm, gravida 2, para 1, was followed because of polyhydramniosis diagnosed at 24 weeks′ gestation. During a routine ultrasonographic examination at 35 weeks′ gestation, it was noticed that the fetus had a tumoral mass on the anterior neck, the mass had cystic and calcified components and with a size of was 10 x 6 x5 cm. The patient with physical status ASA I, was submitted to caesarean section under general anaesthesia with mechanically controlled ventilation for exutero intrapartum treatment (EXIT. Anaesthesia was induced in rapid sequence with fentanyl, propofol and rocuronium and was maintained with isoflurane in 2.5 at 3 % in O 2 and N 2 O (50%. After hysterotomy, fetus was partially released assuring uterus-placental circulation, followed by fetal laryngoscopy and tracheal intuba-tion. The infant was intubated with an uncuffed, size 2.5 endotracheal tube. Excision of the mass was performed under general anaesthesia. After surgical intervention, on the fourth postoperative day, the infant was extubated and the newborn was discharged to the pediatric neonatal unit and on the seventh day postoperatively to home without complications. Major recommendations for EXIT are maternal-fetal safety, uterine relaxation to maintain uterine volume and uterus-placental circulation, and fetal immobility to help airway handling. We report one case of cervical teratoma managed successfully with EXIT procedure.

  20. The Effect of Exit Strategy on Optimal Portfolio Selection with Birandom Returns

    Directory of Open Access Journals (Sweden)

    Guohua Cao

    2013-01-01

    Full Text Available The aims of this paper are to use a birandom variable to denote the stock return selected by some recurring technical patterns and to study the effect of exit strategy on optimal portfolio selection with birandom returns. Firstly, we propose a new method to estimate the stock return and use birandom distribution to denote the final stock return which can reflect the features of technical patterns and investors' heterogeneity simultaneously; secondly, we build a birandom safety-first model and design a hybrid intelligent algorithm to help investors make decisions; finally, we innovatively study the effect of exit strategy on the given birandom safety-first model. The results indicate that (1 the exit strategy affects the proportion of portfolio, (2 the performance of taking the exit strategy is better than when the exit strategy is not taken, if the stop-loss point and the stop-profit point are appropriately set, and (3 the investor using the exit strategy become conservative.

  1. 30 CFR 56.4530 - Exits.

    Science.gov (United States)

    2010-07-01

    ... Resources MINE SAFETY AND HEALTH ADMINISTRATION, DEPARTMENT OF LABOR METAL AND NONMETAL MINE SAFETY AND HEALTH SAFETY AND HEALTH STANDARDS-SURFACE METAL AND NONMETAL MINES Fire Prevention and Control... a sufficient number of exits to permit prompt escape in case of fire. ...

  2. STIMULATION METHODS IMPROVEMENT OF EXIT ROUTE ON RAILWAY TRANSPORT

    Directory of Open Access Journals (Sweden)

    A. I. Verlan

    2014-01-01

    Full Text Available Purpose. The purpose of the article is to assess the costs, which are redistributed in the system «shipper−railroad−consignee» during routing of rail transportation and the development of tariff simulation methods of shippers to the exit routes formation. Methodology. Using economic and mathematical analysis the distribution of costs among the various participants of transportation process during the exit routes formation is investigated in the article. Findings. For implementation of the tariff simulation methods of exit routes and retention of the existing tariff structure it is proposed to provide in the «Tariff catalogue for freight transportation by railway transport of Ukraine» the discount, differentiated from haulage distance. A new method for determining the fees amount for cars supply and removal on approach tracks by train locomotives was also offered. Originality. As a result of the research a new method for determination of the exit rout efficiency that, unlike the existing one, takes into account the various interests of the individual participants in the transportation process was proposed. The dependence of the correction factors to the tariff for freight transportation in their own cars by direct exit routes from distance haulage was obtained. Implementation of these coefficients provides an approximation of railway tariffs to the traffic handling costs. A method for determination the rate of fees for cars supply and removal on approach tracks by train locomotives was offered. Practical value. InUkraine creation of the tariff discounts system for freight transportation by exit routes consistent with international practice and allows bringing the tariff to the real traffic handling cost. This change on the one hand will provide stimulation for private capital investments in infrastructure development and shunting means of approach tracks, on the other – it will fix the shippers to the railroads and stop their outflow

  3. The adjustment of γ-aminobutyric acidA tonic subunits in Huntington's disease: from transcription to translation to synaptic levels into the neostriatum

    Directory of Open Access Journals (Sweden)

    Abraham Rosas-Arellano

    2018-01-01

    Full Text Available γ-Aminobutyric acid (GABA, plays a key role in all stages of life, also is considered the main inhibitory neurotransmitter. GABA activates two kind of membrane receptors known as GABAA and GABAB, the first one is responsible to render tonic inhibition by pentameric receptors containing α4−6, β3, δ, or ρ1−3 subunits, they are located at perisynaptic and/or in extrasynaptic regions. The biophysical properties of GABAA tonic inhibition have been related with cellular protection against excitotoxic injury and cell death in presence of excessive excitation. On this basis, GABAA tonic inhibition has been proposed as a potential target for therapeutic intervention of Huntington's disease. Huntington's disease is a neurodegenerative disorder caused by a genetic mutation of the huntingtin protein. For experimental studies of Huntington's disease mouse models have been developed, such as R6/1, R6/2, HdhQ92, HdhQ150, as well as YAC128. In all of them, some key experimental reports are focused on neostriatum. The neostriatum is considered as the most important connection between cerebral cortex and basal ganglia structures, its cytology display two pathways called direct and indirect constituted by medium sized spiny neurons expressing dopamine D1 and D2 receptors respectively, they display strong expression of many types of GABAA receptors, including tonic subunits. The studies about of GABAA tonic subunits and Huntington's disease into the neostriatum are rising in recent years, suggesting interesting changes in their expression and localization which can be used as a strategy to delay the cellular damage caused by the imbalance between excitation and inhibition, a hallmark of Huntington's disease.

  4. Accuracy synthesis of T-shaped exit fixed mechanism in a double-crystal monochromator

    International Nuclear Information System (INIS)

    Wang Fengqin; Cao Chongzhen; Wang Jidai; Li Yushan; Gao Xueguan

    2007-01-01

    It is a key performance requirement for a double-crystal monochromator that the exit is fixed, and in order to improve the height accuracy of the exit in T-shaped exit fixed mechanism, the expression between the height of the exit and various original errors was put forward using geometrical analysis method. According to the independent action principle of original errors, accuracy synthesis of T-shaped exit fixed mechanism was studied by using the equal accuracy method, and the tolerance ranges of original errors were obtained. How to calculate the tolerance ranges of original errors was explained by giving an example. (authors)

  5. Technological Progress, Exit and Trade

    DEFF Research Database (Denmark)

    Schröder, Philipp; Sørensen, Allan

    productivity exporters are more likely to continue to export, and market exit is typically preceded by periods of contracting market shares. We show that the simple inclusion of exogenous economy wide technological progress into the standard Melitz (2003) model generates a tractable dynamic framework...

  6. Do spouses coordinate their work exits? A combined survey and register analysis from Norway.

    Science.gov (United States)

    Syse, Astri; Solem, Per Erik; Ugreninov, Elisabeth; Mykletun, Reidar; Furunes, Trude

    2014-09-01

    Research on spouses' joint work exits is scarce, although household factors such as spouses' work status, marital quality, and caregiving burdens are likely to affect seniors' work engagement. We therefore examine whether the work exit probability of one spouse affects that of the other. Discrete-time hazard regression analyses of survey data linked to later registry information including all gainfully employed married respondents aged 50-74 with a working spouse (N = 1,764) were used to assess subsequent work exits. A spouse's work exit is a strong predictor of a respondent's work exit (hazard ratio 3.1, 95% confidence interval [2.5, 4.0]). Educational attainment, poor marital quality, and spouses' health and care needs do not predict work exits. Surprisingly, no gender differences are observed. Research on larger survey samples to distinguish different work exit routes and reasons for spouses' joint work exits appears warranted. To account for cultural and welfare state characteristics, cross-national studies ought to be undertaken. © The Author(s) 2013.

  7. Experiment of cavitation erosion at the exit of a long orifice

    Energy Technology Data Exchange (ETDEWEB)

    Yagi, Yoshinori; Murase, Michio [Inst. of Nuclear Safety System Inc., Mihama, Fukui (Japan)

    2002-09-01

    We performed experiments to clarify mechanism of cavitation erosion and to predict cavitation erosion rate at the exit of a long orifice equipped at the chemical and volume control system in a pressurized water reactor (PWR). In order to find this mechanism, we used a high speed video camera. As the result, we observed bubble collapses near the exit of the orifice when flow condition was oscillating. So the bubble collapses due to the oscillation might cause the first stage erosion at the exit of the orifice. Using the orifice which had the cone-shaped exit, we observed that bubbles collapsed near the exit and then they collapsed at the upstream like a chain reaction. So this bubble collapse mechanism could be explained as follows: shock wave was generated by the bubble collapse near the exit, then it propagated upwards, consequently it caused the bubble collapse at the upstream. And we predicted erosion rate by evaluating the effect of the velocity and comparing the erosion resistance between the test speciment (aluminum) and the plant material (stainless steel) by means of vibratory tests. We compared the predicted erosion rate with that of the average value estimated from plant investigation, then we examined the applicability of these method to the plant evaluations. (author)

  8. “Understanding exit from the founder’s business in family firms”

    OpenAIRE

    C. Salvato; F. Chirico; P. Sharma

    2010-01-01

    In this chapter we investigate the role of family-specific factors in facilitating or constraining business exit in family firms. Family business literature seems to have an implicit bias towards continuity and persistence in the founder’s business. This is explained by heavy emotional involvement and development of path-dependent core competences over generations. However, several long-lived family firms were able to successfully exit the founder’s business. Exit allowed them to free signifi...

  9. Water-Exit Process Modeling and Added-Mass Calculation of the Submarine-Launched Missile

    Directory of Open Access Journals (Sweden)

    Yang Jian

    2017-11-01

    Full Text Available In the process that the submarine-launched missile exits the water, there is the complex fluid solid coupling phenomenon. Therefore, it is difficult to establish the accurate water-exit dynamic model. In the paper, according to the characteristics of the water-exit motion, based on the traditional method of added mass, considering the added mass changing rate, the water-exit dynamic model is established. And with help of the CFX fluid simulation software, a new calculation method of the added mass that is suit for submarine-launched missile is proposed, which can effectively solve the problem of fluid solid coupling in modeling process. Then by the new calculation method, the change law of the added mass in water-exit process of the missile is obtained. In simulated analysis, for the water-exit process of the missile, by comparing the results of the numerical simulation and the calculation of theoretical model, the effectiveness of the new added mass calculation method and the accuracy of the water-exit dynamic model that considers the added mass changing rate are verified.

  10. [Predictors of mental and physical quality of life in Huntington's disease].

    Science.gov (United States)

    Brugger, F; Hepperger, C; Hametner, E-M; Holl, A K; Painold, A; Schusterschitz, C; Bonelli, R; Holas, C; Wenning, G K; Poewe, W; Seppi, K

    2015-02-01

    The assessment of health-related quality of life (hrQoL) is an important tool in therapy studies and in the treatment of patients with Huntington's disease (HD). In the absence of causal interventions, HD therapy targets the alleviation of symptoms aiming to improve impaired hrQoL. The aim of this study was to determine the impact of disease characteristics on hrQoL in HD. A total of 80 genetically confirmed HD patients underwent an assessment using the Unified Huntington's Disease Rating Scale, the Beck Depression Inventory, the Hamilton Rating Scale and the SF-36, a scale for the assessment of physical and mental QoL. Multiple regression analysis revealed that health-related physical and mental QoL was considerably influenced by the functional capacity. The mental QoL also correlated with the degree of depressive symptoms, age and the number of CAG repeats. However, there was no statistical relation between QoL and motor and cognitive abilities. This study underlines the relationship between function capacity and depressive symptoms with mental and physical QoL. This is the first time that hrQoL has been investigated in a German speaking cohort. The results are in accordance with previous studies of hrQoL in HD.

  11. Sensory modulation intervention and behaviour support modification for the treatment of severe aggression in Huntington's disease. A single case experimental design.

    Science.gov (United States)

    Fisher, Caroline A; Brown, Anahita

    2017-09-01

    Aggression is common in Huntington's disease. However, at present there are no standard guidelines for managing aggression in Huntington's sufferers due to a lack of empirical research. This paper presents a case study of the treatment of very high levels of aggression with sensory modulation and behaviour support intervention in a Huntington's sufferer. The client exhibited a range of aggressive behaviours, including physical aggression to people, furniture and objects, and verbal aggression. Following an eight week baseline phase, five weeks of sensory modulation intervention were employed. A behaviour support plan was then implemented as an adjunct to the sensory intervention, with aggressive behaviour systematically audited for a further 11 weeks. The results indicate a significant reduction in reported levels of aggression during the combined sensory modulation and behaviour support phase, compared to both the baseline and the sensory modulation therapy alone phases. This case study highlights the efficacy non-pharmacological interventions may have for reducing aggression in HD.

  12. The V471A polymorphism in autophagy-related gene ATG7 modifies age at onset specifically in Italian Huntington disease patients

    DEFF Research Database (Denmark)

    Metzger, Silke; Walter, Carolin; Riess, Olaf

    2013-01-01

    The cause of Huntington disease (HD) is a polyglutamine repeat expansion of more than 36 units in the huntingtin protein, which is inversely correlated with the age at onset of the disease. However, additional genetic factors are believed to modify the course and the age at onset of HD. Recently......, we identified the V471A polymorphism in the autophagy-related gene ATG7, a key component of the autophagy pathway that plays an important role in HD pathogenesis, to be associated with the age at onset in a large group of European Huntington disease patients. To confirm this association in a second...... independent patient cohort, we analysed the ATG7 V471A polymorphism in additional 1,464 European HD patients of the "REGISTRY" cohort from the European Huntington Disease Network (EHDN). In the entire REGISTRY cohort we could not confirm a modifying effect of the ATG7 V471A polymorphism. However, analysing...

  13. Huntington's disease predictive testing: the case for an assessment approach to requests from adolescents.

    Science.gov (United States)

    Binedell, J; Soldan, J R; Scourfield, J; Harper, P S

    1996-01-01

    Adolescents who are actively requesting Huntington's predictive testing of their own accord pose a dilemma to those providing testing. In the absence of empirical evidence as regards the impact of genetic testing on minors, current policy and guidelines, based on the ethical principles of non-maleficence and respect for individual autonomy and confidentiality, generally exclude the testing of minors. It is argued that adherence to an age based exclusion criterion in Huntington's disease predictive testing protocols is out of step with trends in UK case law concerning minors' consent to medical treatment. Furthermore, contributions from developmental psychology and research into adolescents' decision making competence suggest that adolescents can make informed choices about their health and personal lives. Criteria for developing an assessment approach to such requests are put forward and the implications of a case by case evaluation of competence to consent in terms of clinicians' tolerance for uncertainty are discussed. PMID:8950670

  14. Collective behavior of mice passing through an exit under panic

    Science.gov (United States)

    Zhang, Teng; Zhang, Xuelin; Huang, Shenshi; Li, Changhai; Lu, Shouxiang

    2018-04-01

    Collective movement of animal in emergency condition has attracted growing attentions among researchers. However, many rules still need to be confirmed with adequate explanation. Study of collective behavior of mice can improve our understanding about the dynamics of pedestrian movement. However, its rules still need to be confirmed with adequate explanation. In this paper, collective behavior of mice passing through an exit under panic was investigated. The results showed that the total evacuation time decreased with exit width increasing in a certain range. Based on the different tendency of the curve in temporal evolution, the process of mice flow was divided into three stages. The density of mice near the exit peaks at a certain horizontal offset and starts to decrease over time. With the increase of the exit width, the duration of the higher density state decreased. We found that the frequency of time intervals obeyed a lognormal distribution or an exponential decay for different exit widths. In addition, the relationship between the group size and the group flow rate in different scenarios was analyzed. The phenomena found in our experiments show the collective behavioral characteristic of mice under panic. Our analysis in this paper will deepen our understanding of crowd dynamics in emergency condition.

  15. 77 FR 51064 - Huntington Foam LLC, Fort Smith, AR; Notice of Affirmative Determination Regarding Application...

    Science.gov (United States)

    2012-08-23

    ... DEPARTMENT OF LABOR Employment and Training Administration [TA-W-81,475] Huntington Foam LLC, Fort Smith, AR; Notice of Affirmative Determination Regarding Application for Reconsideration By application dated May 21, 2012, the State Workforce Office requested administrative reconsideration of the negative...

  16. Polysomnographic Findings and Clinical Correlates in Huntington Disease: A Cross-Sectional Cohort Study

    Science.gov (United States)

    Piano, Carla; Losurdo, Anna; Della Marca, Giacomo; Solito, Marcella; Calandra-Buonaura, Giovanna; Provini, Federica; Bentivoglio, Anna Rita; Cortelli, Pietro

    2015-01-01

    Study Objectives: To evaluate the sleep pattern and the motor activity during sleep in a cohort of patients affected by Huntington disease (HD). Design: Cross-sectional cohort study. Setting: Sleep laboratory. Patients: Thirty HD patients, 16 women and 14 men (mean age 57.3 ± 12.2 y); 30 matched healthy controls (mean age 56.5 ± 11.8 y). Interventions: Subjective sleep evaluation: Epworth Sleepiness Scale (ESS); Berlin's Questionnaire, interview for restless legs syndrome (RLS), questionnaire for REM sleep behavior disorder (RBD). Clinical evaluation: disease duration, clinical severity (unified Huntington disease motor rating scale [UHDMRS]), genetic tests. Laboratory-based full-night attended video-polysomnography (V-PSG). Measurements and Results: The duration of the disease was 9.4 ± 4.4 y, UHMDRS score was 55.5 ± 23.4, CAG repeats were 44.3 ± 4.1. Body mass index was 21.9 ± 4.0 kg/m2. No patients or caregivers reported poor sleep quality. Two patients reported symptoms of RLS. Eight patients had an ESS score ≥ 9. Eight patients had high risk of obstructive sleep apnea. At the RBD questionnaire, two patients had a pathological score. HD patients, compared to controls, showed shorter sleep, reduced sleep efficiency index, and increased arousals and awakenings. Four patients presented with sleep disordered breathing (SDB). Periodic limb movements (PLMs) during wake and sleep were observed in all patients. No episode of RBD was observed in the V-PSG recordings, and no patients showed rapid eye movement (REM) sleep without atonia. The disease duration correlated with ESS score (P Marca G, Solito M, Calandra-Buonaura G, Provini F, Bentivoglio AR, Cortelli P. Polysomnographic findings and clinical correlates in Huntington disease: a cross-sectional cohort study. SLEEP 2015;38(9):1489–1495. PMID:25845698

  17. Drug and alcohol-impaired driving among electronic music dance event attendees.

    Science.gov (United States)

    Furr-Holden, Debra; Voas, Robert B; Kelley-Baker, Tara; Miller, Brenda

    2006-10-15

    Drug-impaired driving has received increased attention resulting from development of rapid drug-screening procedures used by police and state laws establishing per se limits for drug levels in drivers. Venues that host electronic music dance events (EMDEs) provide a unique opportunity to assess drug-impaired driving among a high proportion of young adult drug users. EMDEs are late-night dance parties marked by a substantial number of young adult attendees and elevated drug involvement. No studies to date have examined drug-impaired driving in a natural environment with active drug and alcohol users. Six EMDEs were sampled in San Diego, California, and Baltimore, Maryland. A random sample of approximately 40 attendees per event were administered surveys about alcohol and other drug (AOD) use and driving status, given breath tests for alcohol, and asked to provide oral fluid samples to test for illicit drug use upon entering and exiting the events. Driving status reduced the level of alcohol use (including abstaining) but the impact on drug-taking was not significant. However, 62% of individuals who reported their intention to drive away from the events were positive for drugs or alcohol upon leaving. This suggests that these events and settings are appropriate ones for developing interventions for reducing risks for young adults.

  18. 30 CFR 57.4530 - Exits for surface buildings and structures.

    Science.gov (United States)

    2010-07-01

    ... 30 Mineral Resources 1 2010-07-01 2010-07-01 false Exits for surface buildings and structures. 57... Fire Prevention and Control Installation/construction/maintenance § 57.4530 Exits for surface buildings and structures. Surface buildings or structures in which persons work shall have a sufficient number...

  19. Exit, punishment and rewards in commons dilemmas: an experimental study.

    Directory of Open Access Journals (Sweden)

    Giangiacomo Bravo

    Full Text Available Commons dilemmas are interaction situations where a common good is provided or exploited by a group of individuals so that optimal collective outcomes clash with private interests. Although in these situations, social norms and institutions exist that might help individuals to cooperate, little is known about the interaction effects between positive and negative incentives and exit options by individuals. We performed a modified public good game experiment to examine the effect of exit, rewards and punishment, as well as the interplay between exit and rewards and punishment. We found that punishment had a stronger effect than rewards on cooperation if considered by itself, whereas rewards had a stronger effect when combined with voluntary participation. This can be explained in terms of the 'framing effect', i.e., as the combination of exit and rewards might induce people to attach higher expected payoffs to cooperative strategies and expect better behaviour from others.

  20. Critical heat flux and exit film flow rate in a flow boiling system

    International Nuclear Information System (INIS)

    Ueda, Tatsuhiro; Isayama, Yasushi

    1981-01-01

    The critical heat flux in a flowing boiling system is an important problem in the evaporating tubes with high thermal load such as nuclear reactors and boilers, and gives the practical design limit. When the heat flux in uniformly heated evaporating tubes is gradually raised, the tube exit quality increases, and soon, the critical heat flux condition arises, and the wall temperature near tube exit rises rapidly. In the region of low exit quality, the critical heat flux condition is caused by the transition from nucleating boiling, and in the region of high exit quality, it is caused by dry-out. But the demarcation of both regions is not clear. In this study, for the purpose of obtaining the knowledge concerning the critical heat flux condition in a flowing boiling system, the relation between the critical heat flux and exit liquid film flow rate was examined. For the experiment, a uniformly heated vertical tube supplying R 113 liquid was used, and the measurement in the range of higher heating flux and mass velocity than the experiment by Ueda and Kin was carried out. The experimental setup and experimental method, the critical heat flux and exit quality, the liquid film flow rate at heating zone exit, and the relation between the critical heat flux and the liquid film flow rate at exit are described. (Kako, I.)

  1. First Degree Pacemaker Exit Block

    Directory of Open Access Journals (Sweden)

    Johnson Francis

    2016-10-01

    Full Text Available Usually atrial and ventricular depolarizations follow soon after the pacemaker stimulus (spike on the ECG. But there can be an exit block due to fibrosis at the electrode - tissue interface at the lead tip. This can increase the delay between the spike and atrial or ventricular depolarization.

  2. Exit Planning At Joost El

    DEFF Research Database (Denmark)

    Nellemann, Camilla

    2017-01-01

    This is a Danish version. This case focuses on an owner-manager's considerations regarding his exit. Carsten Joost established Joost El in 2005. It currently employs 10 people. He has two sons but no intention to hand over the business to them. Rather, he hopes that his key employee eventually...

  3. Effects of injection nozzle exit width on rotating detonation engine

    Science.gov (United States)

    Sun, Jian; Zhou, Jin; Liu, Shijie; Lin, Zhiyong; Cai, Jianhua

    2017-11-01

    A series of numerical simulations of RDE modeling real injection nozzles with different exit widths are performed in this paper. The effects of nozzle exit width on chamber inlet state, plenum flowfield and detonation propagation are analyzed. The results are compared with that using an ideal injection model. Although the ideal injection model is a good approximation method to model RDE inlet, the two-dimensional effects of real nozzles are ignored in the ideal injection model so that some complicated phenomena such as the reflected waves caused by the nozzle walls and the reversed flow into the nozzles can not be modeled accurately. Additionally, the ideal injection model overpredicts the block ratio. In all the cases that stabilize at one-wave mode, the block ratio increases as the nozzle exit width gets smaller. The dual-wave mode case also has a relatively high block ratio. A pressure oscillation in the plenum with the same main frequency with the rotating detonation wave is observed. A parameter σ is applied to describe the non-uniformity in the plenum. σ increases as the nozzle exit width gets larger. Under some condition, the heat release on the interface of fresh premixed gas layer and detonation products can be strong enough to induce a new detonation wave. A spontaneous mode-transition process is observed for the smallest exit width case. Due to the detonation products existing in the premixed gas layer before the detonation wave, the detonation wave will propagate through reactants and products alternately, and therefore its strength will vary with time, especially near the chamber inlet. This tendency gets weaker as the injection nozzle exit width increases.

  4. Revisit the faster-is-slower effect for an exit at a corner

    Science.gov (United States)

    Chen, Jun Min; Lin, Peng; Wu, Fan Yu; Li Gao, Dong; Wang, Guo Yuan

    2018-02-01

    The faster-is-slower effect (FIS), which means that crowd at a high enough velocity could significantly increase the evacuation time to escape through an exit, is an interesting phenomenon in pedestrian dynamics. Such phenomenon had been studied widely and has been experimentally verified in different systems of discrete particles flowing through a centre exit. To experimentally validate this phenomenon by using people under high pressure is difficult due to ethical issues. A mouse, similar to a human, is a kind of self-driven and soft body creature with competitive behaviour under stressed conditions. Therefore, mice are used to escape through an exit at a corner. A number of repeated tests are conducted and the average escape time per mouse at different levels of stimulus are analysed. The escape times do not increase obviously with the level of stimulus for the corner exit, which is contrary to the experiment with the center exit. The experimental results show that the FIS effect is not necessary a universal law for any discrete system. The observation could help the design of buildings by relocating their exits to the corner in rooms to avoid the formation of FIS effect.

  5. Radiosensitivity in Huntington's disease: implications for pathogenesis and presymptomatic diagnosis

    International Nuclear Information System (INIS)

    Moshell, A.N.; Tarone, R.E.; Barrett, S.F.; Robbins, J.H.

    1980-01-01

    Huntington's disease (HD) is a dominantly inherited fatal disorder characterised by premature death of nerve cells. Cultured lymphocyte lines from four patients with HD were abnormally sensitive to the lethal effects of X rays, as were lines from two of five subjects at risk for HD. The hypersensitivity is specific for ionising radiation, since HD lines had normal survival after exposure to ultraviolet radiation. The hypersensitivity, which may reflect an inherited defect in DNA repair, provides the basis for a presymptomatic diagnostic test for the disease. (author)

  6. Driving behaviors and accident risk under lifetime license revocation.

    Science.gov (United States)

    Chang, Hsin-Li; Woo, T Hugh; Tseng, Chien-Ming; Tseng, I-Yen

    2011-07-01

    This study explored the driving behaviors and crash risk of 768 drivers who were under administrative lifetime driver's license revocation (ALLR). It was found that most of the ALLR offenders (83.2%) were still driving and only a few (16.8%) of them gave up driving completely. Of the offenders still driving, 67.6% experienced encountering a police roadside check, but were not detained or ticketed by the police. Within this group, 50.6% continued driving while encountering a police check, 18.0% of them made an immediate U-turn and 9.5% of them parked and exited their car. As to crash risk, 15.2% of the ALLR offenders had at least one crash experience after the ALLR had been imposed. The results of the logistic regression models showed that the offenders' crash risk while under the ALLR was significantly correlated with their personal characteristics (personal income), penalty status (incarceration, civil compensation and the time elapsed since license revocation), annual distance driven, and needs for driving (working, commuting and driving kids). Low-income offenders were more inclined to have a crash while driving under the ALLR. Offenders penalized by being incarcerated or by paying a high civil compensation drove more carefully and were less of a crash risk under the ALLR. The results also showed there were no differences in crash risk under the ALLR between hit-and-run offences and drunk driving offences or for offenders with a professional license or an ordinary license. Generally, ALLR offenders drove somewhat more carefully and were less of a crash risk (4.3 crashes per million km driven) than legal licensed drivers (23.1 crashes per million km driven). Moreover, they seemed to drive more carefully than drivers who were under short-term license suspension/revocation which previous studies have found. Copyright © 2011 Elsevier Ltd. All rights reserved.

  7. An interplay between extracellular signalling and the dynamics of the exit from pluripotency drives cell fate decisions in mouse ES cells

    Directory of Open Access Journals (Sweden)

    David A. Turner

    2014-06-01

    Full Text Available Embryonic Stem cells derived from the epiblast tissue of the mammalian blastocyst retain the capability to differentiate into any adult cell type and are able to self-renew indefinitely under appropriate culture conditions. Despite the large amount of knowledge that we have accumulated to date about the regulation and control of self-renewal, efficient directed differentiation into specific tissues remains elusive. In this work, we have analysed in a systematic manner the interaction between the dynamics of loss of pluripotency and Activin/Nodal, BMP4 and Wnt signalling in fate assignment during the early stages of differentiation of mouse ES cells in culture. During the initial period of differentiation, cells exit from pluripotency and enter an Epi-like state. Following this transient stage, and under the influence of Activin/Nodal and BMP signalling, cells face a fate choice between differentiating into neuroectoderm and contributing to Primitive Streak fates. We find that Wnt signalling does not suppress neural development as previously thought and that it aids both fates in a context dependent manner. Our results suggest that as cells exit pluripotency they are endowed with a primary neuroectodermal fate and that the potency to become endomesodermal rises with time. We suggest that this situation translates into a “race for fates” in which the neuroectodermal fate has an advantage.

  8. Application of an entry-exit tariff model to the gas transport system in Spain

    International Nuclear Information System (INIS)

    Alonso, Alejandro; Serrano, Miguel; Olmos, Luis

    2010-01-01

    Under an entry-exit gas tariff system, reservation of capacity is split into entry capacity, to transport gas from the injection points to a virtual balancing point, and exit capacity, to transport gas from the balancing point to the exit points in the system. Entry-exit tariff for gas transport systems have been recommended by the 3rd EU Energy Package, since they are cost reflective, facilitate gas trade and can provide signals for the location of gas injections or off-takes. The advisability of applying an entry-exit tariff system is discussed in this paper. Apart from this, authors propose an entry-exit tariff model and apply it to compute charges for the Spanish gas transport system in 2009. Results produced by the model are presented as coefficients which should multiply the current postal transport tariff. The paper concludes that entry-exit tariffs would be useful location signals which would result in a better use of the gas transport system in Spain. In those cases where demand exceeds available capacity, as it occurs at the congested connection with France, entry-exit tariffs could be supplemented by capacity charges at entry points resulting from auctions. (author)

  9. The use of the exit interview to reduce turnover amongst healthcare professionals.

    Science.gov (United States)

    Flint, Anndrea; Webster, Joan

    2011-01-19

    Exit interviews are widely used in healthcare organisations to identify reasons for staff attrition, yet their usefulness in limiting turnover is unclear. To determine the effectiveness of various exit interview strategies in decreasing turnover rates amongst healthcare professionals. We used a comprehensive search strategy including an electronic search of the following databases: DARE, CENTRAL, MEDLINE, EMBASE, CINAHL, PsycINFO, ERIC (search date: 7 September 2010) and EPOC Specialised Register (search date: 30 September 2009). We also screened the reference lists of included studies and relevant reviews. Randomised controlled trials, controlled clinical trials, controlled before and after studies and interrupted time series studies comparing turnover rates between healthcare professionals who had undergone one form of exit interview with another form of exit interview or with no interview. Two review authors independently assessed trial quality and extracted data. The search identified 1560 citations of which we considered 19 potentially relevant. The two authors independently reviewed the abstracts of these studies and retrieved the full texts of eight studies. We excluded all eight following independent assessment. They were either interviews, commentaries on how to do an exit interview or descriptive studies about reasons for leaving. We found no trials that matched our inclusion criteria. Evidence about the effectiveness of exit interviews to reduce turnover is currently not available. However, exit interviews may provide useful information about the work environment which, in turn, may be useful in the development of interventions to reduce turnover.

  10. Exploring the Relationship of Exit Flow and Jam Density in Panic Scenarios Using Animal Dynamics

    NARCIS (Netherlands)

    Sobhani, A.; Sarvi, M.; Duives, D.C.; Ejtemai, O.; Aghabayk, K.; Hoogendoorn, S.P.

    2014-01-01

    There are few studies investigating crowd dynamics in panic situations. They used measures such as exit flow rate to explore the exit performance in evacuation scenarios. However, there is limited research exploring the relationship of exit flow rate and density behind the exit for panic scenarios.

  11. Reduced gluconeogenesis and lactate clearance in Huntington's disease

    DEFF Research Database (Denmark)

    Josefsen, Knud; Nielsen, Signe M B; Campos, André

    2010-01-01

    We studied systemic and brain glucose and lactate metabolism in Huntington's disease (HD) patients in response to ergometer cycling. Following termination of exercise, blood glucose increased abruptly in control subjects, but no peak was seen in any of the HD patients (2.0 ± 0.5 vs. 0.0 ± 0.2mM, P...... HD mouse model R6/2 following a lactate challenge, combined with reduced phosphoenolpyruvate carboxykinase and increased pyruvate kinase activity in the mouse liver suggest a reduced capacity...... for gluconeogenesis in HD, possibly contributing to the clinical symptoms of HD. We propose that blood glucose concentration in the recovery from exercise can be applied as a liver function test in HD patients....

  12. Minipig Model of Huntington's Disease: H-1 Magnetic Resonance Spectroscopy of the Brain

    Czech Academy of Sciences Publication Activity Database

    Jozefovičová, M.; Herynek, V.; Jírů, F.; Dezortová, M.; Juhásová, Jana; Juhás, Štefan; Motlík, Jan; Hájek, M.

    2016-01-01

    Roč. 65, č. 1 (2016), s. 155-163 ISSN 0862-8408 R&D Projects: GA TA ČR(CZ) TA01011466; GA MŠk(CZ) 7F14308; GA MŠk ED2.1.00/03.0124 Institutional support: RVO:67985904 Keywords : Huntington´s disease * minipigs * magnetic resonance spectroscopy Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 1.461, year: 2016

  13. International Competition and Small-Firm Exit in US Manufacturing

    OpenAIRE

    Robert M Feinberg

    2013-01-01

    This study analyzes both the determinants of small firm exit rates in US manufacturing over the 1989–2004 period, especially the reaction of domestic firms to the nature of foreign competition as measured by industry-specific real exchange rate movements (interacted with import penetration by industry). These international pressures seem to lead to increased rates of smallest-firm exit in manufacturing, though the magnitudes of these effects are smaller than sometimes discussed. However, high...

  14. CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion

    DEFF Research Database (Denmark)

    Lee, J-M; Ramos, E M; Lee, J-H

    2012-01-01

    Age at onset of diagnostic motor manifestations in Huntington disease (HD) is strongly correlated with an expanded CAG trinucleotide repeat. The length of the normal CAG repeat allele has been reported also to influence age at onset, in interaction with the expanded allele. Due to profound...

  15. Improved social force model based on exit selection for microscopic pedestrian simulation in subway station

    Institute of Scientific and Technical Information of China (English)

    郑勋; 李海鹰; 孟令云; 许心越; 陈旭

    2015-01-01

    An improved social force model based on exit selection is proposed to simulate pedestrians’ microscopic behaviors in subway station. The modification lies in considering three factors of spatial distance, occupant density and exit width. In addition, the problem of pedestrians selecting exit frequently is solved as follows: not changing to other exits in the affected area of one exit, using the probability of remaining preceding exit and invoking function of exit selection after several simulation steps. Pedestrians in subway station have some special characteristics, such as explicit destinations, different familiarities with subway station. Finally, Beijing Zoo Subway Station is taken as an example and the feasibility of the model results is verified through the comparison of the actual data and simulation data. The simulation results show that the improved model can depict the microscopic behaviors of pedestrians in subway station.

  16. Does Business Cycle Have an Impact on Entrants and Exits?

    Directory of Open Access Journals (Sweden)

    Nikolay Sterev

    2017-03-01

    Full Text Available Purpose: The role of entrants and exits has enlarged indisputably over recent years. The basic explanation is connected to the deepening of innovation's influence on industrial growth. Furthermore, new businesses have to be more effective, and based on products, technological or organizational innovations, and exits have to be ineffective (respectively unprofitable, based on denoted products or technology. Design/methodology/approach: According to the above-mentioned prerequisites, policymakers need to manage the role (respectively the impact that entrants (new start-up companies and exits play in industrial growth. Nevertheless, this impact is not a cornerstone of the Bulgarian National Strategy, or the Europe 2020 Strategy. Findings: The paper tries to answer the following two questions: 1 Do new start-up companies and exits have any role and influence on economic growth in Bulgaria? and 2 Does the role (respectively the impact of entrants and exits in industrial growth change according to economic cycle? Research limitations/implications: In addition, according to the Lisbon Strategy, as well as the European Union's (EU Strategy 2020, the current economic policy supports entrepreneurship and innovations. Thus, the establishment of innovative companies, as well as the development of innovative, incumbent business are core issues of EU economic policy for the past decade. Originality/value: The paper builds on the industrial dynamic methodology and on the understanding of how business decisions (entrepreneurship, innovations, and R&D on micro level correspond to macro level (GDP growth and innovation policy.

  17. The comparison of exit-site care with normal saline and povidone-iodine in preventing exit-site infection and peritonitis in children on chronic peritoneal dialysis treatment

    Directory of Open Access Journals (Sweden)

    Onder Yavascan

    2011-01-01

    Full Text Available Peritonitis and catheter exit- site infections (ESI are important causes of hospitalization and catheter loss in patients undergoing chronic peritoneal dialysis (CPD. The frequency of infection can be reduced by scrupulous exit- site care with or without topical antiseptics. There are no studies showing any benefit in the use of povidone-iodine or normal saline for care of exit- sites in long- term CPD patients. In this study, we aimed to determine the potential effectiveness of the application of povidone-iodine or normal saline at the catheter exit- site in preventing ESI and peritonitis in children on CPD. A total of 98 patients treated with either povidone-iodine or normal saline were included in this study. Group I (34 patients used povidone-iodine and group II (64 patients simply cleansed the exit- site with normal saline (0.9% NaCl. Dressings were changed 2 to 3 times in a week. The total cumulative follow- up time was 3233 patient- months. ESIs occurred in 10 (29.4% of 34 patients using povidone-iodine and in 10 (15.6% of 64 patients using normal saline. The frequency of ESI was significantly high in group I (povidone-iodine patients. The mean rate of ESI was 1 episode/60.8 patient- months for group I versus 1 episode/144 patient- months for group II (P 0.05. In conclusion, exit- site care with normal saline is an effective strategy in reducing the incidence of ESI in children on CPD. It can thus significantly reduce morbidity, catheter loss, and the need to transfer patients on peritoneal dialysis to hemodialysis.

  18. Genetic Contributors to Intergenerational CAG Repeat Instability in Huntington's Disease Knock-In Mice.

    Science.gov (United States)

    Neto, João Luís; Lee, Jong-Min; Afridi, Ali; Gillis, Tammy; Guide, Jolene R; Dempsey, Stephani; Lager, Brenda; Alonso, Isabel; Wheeler, Vanessa C; Pinto, Ricardo Mouro

    2017-02-01

    Huntington's disease (HD) is a neurodegenerative disorder caused by the expansion of a CAG trinucleotide repeat in exon 1 of the HTT gene. Longer repeat sizes are associated with increased disease penetrance and earlier ages of onset. Intergenerationally unstable transmissions are common in HD families, partly underlying the genetic anticipation seen in this disorder. HD CAG knock-in mouse models also exhibit a propensity for intergenerational repeat size changes. In this work, we examine intergenerational instability of the CAG repeat in over 20,000 transmissions in the largest HD knock-in mouse model breeding datasets reported to date. We confirmed previous observations that parental sex drives the relative ratio of expansions and contractions. The large datasets further allowed us to distinguish effects of paternal CAG repeat length on the magnitude and frequency of expansions and contractions, as well as the identification of large repeat size jumps in the knock-in models. Distinct degrees of intergenerational instability were observed between knock-in mice of six background strains, indicating the occurrence of trans-acting genetic modifiers. We also found that lines harboring a neomycin resistance cassette upstream of Htt showed reduced expansion frequency, indicative of a contributing role for sequences in cis, with the expanded repeat as modifiers of intergenerational instability. These results provide a basis for further understanding of the mechanisms underlying intergenerational repeat instability. Copyright © 2017 by the Genetics Society of America.

  19. Influence of drill helical direction on exit damage development in drilling carbon fiber reinforced plastic

    Science.gov (United States)

    Bai, Y.; Jia, Z. Y.; Wang, F. J.; Fu, R.; Guo, H. B.; Cheng, D.; Zhang, B. Y.

    2017-06-01

    Drilling is inevitable for CFRP components’ assembling process in the aviation industry. The exit damage frequently occurs and affects the load carrying capacity of components. Consequently, it is of great urgency to enhance drilling exit quality on CFRP components. The article aims to guide the reasonable choice of drill helical direction and effectively reduce exit damage. Exit observation experiments are carried out with left-hand helical, right-hand helical and straight one-shot drill drilling T800S CFRP laminates separately. The development rules of exit damage and delamination factor curves are obtained. Combined with loading conditions and fracture modes of push-out burrs, and thrust force curves, the influence of drill helical direction on exit damage development is derived. It is found that the main fracture modes for left-hand helical, right-hand helical, and straight one-shot drill are mode I, extrusive fracture, mode III respectively. Among them, mode III has the least effect on exit damage development. Meanwhile, the changing rate of thrust force is relative slow for right-hand helical and straight one-shot drill in the thrust force increasing phase of stage II, which is disadvantaged for exit damage development. Therefore, straight one-shot drill’s exit quality is the best.

  20. 75 FR 11939 - Fisher & Paykel Appliances, Inc., Huntington Beach, CA; Notice of Termination of Investigation

    Science.gov (United States)

    2010-03-12

    ... DEPARTMENT OF LABOR Employment and Training Administration [TA-W-71,749] Fisher & Paykel Appliances, Inc., Huntington Beach, CA; Notice of Termination of Investigation Pursuant to Section 221 of the Trade Act of 1974, as amended, an investigation was initiated in response to a petition filed on July 21...

  1. Dynamics of the connectome in Huntington's disease: A longitudinal diffusion MRI study

    OpenAIRE

    Odish, Omar F.F.; Caeyenberghs, Karen; Hosseini, Hadi; van den Bogaard, Simon J.A.; Roos, Raymund A.C.; Leemans, Alexander

    2015-01-01

    Abstract Objectives To longitudinally investigate the connectome in different stages of Huntington's disease (HD) by applying graph theoretical analysis to diffusion MRI data. Experimental design We constructed weighted structural networks and calculated their topological properties. Twenty-two premanifest (preHD), 10 early manifest HD and 24 healthy controls completed baseline and 2 year follow-up scans. We stratified the preHD group based on their predicted years to disease onset into a far...

  2. Epigenetics as a First Exit Problem

    Science.gov (United States)

    Aurell, E.; Sneppen, K.

    2002-01-01

    We develop a framework to discuss the stability of epigenetic states as first exit problems in dynamical systems with noise. We consider in particular the stability of the lysogenic state of the λ prophage. The formalism defines a quantitative measure of robustness of inherited states.

  3. Evaluation of six TPS algorithms in computing entrance and exit doses

    Science.gov (United States)

    Metwaly, Mohamed; Glegg, Martin; Baggarley, Shaun P.; Elliott, Alex

    2014-01-01

    Entrance and exit doses are commonly measured in in vivo dosimetry for comparison with expected values, usually generated by the treatment planning system (TPS), to verify accuracy of treatment delivery. This report aims to evaluate the accuracy of six TPS algorithms in computing entrance and exit doses for a 6 MV beam. The algorithms tested were: pencil beam convolution (Eclipse PBC), analytical anisotropic algorithm (Eclipse AAA), AcurosXB (Eclipse AXB), FFT convolution (XiO Convolution), multigrid superposition (XiO Superposition), and Monte Carlo photon (Monaco MC). Measurements with ionization chamber (IC) and diode detector in water phantoms were used as a reference. Comparisons were done in terms of central axis point dose, 1D relative profiles, and 2D absolute gamma analysis. Entrance doses computed by all TPS algorithms agreed to within 2% of the measured values. Exit doses computed by XiO Convolution, XiO Superposition, Eclipse AXB, and Monaco MC agreed with the IC measured doses to within 2%‐3%. Meanwhile, Eclipse PBC and Eclipse AAA computed exit doses were higher than the IC measured doses by up to 5.3% and 4.8%, respectively. Both algorithms assume that full backscatter exists even at the exit level, leading to an overestimation of exit doses. Despite good agreements at the central axis for Eclipse AXB and Monaco MC, 1D relative comparisons showed profiles mismatched at depths beyond 11.5 cm. Overall, the 2D absolute gamma (3%/3 mm) pass rates were better for Monaco MC, while Eclipse AXB failed mostly at the outer 20% of the field area. The findings of this study serve as a useful baseline for the implementation of entrance and exit in vivo dosimetry in clinical departments utilizing any of these six common TPS algorithms for reference comparison. PACS numbers: 87.55.‐x, 87.55.D‐, 87.55.N‐, 87.53.Bn PMID:24892349

  4. Structural imaging in premanifest and manifest Huntington disease.

    Science.gov (United States)

    Scahill, Rachael I; Andre, Ralph; Tabrizi, Sarah J; Aylward, Elizabeth H

    2017-01-01

    Huntington disease (HD) neuropathology has a devastating effect on brain structure and consequently brain function; neuroimaging provides a means to assess these effects in gene carriers. In this chapter we first outline the unique utility of structural imaging in understanding HD and discuss some of the acquisition and analysis techniques currently available. We review the existing literature to summarize what we know so far about structural brain changes across the spectrum of disease from premanifest through to manifest disease. We then consider how these neuroimaging findings relate to patient function and nonimaging biomarkers, and can be used to predict disease onset. Finally we review the utility of imaging measures for assessment of treatment efficacy in clinical trials. Copyright © 2017 Elsevier B.V. All rights reserved.

  5. Nucleic Acid-Based Therapy Approaches for Huntington's Disease

    Directory of Open Access Journals (Sweden)

    Tatyana Vagner

    2012-01-01

    Full Text Available Huntington's disease (HD is caused by a dominant mutation that results in an unstable expansion of a CAG repeat in the huntingtin gene leading to a toxic gain of function in huntingtin protein which causes massive neurodegeneration mainly in the striatum and clinical symptoms associated with the disease. Since the mutation has multiple effects in the cell and the precise mechanism of the disease remains to be elucidated, gene therapy approaches have been developed that intervene in different aspects of the condition. These approaches include increasing expression of growth factors, decreasing levels of mutant huntingtin, and restoring cell metabolism and transcriptional balance. The aim of this paper is to outline the nucleic acid-based therapeutic strategies that have been tested to date.

  6. Deficient Rab11 activity underlies glucose hypometabolism in primary neurons of Huntington's disease mice

    Energy Technology Data Exchange (ETDEWEB)

    Li, Xueyi, E-mail: xli12@partners.org [Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA 02129 (United States); Valencia, Antonio; McClory, Hollis; Sapp, Ellen; Kegel, Kimberly B. [Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA 02129 (United States); DiFiglia, Marian, E-mail: difiglia@helix.mgh.harvard.edu [Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Charlestown, MA 02129 (United States)

    2012-05-18

    Highlights: Black-Right-Pointing-Pointer Primary Huntington's disease neurons are impaired in taking up glucose. Black-Right-Pointing-Pointer Rab11 modulates glucose uptake in neurons. Black-Right-Pointing-Pointer Increasing Rab11 activity attenuates the glucose uptake defect in disease neurons. Black-Right-Pointing-Pointer We provide a novel mechanism for glucose hypometabolism in Huntington's disease. -- Abstract: Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin gene. Positron emission tomography studies have revealed a decline in glucose metabolism in the brain of patients with HD by a mechanism that has not been established. We examined glucose utilization in embryonic primary cortical neurons of wild-type (WT) and HD knock-in mice, which have 140 CAG repeats inserted in the endogenous mouse huntingtin gene (HD{sup 140Q/140Q}). Primary HD{sup 140Q/140Q} cortical neurons took up significantly less glucose than did WT neurons. Expression of permanently inactive and permanently active forms of Rab11 correspondingly altered glucose uptake in WT neurons, suggesting that normal activity of Rab11 is needed for neuronal uptake of glucose. It is known that Rab11 activity is diminished in HD{sup 140Q/140Q} neurons. Expression of dominant active Rab11 to enhance the activity of Rab11 normalized glucose uptake in HD{sup 140Q/140Q} neurons. These results suggest that deficient activity of Rab11 is a novel mechanism for glucose hypometabolism in HD.

  7. After exit: Academic achievement patterns of former English language learners.

    Directory of Open Access Journals (Sweden)

    Ester J. de Jong

    2004-09-01

    Full Text Available With few exceptions, accountability systems for programs for English language learners (ELLs have focused on the achievement patterns of ELLs who are still considered “limited English proficient” and program evaluations have been unable to answer the question whether ELLs actually catch up with English proficient peers after attending a bilingual or English as a Second Language (ESL program. Disaggregating data for former ELLs can therefore provide important information for long-term district and program accountability. The study was concerned with the achievement patterns in English language arts, Math, and Science of former ELLs who attended a bilingual and a English as a Second Language (ESL program. It also explored whether length of program participation and grade level exited played a significant role in predicting academic achievement patterns for these exited students. Results indicate that 4th grade students more closely paralleled non- ELL students’ achievement patterns than 8th grade students, particularly for the BE students. While length of program participation is not a significant predictor of former ELLs’ academic success, exit grade does emerge as an important variable to take into consideration in setting exit guidelines.

  8. Periodontitis determining the onset and progression of Huntington's disease: review of the literature

    Directory of Open Access Journals (Sweden)

    María Lourdes Rodríguez Coyago

    2015-10-01

    Full Text Available La enfermedad de Hungtinton es un trastorno neurodegenerativo, causado por la expansión de un triplete de citosina, adenina, guanina en el gen de la huntingtina. Se manifiesta con un deterioro físico, cognitivo y psiquiátrico a distintas edades en el adulto, con un pronóstico fatal. Además del número de repeticiones del triplete, existirían otros factores que explicarían el inicio de esta enfermedad a más temprana edad. Se sabe que la neuroinflamación es un protagonista en los trastornos neurodegenerativos, no siendo la enfermedad de Huntington una excepción. La neuroinflamación exacerba el daño neuronal producido por la mutación, al existir activación aberrante de la célula microglía, disfunción de astrocitos y células dendríticas; compromiso de la barrera hematoencefálica y activación de complemento, todas ellas como efecto directo e indirecto de la mutante y otros estímulos como infecciones crónicas. Es el interés del presente trabajo analizar la periodontitis, como modelo de infección bucodental crónica y fuente de inflamación sistémica. Hipotetizamos que el potencial rol de la periodontitis en la enfermedad de Huntington y los mecanismos por los cuales contribuiría a la manifestación temprana y progreso de dicha enfermedad, para lo cual se consideraron revisiones sistemáticas, metanálisis y estudios experimentales publicados tanto en español como en inglés obtenidos del PubMed y SciELO. Son diversos los mecanismos que generan inflamación en el cerebro de estos pacientes, adquiriendo especial protagonismo los mecanismos de la inmunidad innata. Las infecciones buco dentarias crónicas, como la enfermedad periodontal, pueden constituir un factor exacerbante de la neuroinflamación que per se asocia la enfermedad de Huntington.

  9. Drink Specials and the Intoxication Levels of Patrons Exiting College Bars

    Science.gov (United States)

    Thombs, Dennis L.; Dodd, Virginia; Porkorny, Steven B.; Omli, Morrow R.; O'Mara, Ryan; Webb, Monica C.; Lacaci, Diana M.; Werch, Chad

    2008-01-01

    Objectives: To determine whether drink specials independently increase patrons' risk of achieving a high level of intoxication upon exiting drinking establishments. Methods: In a campus community, data were collected from exiting patrons (N=291) via sidewalk interviews and breath tests on 6 nights of 2 consecutive semesters. Results: A…

  10. 14 CFR 135.129 - Exit seating.

    Science.gov (United States)

    2010-01-01

    ... emergency evacuation provided by the certificate holder in printed or graphic form or the ability to... accordance with this section. (d) Each certificate holder shall include on passenger information cards.... (e) Each certificate holder shall include on passenger information cards, at each exit seat— (1) In...

  11. Door locking and exit security measures on acute psychiatric admission wards

    NARCIS (Netherlands)

    Nijman, H.L.I.; Bowers, L.; Haglund, K.; Muir-Cochrane, E.; Simpson, A.; Merwe, M. van der

    2011-01-01

    Locking the exit doors of psychiatric wards is believed to reduce the risk of patients absconding. The aims of the study were to investigate both the prevalence of door locking and other exit security measures on UK admission wards, as well as whether door locking appears to be effective in keeping

  12. EXIT Chart Analysis of Binary Message-Passing Decoders

    DEFF Research Database (Denmark)

    Lechner, Gottfried; Pedersen, Troels; Kramer, Gerhard

    2007-01-01

    Binary message-passing decoders for LDPC codes are analyzed using EXIT charts. For the analysis, the variable node decoder performs all computations in the L-value domain. For the special case of a hard decision channel, this leads to the well know Gallager B algorithm, while the analysis can...... be extended to channels with larger output alphabets. By increasing the output alphabet from hard decisions to four symbols, a gain of more than 1.0 dB is achieved using optimized codes. For this code optimization, the mixing property of EXIT functions has to be modified to the case of binary message......-passing decoders....

  13. Predicting The Exit Time Of Employees In An Organization Using Statistical Model

    Directory of Open Access Journals (Sweden)

    Ahmed Al Kuwaiti

    2015-08-01

    Full Text Available Employees are considered as an asset to any organization and each organization provide a better and flexible working environment to retain its best and resourceful workforce. As such continuous efforts are being taken to avoid or extend the exitwithdrawal of employees from the organization. Human resource managers are facing a challenge to predict the exit time of employees and there is no precise model existing at present in the literature. This study has been conducted to predict the probability of exit of an employee in an organization using appropriate statistical model. Accordingly authors designed a model using Additive Weibull distribution to predict the expected exit time of employee in an organization. In addition a Shock model approach is also executed to check how well the Additive Weibull distribution suits in an organization. The analytical results showed that when the inter-arrival time increases the expected time for the employees to exit also increases. This study concluded that Additive Weibull distribution can be considered as an alternative in the place of Shock model approach to predict the exit time of employee in an organization.

  14. 14 CFR 121.585 - Exit seating.

    Science.gov (United States)

    2010-01-01

    ... section and related to emergency evacuation provided by the certificate holder in printed or graphic form... include on passenger information cards, presented in the language in which briefings and oral commands are... passenger information cards, at each exit seat— (1) In the primary language in which emergency commands are...

  15. Dysfunctional mitochondrial respiration in the striatum of the Huntington's disease transgenic R6/2 mouse model

    DEFF Research Database (Denmark)

    Aidt, Frederik Heurlin; Nielsen, Signe Marie Borch; Kanters, Jørgen

    2013-01-01

    Metabolic dysfunction and mitochondrial involvement are recognised as part of the pathology in Huntington's Disease (HD). Post-mortem examinations of the striatum from end-stage HD patients have shown a decrease in the in vitro activity of complexes II, III and IV of the electron transport system...

  16. Aggregating job exit statuses of a plurality of compute nodes executing a parallel application

    Science.gov (United States)

    Aho, Michael E.; Attinella, John E.; Gooding, Thomas M.; Mundy, Michael B.

    2015-07-21

    Aggregating job exit statuses of a plurality of compute nodes executing a parallel application, including: identifying a subset of compute nodes in the parallel computer to execute the parallel application; selecting one compute node in the subset of compute nodes in the parallel computer as a job leader compute node; initiating execution of the parallel application on the subset of compute nodes; receiving an exit status from each compute node in the subset of compute nodes, where the exit status for each compute node includes information describing execution of some portion of the parallel application by the compute node; aggregating each exit status from each compute node in the subset of compute nodes; and sending an aggregated exit status for the subset of compute nodes in the parallel computer.

  17. Comparison of exit time moment spectra for extrinsic metric balls

    DEFF Research Database (Denmark)

    Hurtado, Ana; Markvorsen, Steen; Palmer, Vicente

    2012-01-01

    We prove explicit upper and lower bounds for the $L^1$-moment spectra for the Brownian motion exit time from extrinsic metric balls of submanifolds $P^m$ in ambient Riemannian spaces $N^n$. We assume that $P$ and $N$ both have controlled radial curvatures (mean curvature and sectional curvature...... obtain new intrinsic comparison results for the exit time spectra for metric balls in the ambient manifolds $N^n$ themselves....

  18. Formal procedures of the exit from a community for a university social media agency

    OpenAIRE

    Peleschyshyn, Andriy; Peleschyshyn, Oksana; Korzh, Roman

    2016-01-01

    The aim of the paper is to consider the process of exiting of a university from an online community. The importance of performing the task of exiting in a proper way is proved. Preconditions and risks of the process are investigated. All the stages of the suggested algorithm for exiting from online community are scrutinized.

  19. Efficiency, Leverage and Exit: The Role of Information Asymmetry in Concentrated Industries

    DEFF Research Database (Denmark)

    Siyahhan, Baran

    2011-01-01

    This paper develops a real options model of imperfect competition with asymmetric information that analyzes firms’ exit decisions. Optimal exit decision is linked to firm characteristics such as financial leverage and efficiency. The model shows that informational asymmetries can lead more...

  20. Efficiency, Leverage and Exit: The Role of Information Asymmetry in Concentrated Industries

    DEFF Research Database (Denmark)

    Siyahhan, Baran

    This paper develops a real options model of imperfect competition with asymmetric information that analyzes firms’ exit decisions. Optimal exit decision is linked to firm characteristics such as financial leverage and efficiency. The model shows that informational asymmetries can lead more...

  1. Nonparametric estimation for censored mixture data with application to the Cooperative Huntington's Observational Research Trial.

    Science.gov (United States)

    Wang, Yuanjia; Garcia, Tanya P; Ma, Yanyuan

    2012-01-01

    This work presents methods for estimating genotype-specific distributions from genetic epidemiology studies where the event times are subject to right censoring, the genotypes are not directly observed, and the data arise from a mixture of scientifically meaningful subpopulations. Examples of such studies include kin-cohort studies and quantitative trait locus (QTL) studies. Current methods for analyzing censored mixture data include two types of nonparametric maximum likelihood estimators (NPMLEs) which do not make parametric assumptions on the genotype-specific density functions. Although both NPMLEs are commonly used, we show that one is inefficient and the other inconsistent. To overcome these deficiencies, we propose three classes of consistent nonparametric estimators which do not assume parametric density models and are easy to implement. They are based on the inverse probability weighting (IPW), augmented IPW (AIPW), and nonparametric imputation (IMP). The AIPW achieves the efficiency bound without additional modeling assumptions. Extensive simulation experiments demonstrate satisfactory performance of these estimators even when the data are heavily censored. We apply these estimators to the Cooperative Huntington's Observational Research Trial (COHORT), and provide age-specific estimates of the effect of mutation in the Huntington gene on mortality using a sample of family members. The close approximation of the estimated non-carrier survival rates to that of the U.S. population indicates small ascertainment bias in the COHORT family sample. Our analyses underscore an elevated risk of death in Huntington gene mutation carriers compared to non-carriers for a wide age range, and suggest that the mutation equally affects survival rates in both genders. The estimated survival rates are useful in genetic counseling for providing guidelines on interpreting the risk of death associated with a positive genetic testing, and in facilitating future subjects at risk

  2. Alpha-7 nicotinic acetylcholine receptor agonist treatment in a rat model of Huntington's disease and involvement of heme oxygenase-1

    Directory of Open Access Journals (Sweden)

    Laura Foucault-Fruchard

    2018-01-01

    Full Text Available Neuroinflammation is a common element involved in the pathophysiology of neurodegenerative diseases. We recently reported that repeated alpha-7 nicotinic acetylcholine receptor (α7nAChR activations by a potent agonist such as PHA 543613 in quinolinic acid-injured rats exhibited protective effects on neurons. To further investigate the underlying mechanism, we established rat models of early-stage Huntington's disease by injection of quinolinic acid into the right striatum and then intraperitoneally injected 12 mg/kg PHA 543613 or sterile water, twice a day during 4 days. Western blot assay results showed that the expression of heme oxygenase-1 (HO-1, the key component of the cholinergic anti-inflammatory pathway, in the right striatum of rat models of Huntington's disease subjected to intraperitoneal injection of PHA 543613 for 4 days was significantly increased compared to the control rats receiving intraperitoneal injection of sterile water, and that the increase in HO-1 expression was independent of change in α7nAChR expression. These findings suggest that HO-1 expression is unrelated to α7nAChR density and the increase in HO-1 expression likely contributes to α7nAChR activation-related neuroprotective effect in early-stage Huntington's disease.

  3. Insights into gait disorders: walking variability using phase plot analysis, Huntington's disease.

    Science.gov (United States)

    Collett, Johnny; Esser, Patrick; Khalil, Hanan; Busse, Monica; Quinn, Lori; DeBono, Katy; Rosser, Anne; Nemeth, Andrea H; Dawes, Helen

    2014-09-01

    Huntington's disease (HD) is a progressive inherited neurodegenerative disorder. Identifying sensitive methodologies to quantitatively measure early motor changes have been difficult to develop. This exploratory observational study investigated gait variability and symmetry in HD using phase plot analysis. We measured the walking of 22 controls and 35 HD gene carriers (7 premanifest (PreHD)), 16 early/mid (HD1) and 12 late stage (HD2) in Oxford and Cardiff, UK. The unified Huntington's disease rating scale-total motor scores (UHDRS-TMS) and disease burden scores (DBS) were used to quantify disease severity. Data was collected during a clinical walk test (8.8 or 10 m) using an inertial measurement unit attached to the trunk. The 6 middle strides were used to calculate gait variability determined by spatiotemporal parameters (co-efficient of variation (CoV)) and phase plot analysis. Phase plots considered the variability in consecutive wave forms from vertical movement and were quantified by SDA (spatiotemporal variability), SDB (temporal variability), ratio ∀ (ratio SDA:SDB) and Δangleβ (symmetry). Step time CoV was greater in manifest HD (p0.05). Phase plot analysis identified differences between manifest HD and controls for SDB, Ratio ∀ and Δangle (all pplot analysis may be a sensitive method of detecting gait changes in HD and can be performed quickly during clinical walking tests. Copyright © 2014 Elsevier B.V. All rights reserved.

  4. Toward 3D structural information from quantitative electron exit wave analysis

    International Nuclear Information System (INIS)

    Borisenko, Konstantin B; Moldovan, Grigore; Kirkland, Angus I; Wang, Amy; Van Dyck, Dirk; Chen, Fu-Rong

    2012-01-01

    Simulations show that using a new direct imaging detector and accurate exit wave restoration algorithms allows nearly quantitative restoration of electron exit wave phase, which can be regarded as only qualitative for conventional indirect imaging cameras. This opens up a possibility of extracting accurate information on 3D atomic structure of the sample even from a single projection.

  5. Association Between Health Plan Exit From Medicaid Managed Care and Quality of Care, 2006-2014.

    Science.gov (United States)

    Ndumele, Chima D; Schpero, William L; Schlesinger, Mark J; Trivedi, Amal N

    2017-06-27

    State Medicaid programs have increasingly contracted with insurers to provide medical care services for enrollees (Medicaid managed care plans). Insurers that provide these plans can exit Medicaid programs each year, with unclear effects on quality of care and health care experiences. To determine the frequency and interstate variation of health plan exit from Medicaid managed care and evaluate the relationship between health plan exit and market-level quality. Retrospective cohort of all comprehensive Medicaid managed care plans (N = 390) during the interval 2006-2014. Plan exit, defined as the withdrawal of a managed care plan from a state's Medicaid program. Eight measures from the Healthcare Effectiveness Data and Information Set were used to construct 3 composite indicators of quality (preventive care, chronic disease care management, and maternity care). Four measures from the Consumer Assessment of Healthcare Providers and Systems were combined into a composite indicator of patient experience, reflecting the proportion of beneficiaries rating experiences as 8 or above on a 0-to-10-point scale. Outcome data were available for 248 plans (68% of plans operating prior to 2014, representing 78% of beneficiaries). Of the 366 comprehensive Medicaid managed care plans operating prior to 2014, 106 exited Medicaid. These exiting plans enrolled 4 848 310 Medicaid beneficiaries, with a mean of 606 039 beneficiaries affected by plan exits annually. Six states had a mean of greater than 10% of Medicaid managed care recipients enrolled in plans that exited, whereas 10 states experienced no plan exits. Plans that exited from a state's Medicaid market performed significantly worse prior to exiting than those that remained in terms of preventive care (57.5% vs 60.4%; difference, 2.9% [95% CI, 0.3% to 5.5%]), maternity care (69.7% vs 73.6%; difference, 3.8% [95% CI, 1.7% to 6.0%]), and patient experience (73.5% vs 74.8%; difference, 1.3% [95% CI, 0.6% to 1

  6. Stor forskel på kommuners bande-exit

    DEFF Research Database (Denmark)

    Mørck, Line Lerche

    2015-01-01

    DEBAT: Kommunerne har meget forskellige exit-tilbud til bande- og rockermedlemmer. Der er brug for mere indgående forskning, så vi ved, hvad der virker bedst, skriver Line Lerche Mørck, lektor i pædagogisk psykologi....

  7. The roles of family members, health care workers, and others in decision-making processes about genetic testing among individuals at risk for Huntington disease.

    Science.gov (United States)

    Klitzman, Robert; Thorne, Deborah; Williamson, Jennifer; Marder, Karen

    2007-06-01

    To understand how individuals at risk for Huntington disease view the roles of others, e.g., family members and health care workers, in decision making about genetic testing. Twenty-one individuals (eight mutation-positive, four mutation-negative, and nine not tested) were interviewed for approximately 2 hours each. Interviewees illuminated several key aspects of the roles of family members and health care workers (in genetics and other fields) in decision making about testing that have been underexplored. Family members often felt strongly about whether an individual should get tested. Health care workers provided information and assistance with decision making and mental health referrals that were often helpful. Yet health care workers varied in knowledge and sensitivity regarding testing issues, and the quality of counseling and testing experiences can range widely. At times, health care workers without specialized knowledge of Huntington disease offered opinions of whether to test. Input from families and health care workers could also conflict with each other and with an individual's own preferences. Larger institutional and geographic contexts shaped decisions as well. Decision-making theories applied to Huntington disease testing have frequently drawn on psychological models, yet the current data highlight the importance of social contexts and relationships in testing decisions. This report, the first to our knowledge to explore individuals' perceptions of social factors (particularly family and health care worker involvement) in Huntington disease testing decisions, has critical implications for practice, education, research, and policy.

  8. Rhes deletion is neuroprotective in the 3-nitropropionic acid model of Huntington's disease

    OpenAIRE

    Mealer, Robert G.; Subramaniam, Srinivasa; Snyder, Solomon H.

    2013-01-01

    Although the mutated protein causing Huntington's disease (HD) is expressed throughout the body, the major pathology of HD is localized to the striatum of the brain. We previously reported that the striatal-enriched protein Rhes binds the mutated huntingtin protein and enhances its cytotoxicity. We now demonstrate that Rhes-deleted mice are dramatically protected from neurotoxicity and motor dysfunction in a striatal-specific model of HD elicited by 3-nitropropionic acid. This finding suggest...

  9. 14 CFR 23.805 - Flightcrew emergency exits.

    Science.gov (United States)

    2010-01-01

    ... emergency exit must be located to allow rapid evacuation of the crew and have a size and shape of at least a... than six feet from the ground, an assisting means must be provided. The assisting means may be a rope...

  10. Exit Polling in an Emergent Democracy: The Complex Case of Ukraine

    Directory of Open Access Journals (Sweden)

    Natalya Nikolaevna Kharchenko

    2010-05-01

    Full Text Available This report considers the methodological specifics of conducting exit polls to verify election returns, mainly using an example of election campaigns in Ukraine from 2002-2007. The deepest public resonance was aroused by exit polls conducted after the second round of Ukrainian presidential elections in 2004. These polls were one of the factors, which led to massive demonstrations (the so-called "Orange Revolution" and the revocation of election results. The authors show that in the environment of administrative pressure on voters, governmental control of mass media, and severe political struggle, inaccuracy increases. Thus, to acquire reliable and valid information, the polling methods used must be modified. The recommendations given for the methodology of conducting exit polls may be useful for emergent democratic countries.

  11. Destination and source memory in Huntington's disease.

    Science.gov (United States)

    El Haj, Mohamad; Caillaud, Marie; Verny, Christophe; Fasotti, Luciano; Allain, Philippe

    2016-03-01

    Destination memory refers to the recall of the destination of previously relayed information, and source memory refers to the recollection of the origin of received information. We compared both memory systems in Huntington's disease (HD) participants. For this, HD participants and healthy adults had to put 12 items in a black or a white box (destination task), and to extract another 12 items from a blue or a red box (source task). Afterwards, they had to decide in which box each item had previously been deposited (destination memory), and from which box each item had previously been extracted (source memory). HD participants showed poorer source as well as destination recall performance than healthy adults in the proposed tasks. Correlation analysis showed that destination recall was significantly correlated with episodic recall in HD participants. Destination memory impairment in HD participants seems to be considerably influenced by their episodic memory performance. © 2014 The British Psychological Society.

  12. Assessing Impairment of Executive Function and Psychomotor Speed in Premanifest and Manifest Huntington's Disease Gene-expansion Carriers

    DEFF Research Database (Denmark)

    Unmack Larsen, Ida; Vinther-Jensen, Tua; Gade, Anders

    2015-01-01

    Executive functions (EF) and psychomotor speed (PMS) has been widely studied in Huntington's disease (HD). Most studies have focused on finding markers of disease progression by comparing group means at different disease stages. Our aim was to investigate performances on nine measures of EF and PMS...

  13. A Diversity Exit Interview/Survey for the Military

    National Research Council Canada - National Science Library

    Knouse, Stephen

    2001-01-01

    The exit interview and survey are means for identifying organizational problems, including diversity issues, through individuals separating from the organization, who are in a unique position to supply candid feedback...

  14. Symmetry associated with symmetry break: Revisiting ants and humans escaping from multiple-exit rooms

    Science.gov (United States)

    Ji, Q.; Xin, C.; Tang, S. X.; Huang, J. P.

    2018-02-01

    Crowd panic has incurred massive injuries or deaths throughout the world, and thus understanding it is particularly important. It is now a common knowledge that crowd panic induces "symmetry break" in which some exits are jammed while others are underutilized. Amazingly, here we show, by experiment, simulation and theory, that a class of symmetry patterns come to appear for ants and humans escaping from multiple-exit rooms while the symmetry break exists. Our symmetry pattern is described by the fact that the ratio between the ensemble-averaging numbers of ants or humans escaping from different exits is equal to the ratio between the widths of the exits. The mechanism lies in the effect of heterogeneous preferences of agents with limited information for achieving the Nash equilibrium. This work offers new insights into how to improve public safety because large public areas are always equipped with multiple exits, and it also brings an ensemble-averaging method for seeking symmetry associated with symmetry breaking.

  15. Exits from Temporary Jobs in Europe: A Competing Risks Analysis

    DEFF Research Database (Denmark)

    D'Addio, Anna Christina; Rosholm, Michael

    2005-01-01

    We study transitions out of temporary jobs using the waves 1994-1999 of the European Community Household Panel applying a discrete time duration model. Specifically, we use a multinomial logitmodel distinguishing between exits into permanent employment and non-employment. Two different specificat......We study transitions out of temporary jobs using the waves 1994-1999 of the European Community Household Panel applying a discrete time duration model. Specifically, we use a multinomial logitmodel distinguishing between exits into permanent employment and non-employment. Two different...

  16. Adolescents Exiting Homelessness over Two Years: The Risk Amplification and Abatement Model

    Science.gov (United States)

    Milburn, Norweeta G.; Rice, Eric; Rotheram-Borus, Mary Jane; Mallett, Shelley; Rosenthal, Doreen; Batterham, Phillip; May, Susanne J.; Witkin, Andrea; Duan, Naihua

    2009-01-01

    The Risk Amplification and Abatement Model (RAAM) demonstrates that negative contact with socializing agents amplify risk, while positive contact abates risk for homeless adolescents. To test this model, the likelihood of exiting homelessness and returning to familial housing at 2 years and stably exiting over time are examined with longitudinal…

  17. Sodium phenylbutyrate in Huntington's disease: a dose-finding study.

    Science.gov (United States)

    Hogarth, Penelope; Lovrecic, Luca; Krainc, Dimitri

    2007-10-15

    Transcriptional dysregulation in Huntington's disease (HD) is mediated in part by aberrant patterns of histone acetylation. We performed a dose-finding study in human HD of sodium phenylbutyrate (SPB), a histone deacetylase inhibitor that ameliorates the HD phenotype in animal models. We used a dose-escalation/de-escalation design, using prespecified toxicity criteria and standard clinical and laboratory safety measures. The maximum tolerated dose was 15 g/day. At higher doses, toxicity included vomiting, lightheadedness, confusion, and gait instability. We saw no significant laboratory or electrocardiographic abnormalities. Gene expression changes in blood suggested an inverse dose-response. In conclusion, SPB at 12 to 15 g/day appears to be safe and well-tolerated in human HD. 2007 Movement Disorder Society

  18. [Speed of ocular saccades in Huntington disease. Prospective study].

    Science.gov (United States)

    García Ruiz, P J; Cenjor, C; Ulmer, E; Hernández, J; Cantarero, S; Fanjul, S; García de Yébenes, J

    2001-02-01

    Oculomotor abnormalities, especially slow saccades, have long been recognized in Huntington's disease (HD). To study prospectively horizontal saccade velocity by videonystagmography in 21 patients with genetically confirmed HD. The study included a baseline analysis and a second evaluation after 18.8 +/- 7.1 months. We included a control group of 15 subjects. HD group exhibited decreased saccade velocity when compared with that from a control group (for predictive and unpredictive target). HD patients showed decreased saccade velocity with the passage of time (for predictive target, p < 0.01). Finally we found statistical significant correlation between saccade velocity and triplet length. The measurement of saccade velocity might be an objective method to study the natural evolution of HD, and thus evaluate the effectiveness of future therapies.

  19. Transgenic C. elegans dauer larvae expressing hookworm phospho null DAF-16/FoxO exit dauer.

    Directory of Open Access Journals (Sweden)

    Verena Gelmedin

    Full Text Available Parasitic hookworms and the free-living model nematode Caenorhabtidis elegans share a developmental arrested stage, called the dauer stage in C. elegans and the infective third-stage larva (L3 in hookworms. One of the key transcription factors that regulate entrance to and exit from developmental arrest is the forkhead transcription factor DAF-16/FoxO. During the dauer stage, DAF-16 is activated and localized in the nucleus. DAF-16 is negatively regulated by phosphorylation by the upstream kinase AKT, which causes DAF-16 to localize out of the nucleus and the worm to exit from dauer. DAF-16 is conserved in hookworms, and hypothesized to control recovery from L3 arrest during infection. Lacking reverse genetic techniques for use in hookworms, we used C. elegans complementation assays to investigate the function of Ancylostoma caninum DAF-16 during entrance and exit from L3 developmental arrest. We performed dauer switching assays and observed the restoration of the dauer phenotype when Ac-DAF-16 was expressed in temperature-sensitive dauer defective C. elegans daf-2(e1370;daf-16(mu86 mutants. AKT phosphorylation site mutants of Ac-DAF-16 were also able to restore the dauer phenotype, but surprisingly allowed dauer exit when temperatures were lowered. We used fluorescence microscopy to localize DAF-16 during dauer and exit from dauer in C. elegans DAF-16 mutant worms expressing Ac-DAF-16, and found that Ac-DAF-16 exited the nucleus during dauer exit. Surprisingly, Ac-DAF-16 with mutated AKT phosphorylation sites also exited the nucleus during dauer exit. Our results suggest that another mechanism may be involved in the regulation DAF-16 nuclear localization during recovery from developmental arrest.

  20. Study on mobile phone use while driving in a sample of Iranian drivers.

    Science.gov (United States)

    Arvin, Ramin; Khademi, Mostafa; Razi-Ardakani, Hesamoddin

    2017-06-01

    The use of cell phone is a significant source of driver distraction. Phone use while driving can impair a number of factors critical for safe driving which can cause serious traffic safety problems. The objective of this paper was to investigate the frequency of using cell phones while driving in Iran's roads through an observational survey with a random sample of drivers, to recognize contributing factors to cell phone usage and to understand the magnitude of the problem. A total of 1794 observations were collected from 12 sites at controlled intersections, entrance and exit points of highways. The cell phone use rate among drivers (talking or texting) was estimated at 10% which is significantly higher than that in other countries such as Australia, USA and Canada. Rate of cell phone use among younger drivers (14.15%) was higher in comparison with other groups. In order to identify factors affecting cell phone use while driving, a binary logit model is estimated. Variables which significantly contribute to the rate of using cell phone were found to be the age of driver, number of passengers, presence of kids under the age of 8, time of observation, vehicle price and type of car.

  1. Study of protein O-GlcNAcylation in the brain tissue in Huntington´s disease

    Czech Academy of Sciences Publication Activity Database

    Ondrušková, N.; Rodinová, M.; Kratochvílová, H.; Klempíř, J.; Roth, J.; Motlík, Jan; Radoslav, M.; Zeman, J.; Hansíková, H.

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 20-20 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk(CZ) 7F14308; GA MŠk ED2.1.00/03.0124 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * glycosylation * N-acetylglucosamine Subject RIV: FH - Neurology

  2. Grunting in genetically modified minipig animal model for Huntington ´s disease - a pilot experiment

    Czech Academy of Sciences Publication Activity Database

    Tykalová, T.; Hlavnička, J.; Mačáková, Monika; Baxa, Monika; Cmejla, R.; Motlík, Jan; Klempíř, J.; Rusz, J.

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 12-13 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) 7F14308 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * mitochondria * DNA damage Subject RIV: FH - Neurology

  3. Pre-clinical evaluation of AAV5-miHTT gene therapy of Huntington´s disease

    Czech Academy of Sciences Publication Activity Database

    Konstantinová, P.; Miniarikova, J.; Blits, B.; Zimmer, V.; Spoerl, A.; Southwell, A.; Hayden, M.; van Deventer, S.; Deglon, N.; Motlík, Jan; Juhás, Štefan; Juhásová, Jana; Richard, Ch.; Petry, H.

    2015-01-01

    Roč. 78, Supl 2 (2015), s. 8-8 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * gene therapy * AAV5-miHTT Subject RIV: EB - Genetics ; Molecular Biology

  4. Two-dimensional exit dosimetry using a liquid-filled electronic portal imaging device and a convolution model

    International Nuclear Information System (INIS)

    Boellaard, Ronald; Herk, Marcel van; Uiterwaal, Hans; Mijnheer, Ben

    1997-01-01

    Background and purpose: To determine the accuracy of two-dimensional exit dose measurements with an electronic portal imaging device, EPID, using a convolution model for a variety of clinically relevant situations. Materials and methods: Exit doses were derived from portal dose images, obtained with a liquid-filled EPID at distances of 50 cm or more behind the patient, by using a convolution model. The resulting on- and off-axis exit dose values were first compared with ionization chamber exit dose measurements for homogeneous and inhomogeneous phantoms in open and wedged 4,8 and 18 MV photon beams. The accuracy of the EPID exit dose measurements was then determined for a number of anthropomorphic phantoms (lung and larynx) irradiated under clinical conditions and for a few patients treated in an 8 MV beam. The latter results were compared with in vivo exit dose measurements using diodes. Results: The exit dose can be determined from portal images with an accuracy of 1.2% (1 SD) compared with ionization chamber measurements for open beams and homogeneous phantoms at all tested beam qualities. In the presence of wedges and for inhomogeneous phantoms the average relative accuracy slightly deteriorated to 1.7% (1 SD). For lung phantoms in a 4 MV beam a similar accuracy was obtained after refinement of our convolution model, which requires knowledge of the patient contour. Differences between diode and EPID exit dose measurements for an anthropomorphic lung phantom in an 8 MV beam were 2.5% at most, with an average agreement within 1% (1 SD). For larynx phantoms in a 4 MV beam exit doses obtained with an ionization chamber and EPID agreed within 1.5% (1 SD). Finally, exit doses in a few patients irradiated in an 8 MV beam could be determined with the EPID with an accuracy of 1.1% (1 SD) relative to exit dose measurements using diodes. Conclusions: Portal images, obtained with our EPID and analyzed with our convolution model, can be used to determine the exit dose

  5. El papel de la terapia ocupacional en la enfermedad de Huntington

    OpenAIRE

    Hernández Lozano, David; Fernández Hawrylak, María; Grau Rubio, Claudia

    2014-01-01

    se analiza el papel del terapeuta ocupacional en el trabajo con las personas afectadas por la enfermedad de Huntington. se realizó una investigación por objetivos a través del diseño y puesta en práctica de un programa de intervención encaminado a mitigar el deterioro producido por la enfermedad mediante el entrenamiento en actividades de la vida diaria y de estimulación cognitiva, y a desarrollar competencias en la familia. la investigación se desarrolló en la Asociación de corea de Huntingt...

  6. Explaining discrepancies in reproductive health indicators from population-based surveys and exit surveys: a case from Rwanda.

    Science.gov (United States)

    Meekers, D; Ogada, E A

    2001-06-01

    Reproductive health programmes often need exit surveys and population-based surveys for monitoring and evaluation. This study investigates why such studies produce discrepant estimates of condom use, sexual behaviour and condom brand knowledge, and discusses the implications for future use of exit surveys for programme monitoring. Logistic regression is used to explain differences between a household survey of 1295 persons and an exit survey among a random sample of 2550 consumers at retail outlets in RWANDA: Discrepancies in ever use of condoms and risky sexual behaviours are due to differences in socioeconomic status of the two samples. After controls, exit surveys at most outlet types have the same results as the household survey. Only exit surveys at bars, nightclubs and hotels yield significantly different estimates. However, the above-average knowledge of Prudence Plus condoms in the exit interviews is not attributable to socioeconomic or demographic variables, most likely because respondents have seen the product at the outlets. Information about condom use and sexual behaviour obtained from exit surveys appears as accurate as that obtained through household surveys. Nevertheless, exit surveys must be used cautiously. Because exit surveys may include wealthier and better-educated respondents, they are not representative of the general population. The composition of exit survey samples should be validated through existing household surveys. Comparisons across survey types are generally unadvisable, unless they control for sample differences. When generalizing to the population at large is not needed (e.g. for studies aimed at identifying the characteristics and behaviour of users of particular products or services), exit surveys can provide an appropriate alternative to household surveys.

  7. Internal consistency of a Brazilian version of the unified Huntington's disease rating scale Consistência interna da versão brasileira da escala unificada para avaliação da doença da Huntington

    Directory of Open Access Journals (Sweden)

    Vitor Tumas

    2004-12-01

    Full Text Available We evaluated the reliability of a translated Brazilian version of the Unified Huntington's Disease Rating Scale (UHDRS to establish the reproducibility of the scale in a population that differs substantially from that on which the scale was originally validated. After a training period with the video and guidelines requested from the Huntington Study Group, we applied the UHDRS, except for the cognitive tests, to a group of 21 Brazilian patients with a molecular diagnosis of Huntington's disease (HD. We found a high degree of internal consistency of the motor section of the UHDRS (Cronbach's alpha= 0.841. There was a negative correlation between the total motor score and the functional assessment, the independence scale and the functional capacity. There was a positive correlation between these 3 scales of functional evaluation and a negative correlation between the age of onset of the disease and the number of CAG repeats. The behavioral scale and disease duration were not correlated with any factor. The clinical characteristics of this sample of patients as described by the UHDRS were roughly similar to those reported in the original validation studies and the correlations described were similar to those reported previously. We conclude that the Brazilian version of the UHDRS is reliable and valid to study patients with HD in the Brazilian setting, that this sample of Brazilian patients had clinical characteristics similar to those observed in other world regions, as expected, and that the clinical training method used for the application of the UHDRS was effective to insure a high degree of clinical reproducibility.Nosso estudo avaliou a confiabilidade da versão brasileira da escala unificada para avaliação da doença de Huntington (UHDRS com o objetivo de estabelecer a reprodutibilidade dessa escala em uma população que difere significativamente daquela em que foi originalmente validada. Após um período de treinamento com um v

  8. Ethnic variations in immigrant poverty exit and female employment: the missing link.

    Science.gov (United States)

    Kaida, Lisa

    2015-04-01

    Despite widespread interest in poverty among recent immigrants and female immigrant employment, research on the link between the two is limited. This study evaluates the effect of recently arrived immigrant women's employment on the exit from family poverty and considers the implications for ethnic differences in poverty exit. It uses the bivariate probit model and the Fairlie decomposition technique to analyze data from the Longitudinal Survey of Immigrants to Canada (LSIC), a nationally representative survey of immigrants arriving in Canada, 2000-2001. Results show that the employment of recently arrived immigrant women makes a notable contribution to lifting families out of poverty. Moreover, the wide ethnic variations in the probability of exit from poverty between European and non-European groups are partially explained by the lower employment rates among non-European women. The results suggest that the equal earner/female breadwinner model applies to low-income recent immigrant families in general, but the male breadwinner model explains the low probability of poverty exit among select non-European groups whose female employment rates are notably low.

  9. Inhibition of the striatal specific phosphodiesterase PDE10A ameliorates striatal and cortical pathology in R6/2 mouse model of Huntington's disease.

    Directory of Open Access Journals (Sweden)

    Carmela Giampà

    2010-10-01

    Full Text Available Huntington's disease is a devastating neurodegenerative condition for which there is no therapy to slow disease progression. The particular vulnerability of striatal medium spiny neurons to Huntington's pathology is hypothesized to result from transcriptional dysregulation within the cAMP and CREB signaling cascades in these neurons. To test this hypothesis, and a potential therapeutic approach, we investigated whether inhibition of the striatal-specific cyclic nucleotide phosphodiesterase PDE10A would alleviate neurological deficits and brain pathology in a highly utilized model system, the R6/2 mouse.R6/2 mice were treated with the highly selective PDE10A inhibitor TP-10 from 4 weeks of age until euthanasia. TP-10 treatment significantly reduced and delayed the development of the hind paw clasping response during tail suspension, deficits in rotarod performance, and decrease in locomotor activity in an open field. Treatment prolonged time to loss of righting reflex. These effects of PDE10A inhibition on neurological function were reflected in a significant amelioration in brain pathology, including reduction in striatal and cortical cell loss, the formation of striatal neuronal intranuclear inclusions, and the degree of microglial activation that occurs in response to the mutant huntingtin-induced brain damage. Striatal and cortical levels of phosphorylated CREB and BDNF were significantly elevated.Our findings provide experimental support for targeting the cAMP and CREB signaling pathways and more broadly transcriptional dysregulation as a therapeutic approach to Huntington's disease. It is noteworthy that PDE10A inhibition in the R6/2 mice reduces striatal pathology, consistent with the localization of the enzyme in medium spiny neurons, and also cortical pathology and the formation of neuronal nuclear inclusions. These latter findings suggest that striatal pathology may be a primary driver of these secondary pathological events. More

  10. Mean-Variance portfolio optimization when each asset has individual uncertain exit-time

    Directory of Open Access Journals (Sweden)

    Reza Keykhaei

    2016-12-01

    Full Text Available The standard Markowitz Mean-Variance optimization model is a single-period portfolio selection approach where the exit-time (or the time-horizon is deterministic. ‎In this paper we study the Mean-Variance portfolio selection problem ‎with ‎uncertain ‎exit-time ‎when ‎each ‎has ‎individual uncertain ‎xit-time‎, ‎which generalizes the Markowitz's model‎. ‎‎‎‎‎‎We provide some conditions under which the optimal portfolio of the generalized problem is independent of the exit-times distributions. Also, ‎‎it is shown that under some general circumstances, the sets of optimal portfolios‎ ‎in the generalized model and the standard model are the same‎.

  11. Neurotensin receptor binding levels in basal ganglia are not altered in Huntington's chorea or schizophrenia

    International Nuclear Information System (INIS)

    Palacios, J.M.; Chinaglia, G.; Rigo, M.; Ulrich, J.; Probst, A.

    1991-01-01

    Autoradiographic techniques were used to examine the distribution and levels of neurotensin receptor binding sites in the basal ganglia and related regions of the human brain. Monoiodo ( 125 I-Tyr3)neurotensin was used as a ligand. High amounts of neurotensin receptor binding sites were found in the substantia nigra pars compacta. Lower but significant quantities of neurotensin receptor binding sites characterized the caudate, putamen, and nucleus accumbens, while very low quantities were seen in both medial and lateral segments of the globus pallidus. In Huntington's chorea, the levels of neurotensin receptor binding sites were found to be comparable to those of control cases. Only slight but not statistically significant decreases in amounts of receptor binding sites were detected in the dorsal part of the head and in the body of caudate nucleus. No alterations in the levels of neurotensin receptor binding sites were observed in the substantia nigra pars compacta and reticulata. These results suggest that a large proportion of neurotensin receptor binding sites in the basal ganglia are located on intrinsic neurons and on extrinsic afferent fibers that do not degenerate in Huntington's disease

  12. Tunneling time, exit time and exit momentum in strong field tunnel ionization

    International Nuclear Information System (INIS)

    Teeny, Nicolas

    2016-01-01

    Tunnel ionization belongs to the fundamental processes of atomic physics. It is still an open question when does the electron tunnel ionize and how long is the duration of tunneling. In this work we solve the time-dependent Schroedinger equation in one and two dimensions and use ab initio quantum calculations in order to answer these questions. Additionally, we determine the exit momentum of the tunnel ionized electron from first principles. We find out results that are different from the assumptions of the commonly employed two-step model, which assumes that the electron ionizes at the instant of electric field maximum with a zero momentum. After determining the quantum final momentum distribution of tunnel ionized electrons we show that the two-step model fails to predict the correct final momentum. Accordingly we suggest how to correct the two-step model. Furthermore, we determine the instant at which tunnel ionization starts, which turns out to be different from the instant usually assumed. From determining the instant at which it is most probable for the electron to enter the tunneling barrier and the instant at which it exits we determine the most probable time spent under the barrier. Moreover, we apply a quantum clock approach in order to determine the duration of tunnel ionization. From the quantum clock we determine an average tunneling time which is different in magnitude and origin with respect to the most probable tunneling time. By defining a probability distribution of tunneling times using virtual detectors we relate both methods and explain the apparent discrepancy. The results found have in general an effect on the interpretation of experiments that measure the spectra of tunnel ionized electrons, and specifically on the calibration of the so called attoclock experiments, because models with imprecise assumptions are usually employed in order to interpret experimental results.

  13. Tunneling time, exit time and exit momentum in strong field tunnel ionization

    Energy Technology Data Exchange (ETDEWEB)

    Teeny, Nicolas

    2016-10-18

    Tunnel ionization belongs to the fundamental processes of atomic physics. It is still an open question when does the electron tunnel ionize and how long is the duration of tunneling. In this work we solve the time-dependent Schroedinger equation in one and two dimensions and use ab initio quantum calculations in order to answer these questions. Additionally, we determine the exit momentum of the tunnel ionized electron from first principles. We find out results that are different from the assumptions of the commonly employed two-step model, which assumes that the electron ionizes at the instant of electric field maximum with a zero momentum. After determining the quantum final momentum distribution of tunnel ionized electrons we show that the two-step model fails to predict the correct final momentum. Accordingly we suggest how to correct the two-step model. Furthermore, we determine the instant at which tunnel ionization starts, which turns out to be different from the instant usually assumed. From determining the instant at which it is most probable for the electron to enter the tunneling barrier and the instant at which it exits we determine the most probable time spent under the barrier. Moreover, we apply a quantum clock approach in order to determine the duration of tunnel ionization. From the quantum clock we determine an average tunneling time which is different in magnitude and origin with respect to the most probable tunneling time. By defining a probability distribution of tunneling times using virtual detectors we relate both methods and explain the apparent discrepancy. The results found have in general an effect on the interpretation of experiments that measure the spectra of tunnel ionized electrons, and specifically on the calibration of the so called attoclock experiments, because models with imprecise assumptions are usually employed in order to interpret experimental results.

  14. Ecologies of ideologies : Explaining party entry and exit in European parliaments, 1945-2013

    NARCIS (Netherlands)

    van de Wardt, Marc; Berkhout, Joost; Vermeulen, Floris

    This study introduces a population-ecological approach to the entry and exit of political parties. A primary proposition of population ecology is that organizational entry and exit depends on the number of organizations already present: that is, density. We propose that political parties mainly

  15. Market entry and exit by biotech and device companies funded by venture capital.

    Science.gov (United States)

    Burns, Lawton R; Housman, Michael G; Robinson, Charles A

    2009-01-01

    Start-up companies in the biotechnology and medical device sectors are important sources of health care innovation. This paper describes the role of venture capital in supporting these companies and charts the growth in venture capital financial support. The paper then uses longitudinal data to describe market entry and exit by these companies. Similar factors are associated with entry and exit in the two sectors. Entries and exits in one sector also appear to influence entry in the other. These findings have important implications for developing innovative technologies and ensuring competitive markets in the life sciences.

  16. EMQN/CMGS best practice guidelines for the molecular genetic testing of Huntington disease

    OpenAIRE

    Losekoot, Monique; van Belzen, Martine J; Seneca, Sara; Bauer, Peter; Stenhouse, Susan A R; Barton, David E

    2012-01-01

    Huntington disease (HD) is caused by the expansion of an unstable polymorphic trinucleotide (CAG)n repeat in exon 1 of the HTT gene, which translates into an extended polyglutamine tract in the protein. Laboratory diagnosis of HD involves estimation of the number of CAG repeats. Molecular genetic testing for HD is offered in a wide range of laboratories both within and outside the European community. In order to measure the quality and raise the standard of molecular genetic testing in these ...

  17. Wives' Relative Wages, Husbands' Paid Work Hours, and Wives' Labor-Force Exit

    Science.gov (United States)

    Shafer, Emily Fitzgibbons

    2011-01-01

    Economic theories predict that women are more likely to exit the labor force if their partners' earnings are higher and if their own wage rate is lower. In this article, I use the National Longitudinal Survey of Youth (N = 2,254) and discrete-time event-history analysis to show that wives' relative wages are more predictive of their exit than are…

  18. First exit times of harmonically trapped particles: a didactic review

    International Nuclear Information System (INIS)

    Grebenkov, Denis S

    2015-01-01

    We revise the classical problem of characterizing first exit times of a harmonically trapped particle whose motion is described by a one- or multidimensional Ornstein–Uhlenbeck process. We start by recalling the main derivation steps of a propagator using Langevin and Fokker–Planck equations. The mean exit time, the moment-generating function and the survival probability are then expressed through confluent hypergeometric functions and thoroughly analyzed. We also present a rapidly converging series representation of confluent hypergeometric functions that is particularly well suited for numerical computation of eigenvalues and eigenfunctions of the governing Fokker–Planck operator. We discuss several applications of first exit times, such as the detection of time intervals during which motor proteins exert a constant force onto a tracer in optical tweezers single-particle tracking experiments; adhesion bond dissociation under mechanical stress; characterization of active periods of trend-following and mean-reverting strategies in algorithmic trading on stock markets; relation to the distribution of first crossing times of a moving boundary by Brownian motion. Some extensions are described, including diffusion under quadratic double-well potential and anomalous diffusion. (topical review)

  19. Mismatch repair genes Mlh1 and Mlh3 modify CAG instability in Huntington's disease mice: genome-wide and candidate approaches.

    Science.gov (United States)

    Pinto, Ricardo Mouro; Dragileva, Ella; Kirby, Andrew; Lloret, Alejandro; Lopez, Edith; St Claire, Jason; Panigrahi, Gagan B; Hou, Caixia; Holloway, Kim; Gillis, Tammy; Guide, Jolene R; Cohen, Paula E; Li, Guo-Min; Pearson, Christopher E; Daly, Mark J; Wheeler, Vanessa C

    2013-10-01

    The Huntington's disease gene (HTT) CAG repeat mutation undergoes somatic expansion that correlates with pathogenesis. Modifiers of somatic expansion may therefore provide routes for therapies targeting the underlying mutation, an approach that is likely applicable to other trinucleotide repeat diseases. Huntington's disease Hdh(Q111) mice exhibit higher levels of somatic HTT CAG expansion on a C57BL/6 genetic background (B6.Hdh(Q111) ) than on a 129 background (129.Hdh(Q111) ). Linkage mapping in (B6x129).Hdh(Q111) F2 intercross animals identified a single quantitative trait locus underlying the strain-specific difference in expansion in the striatum, implicating mismatch repair (MMR) gene Mlh1 as the most likely candidate modifier. Crossing B6.Hdh(Q111) mice onto an Mlh1 null background demonstrated that Mlh1 is essential for somatic CAG expansions and that it is an enhancer of nuclear huntingtin accumulation in striatal neurons. Hdh(Q111) somatic expansion was also abolished in mice deficient in the Mlh3 gene, implicating MutLγ (MLH1-MLH3) complex as a key driver of somatic expansion. Strikingly, Mlh1 and Mlh3 genes encoding MMR effector proteins were as critical to somatic expansion as Msh2 and Msh3 genes encoding DNA mismatch recognition complex MutSβ (MSH2-MSH3). The Mlh1 locus is highly polymorphic between B6 and 129 strains. While we were unable to detect any difference in base-base mismatch or short slipped-repeat repair activity between B6 and 129 MLH1 variants, repair efficiency was MLH1 dose-dependent. MLH1 mRNA and protein levels were significantly decreased in 129 mice compared to B6 mice, consistent with a dose-sensitive MLH1-dependent DNA repair mechanism underlying the somatic expansion difference between these strains. Together, these data identify Mlh1 and Mlh3 as novel critical genetic modifiers of HTT CAG instability, point to Mlh1 genetic variation as the likely source of the instability difference in B6 and 129 strains and suggest that MLH1

  20. Mismatch repair genes Mlh1 and Mlh3 modify CAG instability in Huntington's disease mice: genome-wide and candidate approaches.

    Directory of Open Access Journals (Sweden)

    Ricardo Mouro Pinto

    2013-10-01

    Full Text Available The Huntington's disease gene (HTT CAG repeat mutation undergoes somatic expansion that correlates with pathogenesis. Modifiers of somatic expansion may therefore provide routes for therapies targeting the underlying mutation, an approach that is likely applicable to other trinucleotide repeat diseases. Huntington's disease Hdh(Q111 mice exhibit higher levels of somatic HTT CAG expansion on a C57BL/6 genetic background (B6.Hdh(Q111 than on a 129 background (129.Hdh(Q111 . Linkage mapping in (B6x129.Hdh(Q111 F2 intercross animals identified a single quantitative trait locus underlying the strain-specific difference in expansion in the striatum, implicating mismatch repair (MMR gene Mlh1 as the most likely candidate modifier. Crossing B6.Hdh(Q111 mice onto an Mlh1 null background demonstrated that Mlh1 is essential for somatic CAG expansions and that it is an enhancer of nuclear huntingtin accumulation in striatal neurons. Hdh(Q111 somatic expansion was also abolished in mice deficient in the Mlh3 gene, implicating MutLγ (MLH1-MLH3 complex as a key driver of somatic expansion. Strikingly, Mlh1 and Mlh3 genes encoding MMR effector proteins were as critical to somatic expansion as Msh2 and Msh3 genes encoding DNA mismatch recognition complex MutSβ (MSH2-MSH3. The Mlh1 locus is highly polymorphic between B6 and 129 strains. While we were unable to detect any difference in base-base mismatch or short slipped-repeat repair activity between B6 and 129 MLH1 variants, repair efficiency was MLH1 dose-dependent. MLH1 mRNA and protein levels were significantly decreased in 129 mice compared to B6 mice, consistent with a dose-sensitive MLH1-dependent DNA repair mechanism underlying the somatic expansion difference between these strains. Together, these data identify Mlh1 and Mlh3 as novel critical genetic modifiers of HTT CAG instability, point to Mlh1 genetic variation as the likely source of the instability difference in B6 and 129 strains and suggest

  1. Inhibition of the mitotic exit network in response to damaged telomeres.

    Directory of Open Access Journals (Sweden)

    Mauricio Valerio-Santiago

    Full Text Available When chromosomal DNA is damaged, progression through the cell cycle is halted to provide the cells with time to repair the genetic material before it is distributed between the mother and daughter cells. In Saccharomyces cerevisiae, this cell cycle arrest occurs at the G2/M transition. However, it is also necessary to restrain exit from mitosis by maintaining Bfa1-Bub2, the inhibitor of the Mitotic Exit Network (MEN, in an active state. While the role of Bfa1 and Bub2 in the inhibition of mitotic exit when the spindle is not properly aligned and the spindle position checkpoint is activated has been extensively studied, the mechanism by which these proteins prevent MEN function after DNA damage is still unclear. Here, we propose that the inhibition of the MEN is specifically required when telomeres are damaged but it is not necessary to face all types of chromosomal DNA damage, which is in agreement with previous data in mammals suggesting the existence of a putative telomere-specific DNA damage response that inhibits mitotic exit. Furthermore, we demonstrate that the mechanism of MEN inhibition when telomeres are damaged relies on the Rad53-dependent inhibition of Bfa1 phosphorylation by the Polo-like kinase Cdc5, establishing a new key role of this kinase in regulating cell cycle progression.

  2. Visual Barriers to Prevent Ambulatory ALzheimer's Patients from Exiting through an Emergency Door.

    Science.gov (United States)

    Namazi, Kevan H.; And Others

    1989-01-01

    Conducted study on Alzheimer's unit to test seven different visual barrier conditions for reducing patient exits. Findings indicated that exiting was eliminated under two conditions. Results suggest visual agnosia, the inability to interpret what the eye sees, may be used as tool in managing wandering behavior of Alzheimer's patients. (Author/NB)

  3. Entrance, exit, and reentrance of one shot with a shotgun

    DEFF Research Database (Denmark)

    Gulmann, C; Hougen, H P

    1999-01-01

    The case being reported is one of a homicidal shotgun fatality with an unusual wound pattern. A 34-year-old man was shot at close range with a 12-gauge shotgun armed with No. 5 birdshot ammunition. The shot entered the left axillary region, exited through the left infraclavicular region, and ther......The case being reported is one of a homicidal shotgun fatality with an unusual wound pattern. A 34-year-old man was shot at close range with a 12-gauge shotgun armed with No. 5 birdshot ammunition. The shot entered the left axillary region, exited through the left infraclavicular region...

  4. Formation Mechanisms for Entry and Exit Defects in Bobbin Friction Stir Welding

    Directory of Open Access Journals (Sweden)

    Abbas Tamadon

    2018-01-01

    Full Text Available Bobbin friction stir welding (BFSW is an innovative variant for the solid state welding process whereby a rotating symmetrical tool causes a fully penetrated bond. Despite the process development, there are still unknown variables in the characterization of the process parameters which can cause uncontrolled weld defects. The entry zone and the exit zone consist of two discontinuity-defects and removing them is one of the current challenges for improving the weld quality. In the present research, the characteristic features of the entry and exit defects in the weld structure and formation mechanism of them during the BFSW processing was investigated. Using stacked layers of multi-colour plasticine the material flow, analogous to metal flow, can be visualised. By using different colours as the path markers of the analogue model, the streamline flow can be easily delineated in the discontinuity defects compared with the metal welds. AA6082-T6 aluminium plates and multi-layered plasticine slabs were employed to replicate the entry-exit defects in the metal weld and analogue samples. The fixed-bobbin tool utilized for this research was optimized by adding a thread feature and tri-flat geometry to the pin and closed-end spiral scrolls on both shoulder surfaces. Samples were processed at different rotating and longitudinal speeds to show the degree of dependency on the welding parameters for the defects. The analogue models showed that the entry zone and the exit zone of the BFSW are affected by the inhomogeneity of the material flow regime which causes the ejection or disruption of the plastic flow in the gap between the bobbin shoulders. The trial aluminium welds showed that the elimination of entry-exit defects in the weld body is not completely possible but the size of the defects can be minimized by modification of the welding parameters. For the entry zone, the flow pattern evolution suggested formation mechanisms for a sprayed tail, island zone

  5. The impact of ill health on exit from paid employment in Europe among older workers.

    Science.gov (United States)

    van den Berg, Tilja; Schuring, Merel; Avendano, Mauricio; Mackenbach, Johan; Burdorf, Alex

    2010-12-01

    To determine the impact of ill health on exit from paid employment in Europe among older workers. Participants of the Survey on Health and Ageing in Europe (SHARE) in 11 European countries in 2004 and 2006 were selected when 50-63 years old and in paid employment at baseline (n=4611). Data were collected on self-rated health, chronic diseases, mobility limitations, obesity, smoking, alcohol use, physical activity and work characteristics. Participants were classified into employed, retired, unemployed and disabled at the end of the 2-year follow-up. Multinomial logistic regression was used to estimate the effect of different measures of ill health on exit from paid employment. During the 2-year follow-up, 17% of employed workers left paid employment, mainly because of early retirement. Controlling for individual and work related characteristics, poor self-perceived health was strongly associated with exit from paid employment due to retirement, unemployment or disability (ORs from 1.32 to 4.24). Adjustment for working conditions and lifestyle reduced the significant associations between ill health and exit from paid employment by 0-18.7%. Low education, obesity, low job control and effort-reward imbalance were associated with measures of ill health, but also risk factors for exit from paid employment after adjustment for ill health. Poor self-perceived health was strongly associated with exit from paid employment among European workers aged 50-63 years. This study suggests that the influence of ill health on exit from paid employment could be lessened by measures targeting obesity, problematic alcohol use, job control and effort-reward balance.

  6. Comprehensive behavioral testing in the R6/2 mouse model of Huntington's disease shows no benefit from CoQ10 or minocycline.

    Directory of Open Access Journals (Sweden)

    Liliana B Menalled

    2010-03-01

    Full Text Available Previous studies of the effects of coenzyme Q10 and minocycline on mouse models of Huntington's disease have produced conflicting results regarding their efficacy in behavioral tests. Using our recently published best practices for husbandry and testing for mouse models of Huntington's disease, we report that neither coenzyme Q10 nor minocycline had significant beneficial effects on measures of motor function, general health (open field, rotarod, grip strength, rearing-climbing, body weight and survival in the R6/2 mouse model. The higher doses of minocycline, on the contrary, reduced survival. We were thus unable to confirm the previously reported benefits for these two drugs, and we discuss potential reasons for these discrepancies, such as the effects of husbandry and nutrition.

  7. 49 CFR 238.113 - Emergency window exits.

    Science.gov (United States)

    2010-10-01

    ..., electrical locker, or kitchen); and (B) There are no more than eight seats in the seating area. (4) Cars with...), a bathroom, kitchen, or locomotive cab is not considered a “compartment.” (b) Ease of operability. On or after November 8, 1999, each emergency window exit shall be designed to permit rapid and easy...

  8. Time perception impairment in early-to-moderate stages of Huntington's disease is related to memory deficits.

    Science.gov (United States)

    Righi, Stefania; Galli, Luca; Paganini, Marco; Bertini, Elisabetta; Viggiano, Maria Pia; Piacentini, Silvia

    2016-01-01

    Huntington's disease (HD) primarily affects striatum and prefrontal dopaminergic circuits which are fundamental neural correlates of the timekeeping mechanism. The few studies on HD mainly investigated motor timing performance in second durations. The present work explored time perception in early-to-moderate symptomatic HD patients for seconds and milliseconds with the aim to clarify which component of the scalar expectancy theory (SET) is mainly responsible for HD timing defect. Eleven HD patients were compared to 11 controls employing two separate temporal bisection tasks in second and millisecond ranges. Our results revealed the same time perception deficits for seconds and milliseconds in HD patients. Time perception impairment in early-to-moderate stages of Huntington's disease is related to memory deficits. Furthermore, both the non-systematical defect of temporal sensitivity and the main impairment of timing performance in the extreme value of the psychophysical curves suggested an HD deficit in the memory component of the SET. This result was further confirmed by the significant correlations between time perception performance and long-term memory test scores. Our findings added important preliminary data for both a deeper comprehension of HD time-keeping deficits and possible implications on neuro-rehabilitation practices.

  9. Derivation of Huntington Disease affected Genea046 human embryonic stem cell line

    Directory of Open Access Journals (Sweden)

    Biljana Dumevska

    2016-03-01

    Full Text Available The Genea046 human embryonic stem cell line was derived from a donated, fully commercially consented ART blastocyst, carrying HTT gene CAG expansion of 45 repeats, indicative of Huntington Disease. Following ICM outgrowth on inactivated human feeders, karyotype was confirmed as 46, XX by CGH and STR analysis demonstrated a female Allele pattern. The hESC line had pluripotent cell morphology, 85% of cells expressed Nanog, 92% Oct4, 75% Tra1–60 and 99% SSEA4 and demonstrated Alkaline Phosphatase activity. The cell line was negative for Mycoplasma and visible contamination.

  10. Nonparametric modeling and analysis of association between Huntington's disease onset and CAG repeats.

    Science.gov (United States)

    Ma, Yanyuan; Wang, Yuanjia

    2014-04-15

    Huntington's disease (HD) is a neurodegenerative disorder with a dominant genetic mode of inheritance caused by an expansion of CAG repeats on chromosome 4. Typically, a longer sequence of CAG repeat length is associated with increased risk of experiencing earlier onset of HD. Previous studies of the association between HD onset age and CAG length have favored a logistic model, where the CAG repeat length enters the mean and variance components of the logistic model in a complex exponential-linear form. To relax the parametric assumption of the exponential-linear association to the true HD onset distribution, we propose to leave both mean and variance functions of the CAG repeat length unspecified and perform semiparametric estimation in this context through a local kernel and backfitting procedure. Motivated by including family history of HD information available in the family members of participants in the Cooperative Huntington's Observational Research Trial (COHORT), we develop the methodology in the context of mixture data, where some subjects have a positive probability of being risk free. We also allow censoring on the age at onset of disease and accommodate covariates other than the CAG length. We study the theoretical properties of the proposed estimator and derive its asymptotic distribution. Finally, we apply the proposed methods to the COHORT data to estimate the HD onset distribution using a group of study participants and the disease family history information available on their family members. Copyright © 2013 John Wiley & Sons, Ltd.

  11. NAD+ Is a Food Component That Promotes Exit from Dauer Diapause in Caenorhabditis elegans.

    Directory of Open Access Journals (Sweden)

    Mykola Mylenko

    Full Text Available The free-living soil nematode Caenorhabditis elegans adapts its development to the availability of food. When food is scarce and population density is high, worms enter a developmentally arrested non-feeding diapause stage specialized for long-term survival called the dauer larva. When food becomes available, they exit from the dauer stage, resume growth and reproduction. It has been postulated that compound(s present in food, referred to as the "food signal", promote exit from the dauer stage. In this study, we have identified NAD+ as a component of bacterial extract that promotes dauer exit. NAD+, when dissolved in alkaline medium, causes opening of the mouth and ingestion of food. We also show that to initiate exit from the dauer stage in response to NAD+ worms require production of serotonin. Thus, C. elegans can use redox cofactors produced by dietary organisms to sense food.

  12. Exit, voice, and loyalty in the Italian public health service: macroeconomic and corporate implications.

    Science.gov (United States)

    Ippolito, Adelaide; Impagliazzo, Cira; Zoccoli, Paola

    2013-01-01

    The paper analyses how customers of public health organizations can express their dissatisfaction for the services offered to them. The main aim is to evaluate the effects that possible dissatisfaction of Italian public health service customers can have on public health organizations. We adopted the methodological scheme developed by Hirschman with exit, voice, and loyalty, considering the macroeconomic and corporate implications that it causes for Italian public health organizations. The study investigated the effects developed by exit of the patients on the system of financing of local health authorities considering both the corporate level of analysis and the macroeconomic level. As a result, local health authority management is encouraged to pay greater attention to the exit phenomena through the adoption of tools that promote loyalty, such as the promotion of voice, even if exit is not promoting, at a macroeconomic level, considerable attention to this phenomenon.

  13. Exit, Voice, and Loyalty in the Italian Public Health Service: Macroeconomic and Corporate Implications

    Science.gov (United States)

    Impagliazzo, Cira; Zoccoli, Paola

    2013-01-01

    The paper analyses how customers of public health organizations can express their dissatisfaction for the services offered to them. The main aim is to evaluate the effects that possible dissatisfaction of Italian public health service customers can have on public health organizations. We adopted the methodological scheme developed by Hirschman with exit, voice, and loyalty, considering the macroeconomic and corporate implications that it causes for Italian public health organizations. The study investigated the effects developed by exit of the patients on the system of financing of local health authorities considering both the corporate level of analysis and the macroeconomic level. As a result, local health authority management is encouraged to pay greater attention to the exit phenomena through the adoption of tools that promote loyalty, such as the promotion of voice, even if exit is not promoting, at a macroeconomic level, considerable attention to this phenomenon. PMID:24348148

  14. The story of George Huntington and his disease

    Directory of Open Access Journals (Sweden)

    Kalyan B Bhattacharyya

    2016-01-01

    Full Text Available George Huntington described some families with choreiform movements in 1872 in the United States of America and since then many such families have been described in other parts of the world and works on the genetics of the disease have brought new vistas in the understanding of the disease. In 1958, Americo Negrette, a young Venezuelan physician observed similar subjects in the vicinity of Lake Maracaibo which was presented by his co-worker, Ramon Avilla Giron at New York in 1972 when United States of America had been commemorating the centenary year of Huntington′s disease. Nancy Wexler, a psychoanalyst, whose mother had been suffering from the disease attended the meeting and organized a research team to Venezuela and they systematically studied more than 18,000 individuals in order to work out a common pedigree. They identified the genetic locus of the disease in the short arm of chromosome 4 and observed that it was a trinucleotide repeat disorder.

  15. Making a measurable difference in advanced Huntington disease care.

    Science.gov (United States)

    Moskowitz, Carol Brown; Rao, Ashwini K

    2017-01-01

    Neurologists' role in the care of people with advanced Huntington disease (HD) (total functional capacity speech and language pathology), behavioral and psychiatric professionals for problem-solving strategies, which must be reviewed with direct care staff before implementation; (3) encourage and support qualitative and quantitative interdisciplinary research studies, and randomized controlled studies of nonpharmacologic interventions; and (4) assist in the development of meaningful measures to further document what works to provide a good quality of life for the patient and family and a comfortable thoughtful approach to a good death. Collaborative models of care depend on: (1) clear communication; (2) ongoing education and support programs; with (3) pharmacologic and rehabilitation interventions, always in the context of respect for the person with HD, a preservation of the individuals' dignity, autonomy, and individual preferences. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. Brain atrophy in Huntington's disease: A CT-scan study

    International Nuclear Information System (INIS)

    Starkstein, S.E.; Folstein, S.E.; Brandt, J.; McDonnell, A.; Folstein, M.

    1989-01-01

    CT-scan measurements of cortical and subcortical atrophy were carried out in 34 patients with Huntington's disease (HD). While a significant correlation was observed between parameters of subcortical atrophy (bicaudate ratio, bifrontal ratio and third ventricular ratio) and duration of the disease, there was no significant correlation between these parameters and age. On the other hand, measurements of cortical atrophy (frontal fissure ratio and cortical sulci ratio) correlated significantly with age but not with duration of the disease. When a group of 24 HD patients were compared on CT-scan measurements with a group of 24 age-matched normal controls, significant differences were obtained for all the variables examined, but the bicaudate ratio showed the highest sensitivity and specificity. Even mildly affected patients, with duration of motor symptoms less than 3 years had higher bicaudate ratios than age-matched controls. (orig.)

  17. Ecologies of Ideologies : Explaining Party Entry and Exit in West-European Parliaments, 1945-2013

    NARCIS (Netherlands)

    van de Wardt, M.; Berkhout, J.; Vermeulen, F.

    2017-01-01

    This study introduces a population-ecological approach to the entry and exit of political parties. A primary proposition of population ecology is that organizational entry and exit depends on the number of organizations already present: that is, density. We propose that political parties mainly

  18. Glucose transportation in the brain and its impairment in Huntington disease: one more shade of the energetic metabolism failure?

    Science.gov (United States)

    Morea, Veronica; Bidollari, Eris; Colotti, Gianni; Fiorillo, Annarita; Rosati, Jessica; De Filippis, Lidia; Squitieri, Ferdinando; Ilari, Andrea

    2017-07-01

    Huntington's disease (HD) or Huntington's chorea is the most common inherited, dominantly transmitted, neurodegenerative disorder. It is caused by increased CAG repeats number in the gene coding for huntingtin (Htt) and characterized by motor, behaviour and psychiatric symptoms, ultimately leading to death. HD patients also exhibit alterations in glucose and energetic metabolism, which result in pronounced weight loss despite sustained calorie intake. Glucose metabolism decreases in the striatum of all the subjects with mutated Htt, but affects symptom presentation only when it drops below a specific threshold. Recent evidence points at defects in glucose uptake by the brain, and especially by neurons, as a relevant component of central glucose hypometabolism in HD patients. Here we review the main features of glucose metabolism and transport in the brain in physiological conditions and how these processes are impaired in HD, and discuss the potential ability of strategies aimed at increasing intracellular energy levels to counteract neurological and motor degeneration in HD patients.

  19. Sampling for Patient Exit Interviews: Assessment of Methods Using Mathematical Derivation and Computer Simulations.

    Science.gov (United States)

    Geldsetzer, Pascal; Fink, Günther; Vaikath, Maria; Bärnighausen, Till

    2018-02-01

    (1) To evaluate the operational efficiency of various sampling methods for patient exit interviews; (2) to discuss under what circumstances each method yields an unbiased sample; and (3) to propose a new, operationally efficient, and unbiased sampling method. Literature review, mathematical derivation, and Monte Carlo simulations. Our simulations show that in patient exit interviews it is most operationally efficient if the interviewer, after completing an interview, selects the next patient exiting the clinical consultation. We demonstrate mathematically that this method yields a biased sample: patients who spend a longer time with the clinician are overrepresented. This bias can be removed by selecting the next patient who enters, rather than exits, the consultation room. We show that this sampling method is operationally more efficient than alternative methods (systematic and simple random sampling) in most primary health care settings. Under the assumption that the order in which patients enter the consultation room is unrelated to the length of time spent with the clinician and the interviewer, selecting the next patient entering the consultation room tends to be the operationally most efficient unbiased sampling method for patient exit interviews. © 2016 The Authors. Health Services Research published by Wiley Periodicals, Inc. on behalf of Health Research and Educational Trust.

  20. Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease

    OpenAIRE

    Keene, C. Dirk; Rodrigues, Cecilia M. P.; Eich, Tacjana; Chhabra, Manik S.; Steer, Clifford J.; Low, Walter C.

    2002-01-01

    Huntington's disease (HD) is an untreatable neurological disorder caused by selective and progressive degeneration of the caudate nucleus and putamen of the basal ganglia. Although the etiology of HD pathology is not fully understood, the observed loss of neuronal cells is thought to occur primarily through apoptosis. Furthermore, there is evidence in HD that cell death is mediated through mitochondrial pathways, and mitochondrial deficits are commonly associated with HD. We have previously r...

  1. Human glia can both induce and rescue aspects of disease phenotype in Huntington disease

    DEFF Research Database (Denmark)

    Benraiss, Abdellatif; Wang, Su; Herrlinger, Stephanie

    2016-01-01

    The causal contribution of glial pathology to Huntington disease (HD) has not been heavily explored. To define the contribution of glia to HD, we established human HD glial chimeras by neonatally engrafting immunodeficient mice with mutant huntingtin (mHTT)-expressing human glial progenitor cells...... chimeras are hyperexcitable. Conversely, normal glia can ameliorate disease phenotype in transgenic HD mice, as striatal transplantation of normal glia rescues aspects of electrophysiological and behavioural phenotype, restores interstitial potassium homeostasis, slows disease progression and extends...

  2. The exit-time problem for a Markov jump process

    Science.gov (United States)

    Burch, N.; D'Elia, M.; Lehoucq, R. B.

    2014-12-01

    The purpose of this paper is to consider the exit-time problem for a finite-range Markov jump process, i.e, the distance the particle can jump is bounded independent of its location. Such jump diffusions are expedient models for anomalous transport exhibiting super-diffusion or nonstandard normal diffusion. We refer to the associated deterministic equation as a volume-constrained nonlocal diffusion equation. The volume constraint is the nonlocal analogue of a boundary condition necessary to demonstrate that the nonlocal diffusion equation is well-posed and is consistent with the jump process. A critical aspect of the analysis is a variational formulation and a recently developed nonlocal vector calculus. This calculus allows us to pose nonlocal backward and forward Kolmogorov equations, the former equation granting the various moments of the exit-time distribution.

  3. Suicidal ideation and suicidal behavior according to the C-SSRS in a European cohort of Huntington's disease gene expansion carriers.

    Science.gov (United States)

    van Duijn, Erik; Vrijmoeth, Eslie M; Giltay, Erik J; Bernhard Landwehrmeyer, G

    2018-03-01

    Huntington's disease (HD) gene expansion carriers are at an increased risk of suicide, but so far, no studies have investigated the full spectrum of suicidality, including suicidal ideation, suicidal behavior and self-injurious behavior. We included 1451 HD gene expansion carriers (age 48.4 years (SD 14.0), 54.8% female) of the REGISTRY study of the European Huntington's Disease Network. Lifetime suicidal ideation and suicidal behavior were assessed with the Columbia-Suicidal Severity Rating Scale. Motor symptoms and disease stage were assessed using subscales of the Unified Huntington's Disease Rating Scale, and depressed mood and irritability were assessed by the Problem Behaviors Assessment. Lifetime passive suicidal ideation was reported by 21.2%. Participants in stage II showed the highest prevalence rate of suicidal ideation, while participants in stage IV/V showed the highest prevalence of suicidal behavior. A lifetime suicide attempt was reported by 6.5% of the HD gene expansion carriers. In multivariate regression analyses, both suicidal ideation and suicidal behavior were associated with a depressed mood, and to a lesser extend to irritability. Results may have been affected by denial or recall bias and no conclusions can be made about the temporal and causal relationships with depressed mood and irritability because of the cross-sectional analyses. Given the high prevalence of suicidal ideation and suicidal behavior in all stages of HD, it is important to screen HD gene expansion carriers for suicidal ideation and suicidal behavior on a regular basis in clinical practice. Copyright © 2017 Elsevier B.V. All rights reserved.

  4. Neuropathological Comparison of Adult Onset and Juvenile Huntington's Disease with Cerebellar Atrophy: A Report of a Father and Son.

    Science.gov (United States)

    Latimer, Caitlin S; Flanagan, Margaret E; Cimino, Patrick J; Jayadev, Suman; Davis, Marie; Hoffer, Zachary S; Montine, Thomas J; Gonzalez-Cuyar, Luis F; Bird, Thomas D; Keene, C Dirk

    2017-01-01

    Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by a trinucleotide (CAG) repeat expansion in huntingtin (HTT) on chromosome 4. Anticipation can cause longer repeat expansions in children of HD patients. Juvenile Huntington's disease (JHD), defined as HD arising before age 20, accounts for 5-10% of HD cases, with cases arising in the first decade accounting for approximately 1%. Clinically, JHD differs from the predominately choreiform adult onset Huntington's disease (AOHD) with variable presentations, including symptoms such as myoclonus, seizures, Parkinsonism, and cognitive decline. The neuropathologic changes of AOHD are well characterized, but there are fewer reports that describe the neuropathology of JHD. Here we report a case of a six-year-old boy with paternally-inherited JHD caused by 169 CAG trinucleotide repeats who presented at age four with developmental delay, dysarthria, and seizures before dying at age 6. The boy's clinical presentation and neuropathological findings are directly compared to those of his father, who presented with AOHD and 54 repeats. A full autopsy was performed for the JHD case and a brain-only autopsy was performed for the AOHD case. Histochemically- and immunohistochemically-stained slides were prepared from formalin-fixed, paraffin-embedded tissue sections. Both cases had neuropathology corresponding to Vonsattel grade 3. The boy also had cerebellar atrophy with huntingtin-positive inclusions in the cerebellum, findings not present in the father. Autopsies of father and son provide a unique opportunity to compare and contrast the neuropathologic findings of juvenile and adult onset HD while also providing the first immunohistochemical evidence of cerebellar involvement in JHD. Additionally this is the first known report to include findings from peripheral tissue in a case of JHD.

  5. Orphan drugs in development for Huntington's disease: challenges and progress

    Directory of Open Access Journals (Sweden)

    Burgunder JM

    2015-02-01

    advanced strategies to develop novel treatments in Huntington's disease are examined. Keywords: Huntington's disease, symptomatic treatment, disease-modifying therapy

  6. Probability evolution method for exit location distribution

    Science.gov (United States)

    Zhu, Jinjie; Chen, Zhen; Liu, Xianbin

    2018-03-01

    The exit problem in the framework of the large deviation theory has been a hot topic in the past few decades. The most probable escape path in the weak-noise limit has been clarified by the Freidlin-Wentzell action functional. However, noise in real physical systems cannot be arbitrarily small while noise with finite strength may induce nontrivial phenomena, such as noise-induced shift and noise-induced saddle-point avoidance. Traditional Monte Carlo simulation of noise-induced escape will take exponentially large time as noise approaches zero. The majority of the time is wasted on the uninteresting wandering around the attractors. In this paper, a new method is proposed to decrease the escape simulation time by an exponentially large factor by introducing a series of interfaces and by applying the reinjection on them. This method can be used to calculate the exit location distribution. It is verified by examining two classical examples and is compared with theoretical predictions. The results show that the method performs well for weak noise while may induce certain deviations for large noise. Finally, some possible ways to improve our method are discussed.

  7. Histopathological detection of entry and exit holes in human skin wounds caused by firearms.

    Science.gov (United States)

    Baptista, Marcus Vinícius; d'Ávila, Solange C G P; d'Ávila, Antônio Miguel M P

    2014-07-01

    The judiciary needs forensic medicine to determine the difference between an entry hole and an exit hole in human skin caused by firearms for civilian use. This important information would be most useful if a practical and accurate method could be done with low-cost and minimal technological resources. Both macroscopic and microscopic analyses were performed on skin lesions caused by firearm projectiles, to establish histological features of 14 entry holes and 14 exit holes. Microscopically, in the abrasion area macroscopically observed, there were signs of burns (sub-epidermal cracks and keratinocyte necrosis) in the entrance holes in all cases. These signs were not found in three exit holes which showed an abrasion collar, nor in other exit holes. Some other microscopic features not found in every case were limited either to entry holes, such as cotton fibres, grease deposits, or tattooing in the dermis, or to exit holes, such as adipose tissue, bone or muscle tissue in the dermis. Coagulative necrosis of keratinocytes and sub-epidermal cracks are characteristic of entry holes. Despite the small sample size, it can be safely inferred that this is an important microscopic finding, among others less consistently found, to define an entry hole in questionable cases. Copyright © 2014 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

  8. Exit and Failure of Credit Unions in Brazil: A Risk Analysis

    Directory of Open Access Journals (Sweden)

    Flávio Leonel de Carvalho

    2015-04-01

    Full Text Available This study aims to investigate the factors that affect the market exit of Brazilian singular credit unions from 1995 to 2009; it also identifies and lists the determinants of various types of market exits and analyzes whether profitability is a significant factor for credit union survival. This study was conducted with accounting data provided by the Central Bank of Brazil, which derives only from individual cooperatives, i.e. singular credit unions. Quarterly financial statements from these credit unions that were active from 1995 to the second quarter of 2009 were employed, totaling 71,325 observations for 1,929 credit unions. Based on survival and the model of competing risks (such as the Cox, Exponential, Weibull, Gompertz, and Competing Risk models, the results show that there is no statistical evidence to ensure a correlation between profitability and credit union survival. The results also suggest that the size of credit unions plays a key role in their survival and longevity and that their funding and investment management are related to their survival and risk of market exit. In conclusion, the results confirm the initial idea that the duality inherent to credit unions - cooperative principles versus economic efficiency - might influence the stability, survival, and longevity of these institutions. Such results may also imply that a credit union embracing the rationale of a private bank will become more estranged from its members, something which will hinder its future operations and increase the likelihood of its exit from the market.

  9. A Case of Juvenile Huntington Disease in a 6-Year-Old Boy

    Directory of Open Access Journals (Sweden)

    Jun-Sang Sunwoo

    2010-10-01

    Full Text Available Huntington disease is a neurodegenerative disorder distinguished by the triad of dominant inheritance, choreoathetosis and dementia, usually with onset in the fourth and fifth decades. It is caused by an unstable cytosine-adenine-guanine (CAG trinucleotide repeat expansion in the gene IT15 in locus 4p16.3. Juvenile HD that constitutes about 3% to 10% of all patients is clinically different from adult-onset form and characterized by a larger number of CAG repeats typically exceeding 60. We report a case of a 6-year-old boy with myoclonic seizure and 140 CAG repeats confirmed by molecular genetic analysis.

  10. A SNP in the HTT promoter alters NF-κB binding and is a bidirectional genetic modifier of Huntington disease

    DEFF Research Database (Denmark)

    Bečanović, Kristina; Nørremølle, Anne; Neal, Scott J

    2015-01-01

    Cis-regulatory variants that alter gene expression can modify disease expressivity, but none have previously been identified in Huntington disease (HD). Here we provide in vivo evidence in HD patients that cis-regulatory variants in the HTT promoter are bidirectional modifiers of HD age of onset....

  11. Assessment of motor function, sensory motor gating and recognition memory in a novel BACHD transgenic rat model for Huntington disease

    NARCIS (Netherlands)

    Abada, Yah-se K.; Nguyen, Huu Phuc; Schreiber, Rudy; Ellenbroek, Bart

    2013-01-01

    Rationale: Huntington disease (HD) is frequently first diagnosed by the appearance of motor symptoms; the diagnosis is subsequently confirmed by the presence of expanded CAG repeats (> 35) in the HUNTINGTIN (HTT) gene. A BACHD rat model for HD carrying the human full length mutated HTT with 97

  12. Fast Water Transport in CNTs: length dependence and entrane/exit effects

    DEFF Research Database (Denmark)

    Walther, Jens Honore; Koumoutsakos, Petros

    Superfast water transport in carbon nanotube (CNT) membranes has been reported in experimental studies. We use Molecular Dynamics simulations to elucidate the mechanisms of water entry, exit and transport in 2nm-diameter hydrophobic CNTs embedded in a hydrophilic membrane matrix. We demonstrate......, for the first time, that under imposed pressures of the order of 1 bar, water entry into the CNT cavity and exit from the CNT end, can occur only on pre-wetted membranes. We conduct large scale simulations for up to 500nm long CNTs and observe a previously unseen dependence of the flow enhancement rates...

  13. Forthcoming EU Rules on Exit Taxes for Companies

    NARCIS (Netherlands)

    van de Streek, J.

    2015-01-01

    Rules on exit taxation for the EU as a whole are under construction at European Council level. Formally, the work on this piece of EU tax legislation is part of the controversial Common Consolidated Corporate Tax Base (CCCTB). But it is also thinkable that even without a CCCTB being established in

  14. Customer Protest: Exit, Voice or Negative Word of Mouth

    Directory of Open Access Journals (Sweden)

    Solvang, B. K.

    2008-01-01

    Full Text Available Of the three forms of protest the propensity of word of mouth (WOM seems to be the most common, and the most exclusive form of protest seems to be exit. The propensity for voice lies in between. The costs linked to voice influence the propensity for WOM. The customers seem to do an evaluation between the three forms of protest, yet the rational picture of the customers should be moderated.Leaders should improve their treatment of the customers making complaints. The more they can treat customer complaints in an orderly and nice way the less informal negative word of mouth activity they will experience and they will reduce the exit propensity and lead the customers to the complain organisation. They should also ensure that their customers feel they get equal treatment by the staff.

  15. The molecular chaperone Hsp90 is required for cell cycle exit in Drosophila melanogaster.

    Directory of Open Access Journals (Sweden)

    Jennifer L Bandura

    Full Text Available The coordination of cell proliferation and differentiation is crucial for proper development. In particular, robust mechanisms exist to ensure that cells permanently exit the cell cycle upon terminal differentiation, and these include restraining the activities of both the E2F/DP transcription factor and Cyclin/Cdk kinases. However, the full complement of mechanisms necessary to restrain E2F/DP and Cyclin/Cdk activities in differentiating cells are not known. Here, we have performed a genetic screen in Drosophila melanogaster, designed to identify genes required for cell cycle exit. This screen utilized a PCNA-miniwhite(+ reporter that is highly E2F-responsive and results in a darker red eye color when crossed into genetic backgrounds that delay cell cycle exit. Mutation of Hsp83, the Drosophila homolog of mammalian Hsp90, results in increased E2F-dependent transcription and ectopic cell proliferation in pupal tissues at a time when neighboring wild-type cells are postmitotic. Further, these Hsp83 mutant cells have increased Cyclin/Cdk activity and accumulate proteins normally targeted for proteolysis by the anaphase-promoting complex/cyclosome (APC/C, suggesting that APC/C function is inhibited. Indeed, reducing the gene dosage of an inhibitor of Cdh1/Fzr, an activating subunit of the APC/C that is required for timely cell cycle exit, can genetically suppress the Hsp83 cell cycle exit phenotype. Based on these data, we propose that Cdh1/Fzr is a client protein of Hsp83. Our results reveal that Hsp83 plays a heretofore unappreciated role in promoting APC/C function during cell cycle exit and suggest a mechanism by which Hsp90 inhibition could promote genomic instability and carcinogenesis.

  16. The molecular chaperone Hsp90 is required for cell cycle exit in Drosophila melanogaster.

    Science.gov (United States)

    Bandura, Jennifer L; Jiang, Huaqi; Nickerson, Derek W; Edgar, Bruce A

    2013-01-01

    The coordination of cell proliferation and differentiation is crucial for proper development. In particular, robust mechanisms exist to ensure that cells permanently exit the cell cycle upon terminal differentiation, and these include restraining the activities of both the E2F/DP transcription factor and Cyclin/Cdk kinases. However, the full complement of mechanisms necessary to restrain E2F/DP and Cyclin/Cdk activities in differentiating cells are not known. Here, we have performed a genetic screen in Drosophila melanogaster, designed to identify genes required for cell cycle exit. This screen utilized a PCNA-miniwhite(+) reporter that is highly E2F-responsive and results in a darker red eye color when crossed into genetic backgrounds that delay cell cycle exit. Mutation of Hsp83, the Drosophila homolog of mammalian Hsp90, results in increased E2F-dependent transcription and ectopic cell proliferation in pupal tissues at a time when neighboring wild-type cells are postmitotic. Further, these Hsp83 mutant cells have increased Cyclin/Cdk activity and accumulate proteins normally targeted for proteolysis by the anaphase-promoting complex/cyclosome (APC/C), suggesting that APC/C function is inhibited. Indeed, reducing the gene dosage of an inhibitor of Cdh1/Fzr, an activating subunit of the APC/C that is required for timely cell cycle exit, can genetically suppress the Hsp83 cell cycle exit phenotype. Based on these data, we propose that Cdh1/Fzr is a client protein of Hsp83. Our results reveal that Hsp83 plays a heretofore unappreciated role in promoting APC/C function during cell cycle exit and suggest a mechanism by which Hsp90 inhibition could promote genomic instability and carcinogenesis.

  17. 45 CFR 670.34 - Entry and exit ports.

    Science.gov (United States)

    2010-10-01

    ... ANTARCTIC ANIMALS AND PLANTS Import Into and Export From the United States § 670.34 Entry and exit ports. (a) Any native mammal, native bird, or native plants taken within Antarctica that are imported into or... Orleans, Louisiana. (7) New York, New York. (8) Seattle, Washington. (9) Dallas/Fort Worth, Texas. (10...

  18. Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin

    DEFF Research Database (Denmark)

    Carroll, Jeffrey B; Warby, Simon C; Southwell, Amber L

    2011-01-01

    Huntington disease (HD) is an autosomal dominant neurodegenerative disorder caused by CAG-expansion in the huntingtin gene (HTT) that results in a toxic gain of function in the mutant huntingtin protein (mHTT). Reducing the expression of mHTT is therefore an attractive therapy for HD. However, wild...

  19. Sertraline and venlafaxine improves motor performance and neurobehavioral deficit in quinolinic acid induced Huntington's like symptoms in rats: Possible neurotransmitters modulation.

    Science.gov (United States)

    Gill, Jaskamal Singh; Jamwal, Sumit; Kumar, Puneet; Deshmukh, Rahul

    2017-04-01

    Huntington Disease is autosomal, fatal and progressive neurodegenerative disorder for which clinically available drugs offer only symptomatic relief. Emerging strides have indicated that antidepressants improve motor performance, restore neurotransmitters level, ameliorates striatal atrophy, increases BDNF level and may enhance neurogenesis. Therefore, we investigated sertraline and venlafaxine, clinically available drugs for depression with numerous neuroprotective properties, for their beneficial effects, if any, in quinolinic acid induced Huntington's like symptoms in rats. Rats were administered quinolinic acid (QA) (200 nmol/2μl saline) intrastriatal bilaterally on 0day. Sertraline and venlafaxine (10 and 20mg/kg, po) each were administered for 21days once a day. Motor performance was assessed using rotarod test, grip strength test, narrow beam walk test on weekly basis. On day 22, animals were sacrificed and rat striatum was isolated for biochemical (LPO, GSH and Nitrite), neuroinflammation (TNF-α, IL-1β and IL-6) and neurochemical analysis (GABA, glutamate, norepinephrine, dopamine, serotonin, DOPAC, HVA and 5-HIAA). QA treatment significantly altered body weight, motor performance, oxidative defense (increased LPO, nitrite and decreased GSH), pro-inflammatory cytokines levels (TNF-α, IL-6 and IL-1β), neurochemical level (GABA, glutamate, nor-epinephrine, dopamine, serotonin, HVA, DOPAC, 5-HIAA). Sertraline and venlafaxine at selected doses significantly attenuated QA induced alterations in striatum. The present study suggests that modulation of monoamines level, normalization of GABA and glutamatergic signaling, anti-oxidant and anti-inflammatory properties could underlie the neuroprotective effect of sertraline and venlafaxine in QA induced Huntington's like symptoms. Copyright © 2016. Published by Elsevier Urban & Partner Sp. z o.o.

  20. Therapeutic strategies for circadian rhythm and sleep disturbances in Huntington disease.

    Science.gov (United States)

    van Wamelen, Daniel J; Roos, Raymund Ac; Aziz, Nasir A

    2015-12-01

    Aside from the well-known motor, cognitive and psychiatric signs and symptoms, Huntington disease (HD) is also frequently complicated by circadian rhythm and sleep disturbances. Despite the observation that these disturbances often precede motor onset and have a high prevalence, no studies are available in HD patients which assess potential treatments. In this review, we will briefly outline the nature of circadian rhythm and sleep disturbances in HD and subsequently focus on potential treatments based on findings in other neurodegenerative diseases with similarities to HD, such as Parkinson and Alzheimer disease. The most promising treatment options to date for circadian rhythm and sleep disruption in HD include melatonin (agonists) and bright light therapy, although further corroboration in clinical trials is warranted.

  1. Controlled clinical trial of cannabidiol in Huntington's disease.

    Science.gov (United States)

    Consroe, P; Laguna, J; Allender, J; Snider, S; Stern, L; Sandyk, R; Kennedy, K; Schram, K

    1991-11-01

    Based on encouraging preliminary findings, cannabidiol (CBD), a major nonpsychotropic constituent of Cannabis, was evaluated for symptomatic efficacy and safety in 15 neuroleptic-free patients with Huntington's Disease (HD). The effects of oral CBD (10 mg/kg/day for 6 weeks) and placebo (sesame oil for 6 weeks) were ascertained weekly under a double-blind, randomized cross-over design. A comparison of the effects of CBD and placebo on chorea severity and other therapeutic outcome variables, and on a Cannabis side effect inventory, clinical lab tests and other safety outcome variables, indicated no significant (p greater than 0.05) or clinically important differences. Correspondingly, plasma levels of CBD were assayed by GC/MS, and the weekly levels (mean range of 5.9 to 11.2 ng/ml) did not differ significantly over the 6 weeks of CBD administration. In summary, CBD, at an average daily dose of about 700 mg/day for 6 weeks, was neither symptomatically effective nor toxic, relative to placebo, in neuroleptic-free patients with HD.

  2. A simplified approach for exit dose in vivo measurements in radiotherapy and its clinical application

    International Nuclear Information System (INIS)

    Banjade, D.P.; Shukri, A.; Tajuddin, A.A.; Shrestha, S.L.; Bhat, M.

    2002-01-01

    This is a study using LiF:Mg;Ti thermoluminescent dosimeter (TLD) rods in phantoms to investigate the effect of lack of backscatter on exit dose. Comparing the measured dose with anticipated dose calculated using tissue maximum ratio (TMR) or percentage depth dose (PDD) gives rise to a correction factor. This correction factor may be applied to in-vivo dosimetry results to derive true dose to a point within the patient. Measurements in a specially designed humanoid breast phantom as well as patients undergoing radiotherapy treatment were also been done. TLDs with reproducibility of within ±3% (1 SD) are irradiated in a series of measurements for 6 and 10 MV photon beams from a medical linear accelerator. The measured exit doses for the different phantom thickness for 6 MV beams are found to be lowered by 10.9 to 14.0% compared to the dose derived from theoretical estimation (normalized dose at d max ). The same measurements for 10 MV beams are lowered by 9.0 to 13.5%. The variations of measured exit dose for different field sizes are found to be within 2.5%. The exit doses with added backscatter material from 2 mm up to 15 cm, shows gradual increase and the saturated values agreed within 1.5% with the expected results for both beams. The measured exit doses in humanoid breast phantom as well as in the clinical trial on patients undergoing radiotherapy also agreed with the predicted results based on phantom measurements. The authors' viewpoint is that this technique provides sufficient information to design exit surface bolus to restore build down effect in cases where part of the exit surface is being considered as a target volume. It indicates that the technique could be translated for in vivo dose measurements, which may be a conspicuous step of quality assurance in clinical practice. Copyright (2002) Australasian College of Physical Scientists and Engineers in Medicine

  3. Association of rheumatic diseases with early exit from paid employment in Portugal.

    Science.gov (United States)

    Laires, Pedro A; Gouveia, Miguel

    2014-04-01

    To examine the association between rheumatic diseases (RD) and other chronic morbidity with early exit from paid employment in the Portuguese population. The study population consisted of all people between 50 and 64 years of age (3,762 men and 4,241 women) who participated in the Portuguese National Health Survey, conducted in 2005/2006. Data were collected on demographics, ill-health, lifestyle, and socioeconomic factors. Logistic regression was used to estimate the isolated effect of rheumatic diseases and other chronic diseases on the likelihood of exit from paid employment. At the time of the survey, 45.1 % of the Portuguese population with ages between 50 and 64 years old were not employed. In the nonemployed population, 31.6 % self-reported "poor" to "very poor" health, whereas 16.4 % did so in the employed population. A larger average number of major chronic diseases per capita were also found in those not employed (1.9 vs. 1.4, p paid employment. In particular, rheumatic diseases were more prevalent (43.4 vs. 32.1 %) and associated with early exit from work (OR 1.31; CI 1.12-1.52, p = 0.001). This study suggests an association between RD and other major chronic diseases with early exit from paid employment in Portugal. Thus, health and social protection policies should target these chronic disorders in order to better address sustainability issues and social protection effectiveness.

  4. Airport exit and entry screening for Ebola--August-November 10, 2014.

    Science.gov (United States)

    Brown, Clive M; Aranas, Aaron E; Benenson, Gabrielle A; Brunette, Gary; Cetron, Marty; Chen, Tai-Ho; Cohen, Nicole J; Diaz, Pam; Haber, Yonat; Hale, Christa R; Holton, Kelly; Kohl, Katrin; Le, Amanda W; Palumbo, Gabriel J; Pearson, Kate; Phares, Christina R; Alvarado-Ramy, Francisco; Roohi, Shah; Rotz, Lisa D; Tappero, Jordan; Washburn, Faith M; Watkins, James; Pesik, Nicki

    2014-12-12

    In response to the largest recognized Ebola virus disease epidemic now occurring in West Africa, the governments of affected countries, CDC, the World Health Organization (WHO), and other international organizations have collaborated to implement strategies to control spread of the virus. One strategy recommended by WHO calls for countries with Ebola transmission to screen all persons exiting the country for "unexplained febrile illness consistent with potential Ebola infection." Exit screening at points of departure is intended to reduce the likelihood of international spread of the virus. To initiate this strategy, CDC, WHO, and other global partners were invited by the ministries of health of Guinea, Liberia, and Sierra Leone to assist them in developing and implementing exit screening procedures. Since the program began in August 2014, an estimated 80,000 travelers, of whom approximately 12,000 were en route to the United States, have departed by air from the three countries with Ebola transmission. Procedures were implemented to deny boarding to ill travelers and persons who reported a high risk for exposure to Ebola; no international air traveler from these countries has been reported as symptomatic with Ebola during travel since these procedures were implemented.

  5. Toward electron exit wave tomography of amorphous materials at atomic resolution

    Energy Technology Data Exchange (ETDEWEB)

    Borisenko, Konstantin B., E-mail: konstantin.borisenko@materials.ox.ac.uk [Department of Materials, University of Oxford, Parks Road, Oxford OX1 3PH (United Kingdom); Moldovan, Grigore [Department of Materials, University of Oxford, Parks Road, Oxford OX1 3PH (United Kingdom); Kirkland, Angus I., E-mail: angus.kirkland@materials.ox.ac.uk [Department of Materials, University of Oxford, Parks Road, Oxford OX1 3PH (United Kingdom); Van Dyck, Dirk [Department of Physics, University of Antwerp, Groenenborgerlaan 171, 2020 Antwerp (Belgium); Tang, Hsin-Yu; Chen, Fu-Rong [Department of Engineering and System Science, National Tsing Hua University, Kuang-Fu Road, 300 Hsinchu, Taiwan (China)

    2012-09-25

    Highlights: Black-Right-Pointing-Pointer We suggest a novel electron exit wave tomography approach to obtain three dimensional atomic structures of amorphous materials. Black-Right-Pointing-Pointer Theoretical tests using a model of amorphous Si doped with Au show that it is feasible to reconstruct both Si and Au atoms positions. Black-Right-Pointing-Pointer Reconstructions of the strongly scattering Au atoms positions appear to be insensitive to typical experimental errors. - Abstract: We suggest to use electron exit wave phase for tomographic reconstruction of structure of Au-doped amorphous Si with atomic resolution. In the present theoretical investigation into the approach it is found that the number of projections and the accuracy of defocus in the focal series restoration are the main factors that contribute to the final resolution. Although resolution is ultimately limited by these factors, phase shifts in the exit wave are sufficient to identify the position of Au atoms in an amorphous Si needle model, even when only 19 projections with defocus error of 4 nm are used. Electron beam damage will probably further limit the resolution of such tomographic reconstructions, however beam damage can be mitigated using lower accelerating voltages.

  6. A balance of FGF and BMP signals regulates cell cycle exit and Equarin expression in lens cells

    Science.gov (United States)

    Jarrin, Miguel; Pandit, Tanushree; Gunhaga, Lena

    2012-01-01

    In embryonic and adult lenses, a balance of cell proliferation, cell cycle exit, and differentiation is necessary to maintain physical function. The molecular mechanisms regulating the transition of proliferating lens epithelial cells to differentiated primary lens fiber cells are poorly characterized. To investigate this question, we used gain- and loss-of-function analyses to modulate fibroblast growth factor (FGF) and/or bone morphogenetic protein (BMP) signals in chick lens/retina explants. Here we show that FGF activity plays a key role for proliferation independent of BMP signals. Moreover, a balance of FGF and BMP signals regulates cell cycle exit and the expression of Ccdc80 (also called Equarin), which is expressed at sites where differentiation of lens fiber cells occurs. BMP activity promotes cell cycle exit and induces Equarin expression in an FGF-dependent manner. In contrast, FGF activity is required but not sufficient to induce cell cycle exit or Equarin expression. Furthermore, our results show that in the absence of BMP activity, lens cells have increased cell cycle length or are arrested in the cell cycle, which leads to decreased cell cycle exit. Taken together, these findings suggest that proliferation, cell cycle exit, and early differentiation of primary lens fiber cells are regulated by counterbalancing BMP and FGF signals. PMID:22718906

  7. Swallowing endoscopy findings in Huntington's disease: a case report.

    Science.gov (United States)

    Alves, Thaís Coelho; Cola, Paula Cristina; Santos, Rarissa Rúbia Dallaqua Dos; Motonaga, Suely Mayumi; Silva, Roberta Gonçalves da

    2016-01-01

    Huntington's disease (HD) is a degenerative genetic disorder with autosomal-dominant transmission. The triad of symptoms of this disease consists of psychiatric disorders, jerky movements, and dementia. Oropharyngeal dysphagia, which is more evident with disease progression, is also present. Few studies have addressed the swallowing characteristics using objective analysis in this population. The purpose of this research was to describe the swallowing endoscopic findings of the pharyngeal phase in HD. This is a cross-sectional study addressing a clinical case which included two individuals of the same family, male, 32 and 63 years old, designated as individual A and individual B, with progression of the disease for five and 13 years, respectively. Consistent liquid, nectar, and puree were offered during the evaluation. There was presence of posterior oral spillage in liquid and nectar, small amount of pharyngeal residues, and no laryngeal penetration or aspiration in the individuals with HD in this study.

  8. Determination of the availability of core exit thermocouples during severe accident situations

    International Nuclear Information System (INIS)

    Edson, J.L.

    1985-04-01

    This report presents the findings and recommendations of the Nuclear Power Plant Instrumentation Evaluation (NPPIE) program concerning signal validation methods to determine the on-line availability of core exit thermocouples during accident situations. Methods of selecting appropriate signal validation techniques are discussed and sources of error identified. This report shows that through the use of these techniques the existence of high-temperature-caused errors may be detected as they occur. Specific recommendations for application of selected signal validation techniques to core exit thermocouples and other measurement systems are made. 23 refs., 22 figs., 3 tabs

  9. Molecular signature of cell cycle exit induced in human T lymphoblasts by IL-2 withdrawal

    Directory of Open Access Journals (Sweden)

    Pfeifer Aleksandra

    2009-06-01

    Full Text Available Abstract Background The molecular mechanisms of cell cycle exit are poorly understood. Studies on lymphocytes at cell cycle exit after growth factor deprivation have predominantly focused on the initiation of apoptosis. We aimed to study gene expression profile of primary and immortalised IL-2-dependent human T cells forced to exit the cell cycle by growth factor withdrawal, before apoptosis could be evidenced. Results By the Affymetrix microarrays HG-U133 2.0 Plus, 53 genes were distinguished as differentially expressed before and soon after IL-2 deprivation. Among those, PIM1, BCL2, IL-8, HBEGF, DUSP6, OSM, CISH, SOCS2, SOCS3, LIF and IL13 were down-regulated and RPS24, SQSTM1, TMEM1, LRRC8D, ECOP, YY1AP1, C1orf63, ASAH1, SLC25A46 and MIA3 were up-regulated. Genes linked to transcription, cell cycle, cell growth, proliferation and differentiation, cell adhesion, and immune functions were found to be overrepresented within the set of the differentially expressed genes. Conclusion Cell cycle exit of the growth factor-deprived T lymphocytes is characterised by a signature of differentially expressed genes. A coordinate repression of a set of genes known to be induced during T cell activation is observed. However, growth arrest following exit from the cell cycle is actively controlled by several up-regulated genes that enforce the non-dividing state. The identification of genes involved in cell cycle exit and quiescence provides new hints for further studies on the molecular mechanisms regulating the non-dividing state of a cell, the mechanisms closely related to cancer development and to many biological processes.

  10. The Effect of Exit-Site Antibacterial Honey Versus Nasal Mupirocin Prophylaxis on the Microbiology and Outcomes of Peritoneal Dialysis-Associated Peritonitis and Exit-Site Infections: A Sub-Study of the Honeypot Trial.

    Science.gov (United States)

    Zhang, Lei; Badve, Sunil V; Pascoe, Elaine M; Beller, Elaine; Cass, Alan; Clark, Carolyn; de Zoysa, Janak; Isbel, Nicole M; McTaggart, Steven; Morrish, Alicia T; Playford, E Geoffrey; Scaria, Anish; Snelling, Paul; Vergara, Liza A; Hawley, Carmel M; Johnson, David W

    2015-12-01

    ♦ The HONEYPOT study recently reported that daily exit-site application of antibacterial honey was not superior to nasal mupirocin prophylaxis for preventing overall peritoneal dialysis (PD)-related infection. This paper reports a secondary outcome analysis of the HONEYPOT study with respect to exit-site infection (ESI) and peritonitis microbiology, infectious hospitalization and technique failure. ♦ A total of 371 PD patients were randomized to daily exit-site application of antibacterial honey plus usual exit-site care (N = 186) or intranasal mupirocin prophylaxis (in nasal Staphylococcus aureus carriers only) plus usual exit-site care (control, N = 185). Groups were compared on rates of organism-specific ESI and peritonitis, peritonitis- and infection-associated hospitalization, and technique failure (PD withdrawal). ♦ The mean peritonitis rates in the honey and control groups were 0.41 (95% confidence interval [CI] 0.32 - 0.50) and 0.41 (95% CI 0.33 - 0.49) episodes per patient-year, respectively (incidence rate ratio [IRR] 1.01, 95% CI 0.75 - 1.35). When specific causative organisms were examined, no differences were observed between the groups for gram-positive (IRR 0.99, 95% CI 0.66 - 1.49), gram-negative (IRR 0.71, 95% CI 0.39 - 1.29), culture-negative (IRR 2.01, 95% CI 0.91 - 4.42), or polymicrobial peritonitis (IRR 1.08, 95% CI 0.36 - 3.20). Exit-site infection rates were 0.37 (95% CI 0.28 - 0.45) and 0.33 (95% CI 0.26 - 0.40) episodes per patient-year for the honey and control groups, respectively (IRR 1.12, 95% CI 0.81 - 1.53). No significant differences were observed between the groups for gram-positive (IRR 1.10, 95% CI 0.70 - 1.72), gram-negative (IRR: 0.85, 95% CI 0.46 - 1.58), culture-negative (IRR 1.88, 95% CI 0.67 - 5.29), or polymicrobial ESI (IRR 1.00, 95% CI 0.40 - 2.54). Times to first peritonitis-associated and first infection-associated hospitalization were similar in the honey and control groups. The rates of technique failure (PD

  11. EFFECT OF REVENUE INSURANCE ON ENTRY AND EXIT DECISIONS IN TABLE GRAPE PRODUCTION: A REAL OPTION APPROACH

    OpenAIRE

    Seo, Sangtaek; Salin, Victoria; Mitchell, Paul D.; Leatham, David J.

    2004-01-01

    This study determines the entry and exit thresholds of table grape farming with irreversible investment under uncertainty. Real option approach is adopted to consider the investment and management flexibility. Also revenue insurance is introduced to consider the effect of the risk management programs on the entry and exit thresholds. Results show that revenue insurance increases the entry and exit thresholds by 1% and 4%, respectively, thus discouraging new investment and current farming, as ...

  12. The Exit Gradient As a Measure of Groundwater Dependency of Watershed Ecosystem Services

    Science.gov (United States)

    Faulkner, B. R.; Canfield, T. J.; Justin, G. F.

    2014-12-01

    Flux of groundwater to surface water is often of great interest for the determination of the groundwater dependency of ecosystem services, such as maintenance of wetlands and of baseflow as a contributor to stream channel storage. It is difficult to measure. Most methods are based on coarse mass balance estimates or seepage meters. One drawback of these methods is they are not entirely spatially explicit. The exit gradient is commonly used in engineering studies of hydraulic structures affected by groundwater flow. It can be simply defined in the groundwater modeling context as the ratio of the difference between the computed head and the land surface elevation, for each computational cell, to the thickness of the cell, as it varies in space. When combined with calibrated groundwater flow models, it also has the potential to be useful in watershed scale evaluations of groundwater dependency in a spatially explicit way. We have taken advantage of calibrated models for the Calapooia watershed, Oregon, to map exit gradients for the watershed. Streams in the Calapooia are hydraulically well connected with groundwater. Not surprisingly, we found large variations in exit gradients between wet and dry season model runs, supporting the notion of stream expansion, as observed in the field, which may have a substantial influence on water quality. We have mapped the exit gradients in the wet and dry seasons, and compared them to regions which have been mapped in wetland surveys. Those classified as Palustrine types fell largest in the area of contribution from groundwater. In many cases, substantially high exit gradients, even on average, did not correspond to mapped wetland areas, yet nutrient retention ecosystem services may still be playing a role in these areas. The results also reinforce the notion of the importance of baseflow to maintenance of stream flow, even in the dry summer season in this Temperate/Mediterranean climate. Exit gradient mapping is a simple, yet

  13. Hydroelastic slamming of flexible wedges: Modeling and experiments from water entry to exit

    Science.gov (United States)

    Shams, Adel; Zhao, Sam; Porfiri, Maurizio

    2017-03-01

    Fluid-structure interactions during hull slamming are of great interest for the design of aircraft and marine vessels. The main objective of this paper is to establish a semi-analytical model to investigate the entire hydroelastic slamming of a wedge, from the entry to the exit phase. The structural dynamics is described through Euler-Bernoulli beam theory and the hydrodynamic loading is estimated using potential flow theory. A Galerkin method is used to obtain a reduced order modal model in closed-form, and a Newmark-type integration scheme is utilized to find an approximate solution. To benchmark the proposed semi-analytical solution, we experimentally investigate fluid-structure interactions through particle image velocimetry (PIV). PIV is used to estimate the velocity field, and the pressure is reconstructed by solving the incompressible Navier-Stokes equations from PIV data. Experimental results confirm that the flow physics and free-surface elevation during water exit are different from water entry. While water entry is characterized by positive values of the pressure field, with respect to the atmospheric pressure, the pressure field during water exit may be less than atmospheric. Experimental observations indicate that the location where the maximum pressure in the fluid is attained moves from the pile-up region to the keel, as the wedge reverses its motion from the entry to the exit stage. Comparing experimental results with semi-analytical findings, we observe that the model is successful in predicting the free-surface elevation and the overall distribution of the hydrodynamic loading on the wedge. These integrated experimental and theoretical analyses of water exit problems are expected to aid in the design of lightweight structures, which experience repeated slamming events during their operation.

  14. Exit interviews administered to patients participating in the COSTOP placebo controlled randomised trial in Uganda

    Directory of Open Access Journals (Sweden)

    Andrew Nunn

    2016-08-01

    Discussion: The exit interview demonstrated that there was some evidence of open label drug being taken by the participants. However, the results from the interview do not suggest that the trial results would have been seriously compromised. We would recommend the exit interview as a valuable way of assessing adherence to trial procedures.

  15. Driving decisions when leaving electronic music dance events: driver, passenger, and group effects.

    Science.gov (United States)

    Johnson, Mark B; Voas, Robert B; Miller, Brenda A

    2012-01-01

    The goal of this article was to identify characteristics of drivers and passengers that predicted peer groups whose drivers exit dance clubs with alcohol levels indicative of impairment (blood alcohol content [BAC] ≥ 0.05 g/dL). We used the portal survey methodology to randomly sample groups of electronic music dance event (EMDE) patrons as they entered and exited a club. From May through November 2010, data were collected from 38 EMDEs hosted by 8 clubs in the San Francisco Bay area. Data included in these analyses are results from breath samples for measuring BAC and self-report data on demographics, recent drinking history drinking, drinking intentions, travel to and from the clubs, and the familiarity/experience with other group members. These data were collected from a subset of 175 drivers and 272 passengers. Although drivers drank less than passengers, one driver in 5 groups had a BAC indicative of elevated crash risk (BAC ≥ 0.05 g/dL). Groups of drivers and/or passengers with a recent history of binge drinking were more likely to have drivers with BACs ≥ 0.05 g/dL. One unanticipated finding was that drivers who knew more group members relatively well were more likely to exit the club with a BAC ≥ 0.05 g/dL. Additionally, we found that groups with all female passengers were at greater risk for having a driver whose BAC was ≥ 0.05 g/dL. Some group characteristics predicted drivers who exit clubs with BACs ≥ 0.05 g/dL. One intervention strategy to promote safety might be to encourage group members to reconsider who is sober enough to drive away from the club; for some groups, a change of drivers would be a safer choice, because a passenger may have a relatively safe BAC. Groups of females appear to have a particularly elevated risk of having a driver whose BAC exceeds 0.05 g/dL, and new intervention efforts should be particularly directed to these at-risk groups.

  16. New approach of a traditional analysis for predicting near-exit jet liquid instabilities

    Science.gov (United States)

    Jaramillo, Guillermo; Collicott, Steven

    2015-11-01

    Traditional linear instability theory for round liquid jets requires an exit-plane velocity profile be assumed so as to derive the characteristic growth rates and wavelengths of instabilities. This requires solving an eigenvalue problem for the Rayleigh Equation. In this new approach, a hyperbolic tangent velocity profile is assumed at the exit-plane of a round jet and a comparison is made with a hyperbolic secant profile. Temporal and Spatial Stability Analysis (TSA and SSA respectively) are the employed analytical tools to compare results of predicted most-unstable wavelengths from the given analytical velocity profiles and from previous experimental work. The local relevance of the velocity profile in the near-exit region of a liquid jet and the validity of an inviscid formulation through the Rayleigh equation are discussed as well. A comparison of numerical accuracy is made between two different mathematical approaches for the hyperbolic tangent profile with and without the Ricatti transformation. Reynolds number based on the momentum thickness of the boundary layer at the exit plane non-dimensionalizes the problem and, the Re range, based on measurements by Portillo in 2011, is 185 to 600. Wavelength measurements are taken from Portillo's experiment. School of Mechanical Engineering at Universidad del Valle, supported by a grant from Fulbright and Colciencias. Ph.D. student at the School of Aeronautics and Astronautics Purdue University.

  17. Altered selenium status in Huntington's disease: neuroprotection by selenite in the N171-82Q mouse model.

    Science.gov (United States)

    Lu, Zhen; Marks, Eileen; Chen, Jianfang; Moline, Jenna; Barrows, Lorraine; Raisbeck, Merl; Volitakis, Irene; Cherny, Robert A; Chopra, Vanita; Bush, Ashley I; Hersch, Steven; Fox, Jonathan H

    2014-11-01

    Disruption of redox homeostasis is a prominent feature in the pathogenesis of Huntington's disease (HD). Selenium an essential element nutrient that modulates redox pathways and has been reported to provide protection against both acute neurotoxicity (e.g. methamphetamine) and chronic neurodegeneration (e.g. tauopathy) in mice. The objective of our study was to investigate the effect of sodium selenite, an inorganic form of selenium, on behavioral, brain degeneration and biochemical outcomes in the N171-82Q Huntington's disease mouse model. HD mice, which were supplemented with sodium selenite from 6 to 14 weeks of age, demonstrated increased motor endurance, decreased loss of brain weight, decreased mutant huntingtin aggregate burden and decreased brain oxidized glutathione levels. Biochemical studies revealed that selenite treatment reverted HD-associated changes in liver selenium and plasma glutathione in N171-82Q mice and had effects on brain selenoprotein transcript expression. Further, we found decreased brain selenium content in human autopsy brain. Taken together, we demonstrate a decreased selenium phenotype in human and mouse HD and additionally show some protective effects of selenite in N171-82Q HD mice. Modification of selenium metabolism results in beneficial effects in mouse HD and thus may represent a therapeutic strategy. Copyright © 2014 Elsevier Inc. All rights reserved.

  18. Ecologies of ideologies: Explaining party entry and exit in West-European parliaments, 1945-2013.

    Science.gov (United States)

    van de Wardt, Marc; Berkhout, Joost; Vermeulen, Floris

    2017-06-01

    This study introduces a population-ecological approach to the entry and exit of political parties. A primary proposition of population ecology is that organizational entry and exit depends on the number of organizations already present: that is, density. We propose that political parties mainly experience competition from parties in the same ideological niche (left, centre, right). Pooled time-series analyses of 410 parties, 263 elections and 18 West-European countries largely support our expectations. We find that political parties are more likely to exit when density within their niche increases. Also there is competition between adjacent ideological niches, i.e. between centrist and right-wing niches. In contrast to our expectations, neither density nor institutional rules impact party entry. This raises important questions about the rationale of prospective entrants.

  19. Prediction of midline dose from entrance ad exit dose using OSLD measurements for total irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Chang Heon; Park, Jong Min; Park, So Yeon; Chun, Min Soo; Han, Ji Hye; Cho, Jin Dong; Kim, Jung In [Dept. of Radiation Oncology, Seoul National University Hospital, Seoul (Korea, Republic of)

    2017-06-15

    This study aims to predict the midline dose based on the entrance and exit doses from optically stimulated luminescence detector (OSLD) measurements for total body irradiation (TBI). For TBI treatment, beam data sets were measured for 6 MV and 15 MV beams. To evaluate the tissue lateral effect of various thicknesses, the midline dose and peak dose were measured using a solid water phantom (SWP) and ion chamber. The entrance and exit doses were measured using OSLDs. OSLDs were attached onto the central beam axis at the entrance and exit surfaces of the phantom. The predicted midline dose was evaluated as the sum of the entrance and exit doses by OSLD measurement. The ratio of the entrance dose to the exit dose was evaluated at various thicknesses. The ratio of the peak dose to the midline dose was 1.12 for a 30 cm thick SWP at both energies. When the patient thickness is greater than 30 cm, the 15 MV should be used to ensure dose homogeneity. The ratio of the entrance dose to the exit dose was less than 1.0 for thicknesses of less than 30 cm and 40 cm at 6 MV and 15 MV, respectively. Therefore, the predicted midline dose can be underestimated for thinner body. At 15 MV, the ratios were approximately 1.06 for a thickness of 50 cm. In cases where adult patients are treated with the 15 MV photon beam, it is possible for the predicted midline dose to be overestimated for parts of the body with a thickness of 50 cm or greater. The predicted midline dose and OSLD-measured midline dose depend on the phantom thickness. For in-vivo dosimetry of TBI, the measurement dose should be corrected in order to accurately predict the midline dose.

  20. The use of an electronic portal imaging device for exit dosimetry and quality control measurements

    International Nuclear Information System (INIS)

    Kirby, Michael C.; Williams, Peter C.

    1995-01-01

    Purpose: To determine ways in which electronic portal imaging devices (EPIDs) could be used to (a) measure exit doses for external beam radiotherapy and (b) perform quality control checks on linear accelerators. Methods and Materials: When imaging, our fluoroscopic EPID adjusts the gain, offset, and frame acquisition time of the charge coupled device (CCD) camera automatically, to allow for the range of photon transmissions through the patient, and to optimize the signal-to-noise ratio. However, our EPID can be programmed to act as an integrating dosemeter. EPID dosemeter measurements were made for 20 MV photons, for different field sizes and thicknesses of unit density phantom material placed at varying exit surface to detector distances. These were compared with simultaneous Silicon diode exit dose measurements. Our exit dosimetry technique was verified using an anthropomorphic type phantom, and some initial measurements have been made for patients treated with irregularly shaped 20 MV x-ray fields. In this dosimetry mode, our EPID was also used to measure certain quality control parameters, x-ray field flatness, and the verification of segmented intensity modulated field prescriptions. Results: Configured for dosimetry, our EPID exhibited a highly linear response, capable of resolving individual monitor units. Exit doses could be measured to within about 3% of that measured using Silicon diodes. Field flatness was determined to within 1.5% of Farmer dosemeter measurements. Segmented intensity modulated fields can be easily verified. Conclusions: Our EPID has the versatility to assess a range of parameters pertinent to the delivery of high quality, high precision radiotherapy. When configured appropriately, it can measure exit doses in vivo, with reasonable accuracy, perform certain quick quality control checks, and analyze segmented intensity modulated treatment fields

  1. The role of the amygdala during emotional processing in Huntington's disease: from pre-manifest to late stage disease.

    Science.gov (United States)

    Mason, Sarah L; Zhang, Jiaxiang; Begeti, Faye; Guzman, Natalie Valle; Lazar, Alpar S; Rowe, James B; Barker, Roger A; Hampshire, Adam

    2015-04-01

    Deficits in emotional processing can be detected in the pre-manifest stage of Huntington's disease and negative emotion recognition has been identified as a predictor of clinical diagnosis. The underlying neuropathological correlates of such deficits are typically established using correlative structural MRI studies. This approach does not take into consideration the impact of disruption to the complex interactions between multiple brain circuits on emotional processing. Therefore, exploration of the neural substrates of emotional processing in pre-manifest HD using fMRI connectivity analysis may be a useful way of evaluating the way brain regions interrelate in the period prior to diagnosis. We investigated the impact of predicted time to disease onset on brain activation when participants were exposed to pictures of faces with angry and neutral expressions, in 20 pre-manifest HD gene carriers and 23 healthy controls. On the basis of the results of this initial study went on to look at amygdala dependent cognitive performance in 79 Huntington's disease patients from a cross-section of disease stages (pre-manifest to late disease) and 26 healthy controls, using a validated theory of mind task: "the Reading the Mind in the Eyes Test" which has been previously been shown to be amygdala dependent. Psychophysiological interaction analysis identified reduced connectivity between the left amygdala and right fusiform facial area in pre-manifest HD gene carriers compared to controls when viewing angry compared to neutral faces. Change in PPI connectivity scores correlated with predicted time to disease onset (r=0.45, pneural networks underlying social cognition and emotional processing can be detected prior to clinical diagnosis in Huntington's disease. Connectivity between the amygdala and other brain regions is impacted by the disease process in pre-manifest HD and may therefore be a useful way of identifying participants who are approaching a clinical diagnosis

  2. Exit, voice and loyalty in Kenya's French bean industry: What ...

    African Journals Online (AJOL)

    response affected their continued participation in the supermarket business. It applies Hirschman's concept of exit, voice and loyalty to assess the strategies used by ... This strategy of compliance with IFSS has since become the model in

  3. Mitochondrial Metabolism in a Large-Animal Model of Huntington Disease: The Hunt for Biomarkers in the Spermatozoa of Presymptomatic Minipigs

    Czech Academy of Sciences Publication Activity Database

    Křížová, J.; Štufková, H.; Rodinová, M.; Mačáková, Monika; Bohuslavová, Božena; Vidinská, Daniela; Klíma, Jiří; Ellederová, Zdeňka; Pavlok, Antonín; Howland, D. S.; Zeman, J.; Motlík, Jan; Hansíková, H.

    2017-01-01

    Roč. 17, 4-5 (2017), s. 213-226 ISSN 1660-2854 R&D Projects: GA MŠk 7F14308; GA MŠk(CZ) LO1609 Institutional support: RVO:67985904 Keywords : Huntington disease * large animal model * mutant huntingtin Subject RIV: EA - Cell Biology OBOR OECD: Cell biology Impact factor: 2.842, year: 2016

  4. Transgenic Rat Model of Huntington's Disease: A Histopathological Study and Correlations with Neurodegenerative Process in the Brain of HD Patients.

    Czech Academy of Sciences Publication Activity Database

    Mazurová, Y.; Anděrová, Miroslava; Němečková, I.; Bezrouk, A.

    2014-01-01

    Roč. 2014, Aug 03 (2014), s. 291531 ISSN 2314-6133 R&D Projects: GA ČR(CZ) GBP304/12/G069 Grant - others:GA MŠk(CZ) Prvouk P37 Institutional support: RVO:68378041 Keywords : Huntington's Disease * neurodegenerative process in the brain Subject RIV: FH - Neurology Impact factor: 1.579, year: 2014

  5. High resolution electron exit wave reconstruction from a diffraction pattern using Gaussian basis decomposition

    International Nuclear Information System (INIS)

    Borisenko, Konstantin B; Kirkland, Angus I

    2014-01-01

    We describe an algorithm to reconstruct the electron exit wave of a weak-phase object from single diffraction pattern. The algorithm uses analytic formulations describing the diffraction intensities through a representation of the object exit wave in a Gaussian basis. The reconstruction is achieved by solving an overdetermined system of non-linear equations using an easily parallelisable global multi-start search with Levenberg-Marquard optimisation and analytic derivatives

  6. Hedgehog signaling acts with the temporal cascade to promote neuroblast cell cycle exit.

    Directory of Open Access Journals (Sweden)

    Phing Chian Chai

    Full Text Available In Drosophila postembryonic neuroblasts, transition in gene expression programs of a cascade of transcription factors (also known as the temporal series acts together with the asymmetric division machinery to generate diverse neurons with distinct identities and regulate the end of neuroblast proliferation. However, the underlying mechanism of how this "temporal series" acts during development remains unclear. Here, we show that Hh signaling in the postembryonic brain is temporally regulated; excess (earlier onset of Hh signaling causes premature neuroblast cell cycle exit and under-proliferation, whereas loss of Hh signaling causes delayed cell cycle exit and excess proliferation. Moreover, the Hh pathway functions downstream of Castor but upstream of Grainyhead, two components of the temporal series, to schedule neuroblast cell cycle exit. Interestingly, hh is likely a target of Castor. Hence, Hh signaling provides a link between the temporal series and the asymmetric division machinery in scheduling the end of neurogenesis.

  7. Assessing and Modulating Kynurenine Pathway Dynamics in Huntington's Disease: Focus on Kynurenine 3-Monooxygenase.

    Science.gov (United States)

    Sathyasaikumar, Korrapati V; Breda, Carlo; Schwarcz, Robert; Giorgini, Flaviano

    2018-01-01

    The link between disturbances in kynurenine pathway (KP) metabolism and Huntington's disease (HD) pathogenesis has been explored for a number of years. Several novel genetic and pharmacological tools have recently been developed to modulate key regulatory steps in the KP such as the reaction catalyzed by the enzyme kynurenine 3-monooxygenase (KMO). This insight has offered new options for exploring the mechanistic link between this metabolic pathway and HD, and provided novel opportunities for the development of candidate drug-like compounds. Here, we present an overview of the field, focusing on some novel approaches for interrogating the pathway experimentally.

  8. Decreased mitochondrial density and ultrastructural changes of mitochondria in cultivated skin fibroblasts of patients with Huntington´s disease

    Czech Academy of Sciences Publication Activity Database

    Rodinová, M.; Marková, M.; Kratochvílová, H.; Kučerová, I.; Tesařová, M.; Lišková, Irena; Klempíř, J.; Roth, J.; Zeman, J.; Hansíková, H.

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 20-21 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) 7F14308 Institutional support: RVO:67985904 Keywords : Huntington ´s disease * fibroblasts * mitochondrial ultrastructure Subject RIV: FH - Neurology

  9. Condensate subcooling near tube exit during horizontal in-tube condensation

    International Nuclear Information System (INIS)

    Hashizume, K.; Abe, N.; Ozeki, T.

    1992-01-01

    In-tube condensation is encountered in various applications for heat exchangers, such as domestic air-conditioning equipment, industrial air-cooled condensers, and moisture separator reheaters (MSRs) for nuclear power pants. Numerous research work has been conducted to predict the condensation heat transfer coefficient, and we have now enough information for thermal design of heat exchangers with horizontal in-tube condensation. Most of the research is analytical and/or experimental work in the annular or stratified flow regime, or experimental work on bulk condensation, i.e., from saturated vapor to complete condensation. On the other hand, there exist few data about the heat transfer phenomena in the very lower-quality region near the tube exit. The purpose of this paper is to clarify the condensation heat transfer phenomena near the tube exit experimentally and analytically, and to predict the degree of condensate subcooling

  10. Haplotype-based stratification of Huntington's disease.

    Science.gov (United States)

    Chao, Michael J; Gillis, Tammy; Atwal, Ranjit S; Mysore, Jayalakshmi Srinidhi; Arjomand, Jamshid; Harold, Denise; Holmans, Peter; Jones, Lesley; Orth, Michael; Myers, Richard H; Kwak, Seung; Wheeler, Vanessa C; MacDonald, Marcy E; Gusella, James F; Lee, Jong-Min

    2017-11-01

    Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by expansion of a CAG trinucleotide repeat in HTT, resulting in an extended polyglutamine tract in huntingtin. We and others have previously determined that the HD-causing expansion occurs on multiple different haplotype backbones, reflecting more than one ancestral origin of the same type of mutation. In view of the therapeutic potential of mutant allele-specific gene silencing, we have compared and integrated two major systems of HTT haplotype definition, combining data from 74 sequence variants to identify the most frequent disease-associated and control chromosome backbones and revealing that there is potential for additional resolution of HD haplotypes. We have used the large collection of 4078 heterozygous HD subjects analyzed in our recent genome-wide association study of HD age at onset to estimate the frequency of these haplotypes in European subjects, finding that common genetic variation at HTT can distinguish the normal and CAG-expanded chromosomes for more than 95% of European HD individuals. As a resource for the HD research community, we have also determined the haplotypes present in a series of publicly available HD subject-derived fibroblasts, induced pluripotent cells, and embryonic stem cells in order to facilitate efforts to develop inclusive methods of allele-specific HTT silencing applicable to most HD patients. Our data providing genetic guidance for therapeutic gene-based targeting will significantly contribute to the developments of rational treatments and implementation of precision medicine in HD.

  11. Ecologies of Ideologies: Explaining Party Entry and Exit in West-European Parliaments, 1945-2013

    OpenAIRE

    van de Wardt, M.; Berkhout, J.; Vermeulen, F.

    2017-01-01

    This study introduces a population-ecological approach to the entry and exit of political parties. A primary proposition of population ecology is that organizational entry and exit depends on the number of organizations already present: that is, density. We propose that political parties mainly experience competition from parties in the same ideological niche (left, centre, right). Pooled time-series analyses of 410 parties, 263 elections and 18 West-European countries largely support our exp...

  12. Huntington Disease - principles and practice of nutritional management.

    Science.gov (United States)

    Zukiewicz-Sobczak, Wioletta; Król, Renata; Wróblewska, Paula; Piątek, Jacek; Gibas-Dorna, Magdalena

    2014-01-01

    Huntington disease (HD) is a degenerative brain disease clinically manifested by the characteristic triad: physical symptoms including involuntary movements and poor coordination, cognitive changes with less ability to organize routine tasks, and some emotional and behavioral disturbances. For patients with HD, feeding is one of the problems they have to face. People with HD often have lower than average body weight and struggle with malnutrition. As a part of therapy, good nutrition is an intervention maintaining health and functional ability for maximally prolonged time. In the early stages of HD, small amounts of blenderized foods given orally are recommended. In more advanced stages, enteral nutrition is essential using gastric, or jejunal tubes for short term. Most severe cases require gastrostomy or gastrojejunostomy. Although enteral feeding is well tolerated by most of the patients, a number of complications may occur, including damage to the nose, pharynx, or esophagus, aspiration pneumonia, sinusitis, metabolic imbalances due to improper nutrient and fluid supply, adverse effects affecting gastrointestinal system, and refeeding syndrome. Copyright © 2014 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  13. Spanish exit polls. Sampling error or nonresponse bias?

    Directory of Open Access Journals (Sweden)

    Pavía, Jose M.

    2016-09-01

    Full Text Available Countless examples of misleading forecasts on behalf of both pre-election and exit polls can be found all over the world. Non-representative samples due to differential nonresponse have been claimed as being the main reason for inaccurate exit-poll projections. In real inference problems, it is seldom possible to compare estimates and true values. Electoral forecasts are an exception. Comparisons between estimates and final outcomes can be carried out once votes have been tallied. In this paper, we examine the raw data collected in seven exit polls conducted in Spain and test the likelihood that the data collected in each sampled voting location can be considered as a random sample of actual results. Knowing the answer to this is relevant for both electoral analysts and forecasters as, if the hypothesis is rejected, the shortcomings of the collected data would need amending. Analysts could improve the quality of their computations by implementing local correction strategies. We find strong evidence of nonsampling error in Spanish exit polls and evidence that the political context matters. Nonresponse bias is larger in polarized elections and in a climate of fearExiste un gran número de ejemplos de predicciones inexactas obtenidas a partir tanto de encuestas pre-electorales como de encuestas a pie de urna a lo largo del mundo. La presencia de tasas de no-respuesta diferencial entre distintos tipos de electores ha sido la principal razón esgrimida para justificar las proyecciones erróneas en las encuestas a pie de urna. En problemas de inferencia rara vez es posible comparar estimaciones y valores reales. Las predicciones electorales son una excepción. La comparación entre estimaciones y resultados finales puede realizarse una vez los votos han sido contabilizados. En este trabajo, examinamos los datos brutos recogidos en siete encuestas a pie de urna realizadas en España y testamos la hipótesis de que los datos recolectados en cada punto

  14. Repressive histone methylation regulates cardiac myocyte cell cycle exit.

    Science.gov (United States)

    El-Nachef, Danny; Oyama, Kyohei; Wu, Yun-Yu; Freeman, Miles; Zhang, Yiqiang; Robb MacLellan, W

    2018-05-22

    Mammalian cardiac myocytes (CMs) stop proliferating soon after birth and subsequent heart growth comes from hypertrophy, limiting the adult heart's regenerative potential after injury. The molecular events that mediate CM cell cycle exit are poorly understood. To determine the epigenetic mechanisms limiting CM cycling in adult CMs (ACMs) and whether trimethylation of lysine 9 of histone H3 (H3K9me3), a histone modification associated with repressed chromatin, is required for the silencing of cell cycle genes, we developed a transgenic mouse model where H3K9me3 is specifically removed in CMs by overexpression of histone demethylase, KDM4D. Although H3K9me3 is found across the genome, its loss in CMs preferentially disrupts cell cycle gene silencing. KDM4D binds directly to cell cycle genes and reduces H3K9me3 levels at these promotors. Loss of H3K9me3 preferentially leads to increased cell cycle gene expression resulting in enhanced CM cycling. Heart mass was increased in KDM4D overexpressing mice by postnatal day 14 (P14) and continued to increase until 9-weeks of age. ACM number, but not size, was significantly increased in KDM4D expressing hearts, suggesting CM hyperplasia accounts for the increased heart mass. Inducing KDM4D after normal development specifically in ACMs resulted in increased cell cycle gene expression and cycling. We demonstrated that H3K9me3 is required for CM cell cycle exit and terminal differentiation in ACMs. Depletion of H3K9me3 in adult hearts prevents and reverses permanent cell cycle exit and allows hyperplastic growth in adult hearts in vivo. Copyright © 2017. Published by Elsevier Ltd.

  15. The Right of Exit in the Context of Multiculturalism

    Directory of Open Access Journals (Sweden)

    Ana Maria D'Ávila Lopes

    2015-12-01

    Full Text Available http://dx.doi.org/10.5007/2177-7055.2015v36n71p155 The terrorist attack on the Twin Towers in the United States provoked heated discussions about the need to limit and control the performance of some cultural minorities, as well as to create mechanisms to protect members of these minorities against the decisions taken by the group. In this context, this paper aims to analyze the possibilities and limits of the right of exit in the context of Multiculturalism. To this end, a literature research was performed in national and foreign doctrine. After analyzing the data, it was found that the right to exit is a valuable mechanism for protecting members of cultural minorities, however, there are situations, especially in cases where the values of the group are internalized by the members, in which this right is insufficient and should be supplemented by other human rights.

  16. Exploring Genetic Factors Involved in Huntington Disease Age of Onset

    DEFF Research Database (Denmark)

    Valcárcel-Ocete, Leire; Alkorta-Aranburu, Gorka; Iriondo, Mikel

    2015-01-01

    age (motor AO or mAO). Multiple linear regression analyses were performed between genetic variation within 20 candidate genes and eAO or mAO, using DNA and clinical information of 253 HD patients from REGISTRY project. Gene expression analyses were carried out by RT-qPCR with an independent sample......Age of onset (AO) of Huntington disease (HD) is mainly determined by the length of the CAG repeat expansion (CAGexp) in exon 1 of the HTT gene. Additional genetic variation has been suggested to contribute to AO, although the mechanism by which it could affect AO is presently unknown. The aim...... of this study is to explore the contribution of candidate genetic factors to HD AO in order to gain insight into the pathogenic mechanisms underlying this disorder. For that purpose, two AO definitions were used: the earliest age with unequivocal signs of HD (earliest AO or eAO), and the first motor symptoms...

  17. KLF4 Nuclear Export Requires ERK Activation and Initiates Exit from Naive Pluripotency.

    Science.gov (United States)

    Dhaliwal, Navroop K; Miri, Kamelia; Davidson, Scott; Tamim El Jarkass, Hala; Mitchell, Jennifer A

    2018-04-10

    Cooperative action of a transcription factor complex containing OCT4, SOX2, NANOG, and KLF4 maintains the naive pluripotent state; however, less is known about the mechanisms that disrupt this complex, initiating exit from pluripotency. We show that, as embryonic stem cells (ESCs) exit pluripotency, KLF4 protein is exported from the nucleus causing rapid decline in Nanog and Klf4 transcription; as a result, KLF4 is the first pluripotency transcription factor removed from transcription-associated complexes during differentiation. KLF4 nuclear export requires ERK activation, and phosphorylation of KLF4 by ERK initiates interaction of KLF4 with nuclear export factor XPO1, leading to KLF4 export. Mutation of the ERK phosphorylation site in KLF4 (S132) blocks KLF4 nuclear export, the decline in Nanog, Klf4, and Sox2 mRNA, and differentiation. These findings demonstrate that relocalization of KLF4 to the cytoplasm is a critical first step in exit from the naive pluripotent state and initiation of ESC differentiation. Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.

  18. Organized medicine and Scandinavian professional unionism: hospital policies and exit options in Denmark and Sweden.

    Science.gov (United States)

    Heidenheimer, A J; Johansen, L N

    1985-01-01

    Strikes by junior hospital doctors over the issue of on-call remuneration in Denmark and Sweden in 1981 are analyzed to clarify the impact of public-sector cost-control policies on intra- and interprofessional solidarity within the Scandinavian professional peak associations. The junior doctors' grievances could find expression either through increased "voice" within the medical negotiating machinery, or by pursuing the exit option in having the medical associations quit the peak associations. The article explains why the "exit" option was selected in Denmark, while in Sweden the granting of additional voice helped persuade the medical association to withdraw its exit threat and to remain within the peak association. The two cases are interpreted as presaging a divergence in the paths being taken by the various Scandinavian welfare states.

  19. Ecologies of ideologies: Explaining party entry and exit in West-European parliaments, 1945–2013

    Science.gov (United States)

    Berkhout, Joost; Vermeulen, Floris

    2016-01-01

    This study introduces a population-ecological approach to the entry and exit of political parties. A primary proposition of population ecology is that organizational entry and exit depends on the number of organizations already present: that is, density. We propose that political parties mainly experience competition from parties in the same ideological niche (left, centre, right). Pooled time-series analyses of 410 parties, 263 elections and 18 West-European countries largely support our expectations. We find that political parties are more likely to exit when density within their niche increases. Also there is competition between adjacent ideological niches, i.e. between centrist and right-wing niches. In contrast to our expectations, neither density nor institutional rules impact party entry. This raises important questions about the rationale of prospective entrants. PMID:29046613

  20. Stimulating neural plasticity with real-time fMRI neurofeedback in Huntington's disease: A proof of concept study.

    Science.gov (United States)

    Papoutsi, Marina; Weiskopf, Nikolaus; Langbehn, Douglas; Reilmann, Ralf; Rees, Geraint; Tabrizi, Sarah J

    2018-03-01

    Novel methods that stimulate neuroplasticity are increasingly being studied to treat neurological and psychiatric conditions. We sought to determine whether real-time fMRI neurofeedback training is feasible in Huntington's disease (HD), and assess any factors that contribute to its effectiveness. In this proof-of-concept study, we used this technique to train 10 patients with HD to volitionally regulate the activity of their supplementary motor area (SMA). We collected detailed behavioral and neuroimaging data before and after training to examine changes of brain function and structure, and cognitive and motor performance. We found that patients overall learned to increase activity of the target region during training with variable effects on cognitive and motor behavior. Improved cognitive and motor performance after training predicted increases in pre-SMA grey matter volume, fMRI activity in the left putamen, and increased SMA-left putamen functional connectivity. Although we did not directly target the putamen and corticostriatal connectivity during neurofeedback training, our results suggest that training the SMA can lead to regulation of associated networks with beneficial effects in behavior. We conclude that neurofeedback training can induce plasticity in patients with Huntington's disease despite the presence of neurodegeneration, and the effects of training a single region may engage other regions and circuits implicated in disease pathology. © 2017 The Authors. Human Brain Mapping Published by Wiley Periodicals, Inc.

  1. Cognitive change in patients with Huntington disease on the Repeatable Battery for the Assessment of Neuropsychological Status.

    Science.gov (United States)

    Beglinger, Leigh J; Duff, Kevin; Allison, Jessica; Theriault, Danielle; O'Rourke, Justin J F; Leserman, Anne; Paulsen, Jane S

    2010-07-01

    Huntington disease (HD) is a neurodegenerative disease associated with cognitive, motor, and psychiatric deterioration over time. Although there is currently no cure for HD, there has been a surge of clinical trials available to patients with HD over the past 5 years. However, cognitive measures have generally been lacking from these trials. A brief, repeatable neuropsychological battery is needed to assess cognitive endpoints. The Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) may be useful for assessing change in interventional studies or for clinical monitoring. A total of 38 patients with HD were assessed using the RBANS, other cognitive tests, and the standardized HD battery (Unified Huntington's Disease Rating Scale, UHDRS) at two clinic visits approximately 16 months apart. The RBANS Attention Index, as well as individual subtest scores on Coding, Digit Span, List Recognition, Figure Copy, and Figure Recall all declined significantly over this interval. Performance on the UHDRS cognitive tests (Symbol Digit Modalities; Stroop Color, and Stroop Word) also declined, as did functional capacity. Results suggest that cognitive changes were detected both on established cognitive tasks used in HD research and on the RBANS in patients with measurable functional decline. The RBANS provided additional information about other cognitive domains affected (e.g., memory) and may be a useful measure for tracking longitudinal change.

  2. A smooth exit from eternal inflation?

    Science.gov (United States)

    Hawking, S. W.; Hertog, Thomas

    2018-04-01

    The usual theory of inflation breaks down in eternal inflation. We derive a dual description of eternal inflation in terms of a deformed Euclidean CFT located at the threshold of eternal inflation. The partition function gives the amplitude of different geometries of the threshold surface in the no-boundary state. Its local and global behavior in dual toy models shows that the amplitude is low for surfaces which are not nearly conformal to the round three-sphere and essentially zero for surfaces with negative curvature. Based on this we conjecture that the exit from eternal inflation does not produce an infinite fractal-like multiverse, but is finite and reasonably smooth.

  3. The functional implications of motor, cognitive, psychiatric, and social problem-solving states in Huntington's disease.

    Science.gov (United States)

    Van Liew, Charles; Gluhm, Shea; Goldstein, Jody; Cronan, Terry A; Corey-Bloom, Jody

    2013-01-01

    Huntington's disease (HD) is a genetic, neurodegenerative disorder characterized by motor, cognitive, and psychiatric dysfunction. In HD, the inability to solve problems successfully affects not only disease coping, but also interpersonal relationships, judgment, and independent living. The aim of the present study was to examine social problem-solving (SPS) in well-characterized HD and at-risk (AR) individuals and to examine its unique and conjoint effects with motor, cognitive, and psychiatric states on functional ratings. Sixty-three participants, 31 HD and 32 gene-positive AR, were included in the study. Participants completed the Social Problem-Solving Inventory-Revised: Long (SPSI-R:L), a 52-item, reliable, standardized measure of SPS. Items are aggregated under five scales (Positive, Negative, and Rational Problem-Solving; Impulsivity/Carelessness and Avoidance Styles). Participants also completed the Unified Huntington's Disease Rating Scale functional, behavioral, and cognitive assessments, as well as additional neuropsychological examinations and the Symptom Checklist-90-Revised (SCL-90R). A structural equation model was used to examine the effects of motor, cognitive, psychiatric, and SPS states on functionality. The multifactor structural model fit well descriptively. Cognitive and motor states uniquely and significantly predicted function in HD; however, neither psychiatric nor SPS states did. SPS was, however, significantly related to motor, cognitive, and psychiatric states, suggesting that it may bridge the correlative gap between psychiatric and cognitive states in HD. SPS may be worth assessing in conjunction with the standard gamut of clinical assessments in HD. Suggestions for future research and implications for patients, families, caregivers, and clinicians are discussed.

  4. Extended driving impairs nocturnal driving performances.

    Directory of Open Access Journals (Sweden)

    Patricia Sagaspe

    Full Text Available Though fatigue and sleepiness at the wheel are well-known risk factors for traffic accidents, many drivers combine extended driving and sleep deprivation. Fatigue-related accidents occur mainly at night but there is no experimental data available to determine if the duration of prior driving affects driving performance at night. Participants drove in 3 nocturnal driving sessions (3-5 am, 1-5 am and 9 pm-5 am on open highway. Fourteen young healthy men (mean age [+/-SD] = 23.4 [+/-1.7] years participated Inappropriate line crossings (ILC in the last hour of driving of each session, sleep variables, self-perceived fatigue and sleepiness were measured. Compared to the short (3-5 am driving session, the incidence rate ratio of inappropriate line crossings increased by 2.6 (95% CI, 1.1 to 6.0; P<.05 for the intermediate (1-5 am driving session and by 4.0 (CI, 1.7 to 9.4; P<.001 for the long (9 pm-5 am driving session. Compared to the reference session (9-10 pm, the incidence rate ratio of inappropriate line crossings were 6.0 (95% CI, 2.3 to 15.5; P<.001, 15.4 (CI, 4.6 to 51.5; P<.001 and 24.3 (CI, 7.4 to 79.5; P<.001, respectively, for the three different durations of driving. Self-rated fatigue and sleepiness scores were both positively correlated to driving impairment in the intermediate and long duration sessions (P<.05 and increased significantly during the nocturnal driving sessions compared to the reference session (P<.01. At night, extended driving impairs driving performances and therefore should be limited.

  5. Exit From the High Street

    DEFF Research Database (Denmark)

    Bly, Sarah; Gwozdz, Wencke; Reisch, Lucia

    2015-01-01

    by focusing on a previously under-researched group of consumers – ‘sustainable fashion consumption pioneers’ who actively engage and shape their own discourse around the notion of sustainable fashion consumption. These pioneers actively create and communicate strategies for sustainable fashion behaviour...... of sustainable fashion including such key behaviours as purchasing fewer garments of higher quality, exiting the retail market, purchasing only second-hand fashion goods and sewing or upgrading their own clothing. Central to much of these behaviours is the notion that personal style, rather than fashion, can...... bridge the potential disconnect between sustainability and fashion while also facilitating a sense of well-being not found in traditional fashion consumption. As such, our research suggests that for these consumers sustainability is as much about reducing measurable environmental or social impacts...

  6. Health-related quality of life and unmet healthcare needs in Huntington's disease.

    Science.gov (United States)

    van Walsem, Marleen R; Howe, Emilie I; Ruud, Gunvor A; Frich, Jan C; Andelic, Nada

    2017-01-07

    Huntington's disease (HD) is a rare neurodegenerative disorder with a prevalence of 6 per 100.000. Despite increasing research activity on HD, evidence on healthcare utilization, patients' needs for healthcare services and Health-Related Quality of Life (HRQoL) is still sparse. The present study describes HRQoL in a Norwegian cohort of HD patients, and assesses associations between unmet healthcare and social support service needs and HRQoL. In this cross-sectional population-based study, 84 patients with a clinical diagnosis of HD living in the South-East of Norway completed the HRQoL questionnaire EuroQol, EQ-5D-3L. Unmet needs for healthcare and social support services were assessed by the Needs and Provision Complexity Scale (NPCS). Furthermore, functional ability was determined using the Unified Huntington's Disease Rating Scale (UHDRS) Functional assessment scales. Socio-demographics (age, gender, marital status, occupation, residence, housing situation) and clinical characteristics (disease duration, total functional capacity, comorbidity) were also recorded. Descriptive statistics were used to describe the patients' HRQoL. Regression analyses were conducted in order to investigate the relationship between unmet healthcare needs and self-reported HRQoL. The patients were divided across five disease stages as follows: Stage I: n = 12 (14%), Stage II: n = 22 (27%), Stage III: n = 19 (23%), Stage IV: n = 14 (16%), and Stage V: n = 17 (20%). Overall HRQoL was lowest in patients with advanced disease (Stages IV and V), while patients in the middle phase (Stage III) showed the most varied health profile for the five EQ-5D-3L dimensions. The regression model including level of unmet needs, clinical characteristics and demographics (age and education) accounted for 42% of variance in HRQoL. A higher level of unmet needs was associated with lower HRQoL (β value - 0.228; p = 0.018) whereas a better total functional capacity corresponded to

  7. Performance of core exit thermocouple for PWR accident management action in vessel top break LOCA simulation experiment at OECD/NEA ROSA project

    International Nuclear Information System (INIS)

    Suzuki, Mitsuhiro; Takeda, Takeshi; Nakamura, Hideo

    2009-01-01

    Presented are experiment results of the Large Scale Test Facility (LSTF) conducted at the Japan Atomic Energy Agency (JAEA) with a focus on core exit thermocouple (CET) performance to detect core overheat during a vessel top break loss-of-coolant accident (LOCA) simulation experiment. The CET temperatures are used to start accident management (AM) action to quickly depressurize steam generator (SG) secondary side in case of core temperature excursion. Test 6-1 is first test of the OECD/NEA ROSA Project started in 2005, simulating withdraw of a control rod drive mechanism penetration nozzle at the vessel top head. The break size is equivalent to 1.9% cold leg break. The AM action was initiated when CET temperature rose up to 623K. There was no reflux water fallback onto the CETs during the core heat-up period. The core overheat, however, was detected with a time delay of about 230s. In addition, a large temperature discrepancy was observed between the CETs and the hottest core region. This paper clarifies the reason of time delay and temperature discrepancy between the CETs and heated core during boil-off including three-dimensional steam flows in the core and core exit. The paper discusses applicability of the LSTF CET performance to pressurized water reactor (PWR) conditions and a possibility of alternative indicators for earlier AM action than in Test 6-1 is studied by using symptom-based plant parameters such as a reactor vessel water level detection. (author)

  8. Film techniques in radiotherapy for treatment verification, determination of patient exit dose, and detection of localization error

    International Nuclear Information System (INIS)

    Haus, A.G.; Marks, J.E.

    1974-01-01

    In patient radiation therapy, it is important to know that the diseased area is included in the treatment field and that normal anatomy is properly shielded or excluded. Since 1969, a film technique developed for imaging of the complete patient radiation exposure has been applied for treatment verification and for the detection and evaluation of localization errors that may occur during treatment. The technique basically consists of placing a film under the patient during the entire radiation exposure. This film should have proper sensitivity and contrast in the exit dose exposure range encountered in radiotherapy. In this communication, we describe how various exit doses fit the characteristic curve of the film; examples of films exposed to various exit doses; the technique for using the film to determine the spatial distribution of the absorbed exit dose; and types of errors commonly detected. Results are presented illustrating that, as the frequency of use of this film technique is increased, localization error is reduced significantly

  9. Progressive Impairment of Lactate-based Gluconeogenesis in the Huntington's Disease Mouse Model R6/2.

    Science.gov (United States)

    Nielsen, Signe Marie Borch; Hasholt, Lis; Nørremølle, Anne; Josefsen, Knud

    2015-04-20

    Huntington's disease (HD) is a neurodegenerative illness, where selective neuronal loss in the brain caused by expression of mutant huntingtin protein leads to motor dysfunction and cognitive decline in addition to peripheral metabolic changes. In this study we confirm our previous observation of impairment of lactate-based hepatic gluconeogenesis in the transgenic HD mouse model R6/2 and determine that the defect manifests very early and progresses in severity with disease development, indicating a potential to explore this defect in a biomarker context. Moreover, R6/2 animals displayed lower blood glucose levels during prolonged fasting compared to wild type animals.

  10. Exit points, on plasma, of lost fast ions during NBI in TJ-II

    International Nuclear Information System (INIS)

    Guasp, J.

    1995-09-01

    The distribution of the exit points, on plasma border, for the lost fast ions during tangential balanced NBI in TJ-II helical axis Stellarator is theoretically analysed, as well for direct as for delayed losses. The link between the position of those exit points and the corresponding at birth, orbits and drifts is analysed also, it is shown that such relation is rather independent of beam energy and plasma density and is mainly related to the magnetic configuration characteristics. This study is a needed intermediate step to the analysis of impacts of those ions on the vacuum vessel of TJ-II

  11. Monetary Policy of the Federal Reserve System from the Perspective of Exit Strategies

    Directory of Open Access Journals (Sweden)

    Aleksandra Nocoń

    2015-07-01

    Full Text Available Central banks, after the experience of the global financial crisis, are now starting the implementation of the exit strategies, which are the process of normalization of monetary policy. The pace of monetary policy normalization process depends on the market reaction to central bank's decisions and macroeconomic conditions, in which they will be implemented. The main aim of the study is to present the principles of the exit strategy of the Federal Reserve System (Fed, on the background of the changes that have occurred in the United States within the monetary policy during the global financial crisis.

  12. Psychiatric and cognitive symptoms in Huntington's disease are modified by polymorphisms in catecholamine regulating enzyme genes

    DEFF Research Database (Denmark)

    Vinther-Jensen, T; Nielsen, Troels Tolstrup; Budtz-Jørgensen, E

    2016-01-01

    Huntington's disease (HD) is an autosomal dominantly inherited neurodegenerative disorder characterized by motor, psychiatric, and cognitive manifestations. HD is caused by a CAG repeat expansion in the Huntingtin (HTT) gene but the exact pathogenesis remains unknown. Dopamine imbalance has......-described cohort of Danish HD gene-expansion carriers. We show that cognitive impairment and psychiatric symptoms in HD are modified by polymorphisms in the monoamine oxidase A (MAOA) and catechol-O-methyltransferase (COMT) genes and by the 4p16.3 B haplotype. These results support the theory of dopamine imbalance...

  13. SEC16 in COPII coat dynamics at ER exit sites

    NARCIS (Netherlands)

    Sprangers, Joep; Rabouille, Catherine

    Protein export from the endoplasmic reticulum (ER), the first step in protein transport through the secretory pathway, is mediated by coatomer protein II (COPII)-coated vesicles at ER exit sites. COPII coat assembly on the ER is well understood and the conserved large hydrophilic protein Sec16

  14. Explicit solutions for exit-only radioactive decay chains

    International Nuclear Information System (INIS)

    Yuan, Ding; Kernan, Warnick

    2007-01-01

    In this study, we extended Bateman's [Proc. Cambridge Philos. Soc. 15, 423 (1910)] original work for solving radioactive decay chains and explicitly derived analytic solutions for generic exit-only radioactive decay problems under given initial conditions. Instead of using the conventional Laplace transform for solving Bateman's equations, we used a much simpler algebraic approach. Finally, we discuss methods of breaking down certain classes of large decay chains into collections of simpler chains for easy handling

  15. Nuclear energy - no thanks. The long way to the exit

    International Nuclear Information System (INIS)

    Sternstein, Wolfgang

    2013-01-01

    The anti-nuclear movement is one of the most successful social movements of the recent history of Germany and firmly anchored in huge parts of the population. After a struggle lasting more than forty years it has nearly reached its aims: The exit from nuclear energy and a rethinking in direction of alternative energy concepts. Decisive for the nuclear exit of the Federal government were thereby less the nuclear accidents of Harrisburg (1979), Chernobyl (1986), and Fukushima (2011) but rather the decades-long, stubborn commitment of numerous citizens as well as of citizen's initiatives. ''Nuclear energy - no thanks.'' is the analysis of the citizen's movement from Wyhl until Gorleben. The nonviolent activist and peace researcher Sternstein was there from the beginning and reports about anger and indignation that has been condensed to the social movement with political objectives. Extremely helpful are thereby the methods and strategies of nonviolent actions described by him.

  16. Association Between Motor Symptoms and Brain Metabolism in Early Huntington Disease.

    Science.gov (United States)

    Gaura, Véronique; Lavisse, Sonia; Payoux, Pierre; Goldman, Serge; Verny, Christophe; Krystkowiak, Pierre; Damier, Philippe; Supiot, Frédéric; Bachoud-Levi, Anne-Catherine; Remy, Philippe

    2017-09-01

    Brain hypometabolism is associated with the clinical consequences of the degenerative process, but little is known about regional hypermetabolism, sometimes observed in the brain of patients with clinically manifest Huntington disease (HD). Studying the role of regional hypermetabolism is needed to better understand its interaction with the motor symptoms of the disease. To investigate the association between brain hypometabolism and hypermetabolism with motor scores of patients with early HD. This study started in 2001, and analysis was completed in 2016. Sixty symptomatic patients with HD and 15 healthy age-matched control individuals underwent positron emission tomography to measure cerebral metabolism in this cross-sectional study. They also underwent the Unified Huntington's Disease Rating Scale motor test, and 2 subscores were extracted: (1) a hyperkinetic score, combining dystonia and chorea, and (2) a hypokinetic score, combining bradykinesia and rigidity. Statistical parametric mapping software (SPM5) was used to identify all hypo- and hypermetabolic regions in patients with HD relative to control individuals. Correlation analyses (P motor subscores and brain metabolic values were performed for regions with significant hypometabolism and hypermetabolism. Among 60 patients with HD, 22 were women (36.7%), and the mean (SD) age was 44.6 (7.6) years. Of the 15 control individuals, 7 were women (46.7%), and the mean (SD) age was 42.2 (7.3) years. In statistical parametric mapping, striatal hypometabolism was significantly correlated with the severity of all motor scores. Hypermetabolism was negatively correlated only with hypokinetic scores in the cuneus (z score = 3.95, P motor scores were associated with higher metabolic values in the inferior parietal lobule, anterior cingulate, inferior temporal lobule, the dentate nucleus, and the cerebellar lobules IV/V, VI, and VIII bilaterally corresponding to the motor regions of the cerebellum (z score = 3

  17. Striatal hypometabolism in premanifest and manifest Huntington's disease patients

    Energy Technology Data Exchange (ETDEWEB)

    Lopez-Mora, Diego Alfonso; Camacho, Valle; Fernandez, Alejandro; Montes, Alberto; Carrio, Ignasi [Autonomous University of Barcelona, Nuclear Medicine Department, Hospital Sant Pau, Barcelona (Spain); Perez-Perez, Jesus; Martinez-Horta, Sauel; Kulisevsky, Jaime [Autonomous University of Barcelona, Movement Disorders Unit, Neurology Department, Hospital Sant Pau, Barcelona (Spain); Sampedro, Frederic [University of Barcelona, Barcelona (Spain); Lozano-Martinez, Gloria Andrea; Gomez-Anson, Beatriz [Autonomous University of Barcelona, Neuroradiology, Radiology Department, Hospital Sant Pau, Barcelona (Spain)

    2016-11-15

    To assess metabolic changes in cerebral {sup 18}F-FDG PET/CT in premanifest and manifest Huntington's disease (HD) subjects compared to a control group and to correlate {sup 18}F-FDG uptake patterns with different disease stages. Thirty-three gene-expanded carriers (Eight males; mean age: 43 y/o; CAG > 39) were prospectively included. Based on the Unified Huntington's Disease Rating Scale Total Motor Score and the Total Functional Capacity, subjects were classified as premanifest (preHD = 15) and manifest (mHD = 18). Estimated time disease-onset was calculated using the Langbehn formula, which allowed classifying preHD as far-to (preHD-A) and close-to (PreHD-B) disease-onset. Eighteen properly matched participants were included as a control group (CG). All subjects underwent brain {sup 18}F-FDG PET/CT and MRI. {sup 18}F-FDG PET/CT were initially assessed by two nuclear medicine physicians identifying qualitative metabolic changes in the striatum. Quantitative analysis was performed using SPM8 with gray matter atrophy correction using the BPM toolbox. Visual analysis showed a marked striatal hypometabolism in mHD. A normal striatal distribution of {sup 18}F-FDG uptake was observed for most of the preHD subjects. Quantitative analysis showed a significant striatal hypometabolism in mHD subjects compared to CG (p < 0.001 uncorrected, k = 50 voxels). In both preHD groups we observed a significant striatal hypometabolism with respect to CG (p < 0.001 uncorrected, k = 50 voxels). In mHD subjects we observed a significant striatal hypometabolism with respect to both preHD groups (p < 0.001 uncorrected, k = 50 voxels). {sup 18}F-FDG PET/CT might be a helpful tool to identify patterns of glucose metabolism in the striatum across the stages of HD and might be relevant in assessing the clinical status of gene-expanded HD carriers due to the fact that dysfunctional glucose metabolism begins at early preHD stages of the disease. {sup 18}F-FDG PET/CT appears as a

  18. 10 CFR 431.204 - Uniform test method for the measurement of energy consumption of illuminated exit signs.

    Science.gov (United States)

    2010-01-01

    ... 10 Energy 3 2010-01-01 2010-01-01 false Uniform test method for the measurement of energy consumption of illuminated exit signs. 431.204 Section 431.204 Energy DEPARTMENT OF ENERGY ENERGY CONSERVATION... Procedures § 431.204 Uniform test method for the measurement of energy consumption of illuminated exit signs...

  19. Ramp Metering Influence on Freeway Operational Safety near On-ramp Exits

    Directory of Open Access Journals (Sweden)

    Chiu Liu, PhD, PE, PTOE

    2013-06-01

    Full Text Available Ramp metering has been widely installed in urban areas where congestion on a freeway or an expressway may occur recurrently during weekday peak periods to enhance mainline throughput and reduce system-wide delay. These operational benefits may also help reduce vehicular emissions and improve air quality in urban areas. However, the impact on traffic safety due to ramp metering hasn't been explored in details before. Supported by physical understanding and arguments, we characterize the ramp metering influence on freeway safety by examining vehicular collisions near on-ramp exits within the ramp meter operating hours before and after the activation of the ramp metering. Collisions for a sample of 19 operating ramp meters along several freeways in northern California were collected and organized to show that ramp metering can help reduce freeway collisions at the vicinity of on-ramp exits. It was found that the average reductions on freeway collisions in the vicinity of an on-ramp exit are around 36%. Although most of the reduced collisions belong to the property damage only category, a 36% reduction shows the significant safety benefit of ramp metering. The traffic congestion induced by each collision, especially during peak hours when ramp metering is in operation, could last for an hour or two. Consequently, ramp metering must be contributing to the reduction of non-recurrent congestion in addition to mitigating recurrent congestion, which is better documented. This study strongly supports the implementation of ramp metering in California.

  20. Modeling Lane-Changing Behavior in Freeway Off-Ramp Areas from the Shanghai Naturalistic Driving Study

    Directory of Open Access Journals (Sweden)

    Lanfang Zhang

    2018-01-01

    Full Text Available The objective of this study is to investigate lane-changing characteristics in freeway off-ramp areas using Shanghai Naturalistic Driving Study (SH-NDS data, considering a four-lane freeway stretch in various traffic conditions. In SH-NDS, the behavior of drivers is observed unobtrusively in a natural setting for a long period of time. We identified 433 lane-changing events with valid time series data from the whole dataset. Based on the logit model developed to analyze the choice of target lanes, a likelihood analysis of lane-changing behavior was graphed with respect to three traffic conditions: free flow, medium flow, and heavy flow. The results suggested that lane-changing behavior of exiting vehicles is the consequence of the balance between route plan (mandatory incentive and expectation to improve driving condition (discretionary incentive. In higher traffic density, the latter seems to play a significant role. Furthermore, we found that lane-change from the slow lane to the fast lane would lead to higher speed variance value, which indicates a higher crash risk. The findings contribute to a better understanding on drivers’ natural driving behavior in freeway off-ramp areas and can provide important insight into road network design and safety management strategies.

  1. TomoTherapy MLC verification using exit detector data

    Energy Technology Data Exchange (ETDEWEB)

    Chen Quan; Westerly, David; Fang Zhenyu; Sheng, Ke; Chen Yu [TomoTherapy Inc., 1240 Deming Way, Madison, Wisconsin 53717 (United States); Department of Radiation Oncology, University of Colorado School of Medicine, Aurora, Colorado 80045 (United States); Xinghua Cancer Hospital, Xinghua, Jiangsu 225700 (China); Department of Radiation Oncology, University of California-Los Angeles, Los Angeles, California 90095 (United States); TomoTherapy Inc., 1240 Deming Way, Madison, Wisconsin 53717 (United States)

    2012-01-15

    Purpose: Treatment delivery verification (DV) is important in the field of intensity modulated radiation therapy (IMRT). While IMRT and image guided radiation therapy (IGRT), allow us to create more conformal plans and enables the use of tighter margins, an erroneously executed plan can have detrimental effects on the treatment outcome. The purpose of this study is to develop a DV technique to verify TomoTherapy's multileaf collimator (MLC) using the onboard mega-voltage CT detectors. Methods: The proposed DV method uses temporal changes in the MVCT detector signal to predict actual leaf open times delivered on the treatment machine. Penumbra and scattered radiation effects may produce confounding results when determining leaf open times from the raw detector data. To reduce the impact of the effects, an iterative, Richardson-Lucy (R-L) deconvolution algorithm is applied. Optical sensors installed on each MLC leaf are used to verify the accuracy of the DV technique. The robustness of the DV technique is examined by introducing different attenuation materials in the beam. Additionally, the DV technique has been used to investigate several clinical plans which failed to pass delivery quality assurance (DQA) and was successful in identifying MLC timing discrepancies as the root cause. Results: The leaf open time extracted from the exit detector showed good agreement with the optical sensors under a variety of conditions. Detector-measured leaf open times agreed with optical sensor data to within 0.2 ms, and 99% of the results agreed within 8.5 ms. These results changed little when attenuation was added in the beam. For the clinical plans failing DQA, the dose calculated from reconstructed leaf open times played an instrumental role in discovering the root-cause of the problem. Throughout the retrospective study, it is found that the reconstructed dose always agrees with measured doses to within 1%. Conclusions: The exit detectors in the TomoTherapy treatment

  2. TomoTherapy MLC verification using exit detector data

    International Nuclear Information System (INIS)

    Chen Quan; Westerly, David; Fang Zhenyu; Sheng, Ke; Chen Yu

    2012-01-01

    Purpose: Treatment delivery verification (DV) is important in the field of intensity modulated radiation therapy (IMRT). While IMRT and image guided radiation therapy (IGRT), allow us to create more conformal plans and enables the use of tighter margins, an erroneously executed plan can have detrimental effects on the treatment outcome. The purpose of this study is to develop a DV technique to verify TomoTherapy's multileaf collimator (MLC) using the onboard mega-voltage CT detectors. Methods: The proposed DV method uses temporal changes in the MVCT detector signal to predict actual leaf open times delivered on the treatment machine. Penumbra and scattered radiation effects may produce confounding results when determining leaf open times from the raw detector data. To reduce the impact of the effects, an iterative, Richardson-Lucy (R-L) deconvolution algorithm is applied. Optical sensors installed on each MLC leaf are used to verify the accuracy of the DV technique. The robustness of the DV technique is examined by introducing different attenuation materials in the beam. Additionally, the DV technique has been used to investigate several clinical plans which failed to pass delivery quality assurance (DQA) and was successful in identifying MLC timing discrepancies as the root cause. Results: The leaf open time extracted from the exit detector showed good agreement with the optical sensors under a variety of conditions. Detector-measured leaf open times agreed with optical sensor data to within 0.2 ms, and 99% of the results agreed within 8.5 ms. These results changed little when attenuation was added in the beam. For the clinical plans failing DQA, the dose calculated from reconstructed leaf open times played an instrumental role in discovering the root-cause of the problem. Throughout the retrospective study, it is found that the reconstructed dose always agrees with measured doses to within 1%. Conclusions: The exit detectors in the TomoTherapy treatment systems

  3. A study on the impulse wave discharged from the exit of a right-angle pipe bend

    International Nuclear Information System (INIS)

    Lee, D. H.; Hur, S. C.; Kweon, Y. H.; Kim, H. D.

    2001-01-01

    The current study addresses experimental and computational work of impulse wave discharged from the exit of two kinds of right-angle pipe bends, which are attached to the open end of a simple shock tube. The weak normal shock wave with its magnitude of Mach number from 1.02 to 1.20 is employed to obtain the impulse wave propagating outside the exit of the pipe bends. A Schlieren optical system visualizes the impulse wave discharged from the exit of the pipe bends at an instant. The experimental data of the magnitude of the impulse wave and its propagating directivity are analyzed to characterize the impulse waves discharged from the exit of the pipe bends and compared with those discharged from a straight pipe. Computational results well predict the experimented dynamic behaviors of the impulse wave. The results obtained show that a right-angle miter bend considerably reduces the magnitude of the impulse wave and its directivity toward to the pipe axis, compared with the straight pipe and right-angle smooth bend. It is believed that the right-angle miter bend pipe can play a role of a passive control against the impulse wave

  4. An Eclectic Approach to Loyalty-Promoting Instruments in Corporate Law: Revisiting Hirschman's Model of Exit, Voice, and Loyalty

    NARCIS (Netherlands)

    A.A. Bootsma (Bart)

    2013-01-01

    markdownabstract__Abstract__ This essay analyses the shareholder role in corporate governance in terms of Albert Hirschman's Exit, Voice, and Loyalty. The term 'exit' is embedded in a law & economics framework, while 'voice' relates to a corporate constitutional framework. The essay takes an

  5. Neuropsychological correlates of brain atrophy in Huntington's disease: a magnetic resonance imaging study

    International Nuclear Information System (INIS)

    Starkstein, S.E.; Brandt, J.; Bylsma, F.; Peyser, C.; Folstein, M.; Folstein, S.E.

    1992-01-01

    Magnetic resonance imaging and a comprehensive cognitive evaluation were carried out in a series of 29 patients with mild to moderate Huntington's disease (HD). A factor analysis of the neuropsychological test scores provided three factors: A memory/speed-of-processing factor, a 'frontal' factor, and a response inhibition factor. The memory/speed factor correlated significantly with measures of caudate atrophy, frontal atrophy, and atrophy of the left (but not the right) sylvian cistern. There were no significant correlations between the 'frontal' or response inhibition factors and measures of cortical or subcortical brain atrophy. Our findings confirm that subcortical atrophy is significantly correlated with specific cognitive deficits in HD, and demonstrate that cortical atrophy also has important association with the cognitive deficits of patients with HD. (orig.)

  6. Social Cognition, Executive Functions and Self-Report of Psychological Distress in Huntington's Disease

    DEFF Research Database (Denmark)

    Larsen, Ida Unmack; Vinther-Jensen, Tua; Nielsen, Jørgen Erik

    2016-01-01

    OBJECTIVE: Huntington's disease (HD) is characterized by motor symptoms, psychiatric symptoms and cognitive impairment in, inter alia, executive functions and social cognition. The aim of this study was to investigate the relationship between subjective feeling of psychological distress using...... a self-report questionnaire and performances on tests of executive functions and social cognition in a large consecutive cohort of HD patients. METHOD: 50 manifest HD patients were tested in social cognition and executive functions and each answered a self-report questionnaire about current status...... psychological distress was significantly associated with worse performances on social cognitive tests (mean absolute correlation .34) and that there were no significant correlations between perceived psychological distress and performance on tests of executive functions. The correlations between perceived...

  7. Medical management of motor manifestations of Huntington disease.

    Science.gov (United States)

    McCusker, Elizabeth A; Loy, Clement T

    2017-01-01

    The motor and movement disorders of Huntington disease (HD) are managed in the context of the other disease features. Chorea and dystonia are the most common HD-associated movement disorders, and they can be assessed on research rating scales. However other motor manifestations have a significant impact. In particular, dysphagia influences choice and tolerance of treatment for the movement disorder, as will comorbidities, patient awareness, and distress related to the motor feature or movement. Treatment for other disease features may aggravate the motor disorder, e.g., increased swallowing difficulty associated with antipsychotic agents. Basic principles in deciding to institute a treatment are outlined as well as treatment of specific motor manifestations and movements. There is a paucity of evidence to support the treatments available for the motor disorder, with only one agent with class 1 evidence, tetrabenazine, for chorea. There are, however, treatments informed by expert opinion which reflect the management of a wider HD phenotype than that represented in clinical trials. Some treatments are based on evidence from use in other conditions. Medical management is usually undertaken later in the disease with concurrent nonmedical interventions after multidisciplinary assessments. Medication review with HD progression is essential. Copyright © 2017 Elsevier B.V. All rights reserved.

  8. Exit points, on plasma, of lost fast ions during NBI in TJ-II

    International Nuclear Information System (INIS)

    Guasp, J.

    1995-01-01

    The distribution of the exit points, on plasma border, for the lost fast ions during tangential balanced NBI in TJ-II helical axis Stellarator is theoretically analysed, as well for direct as for delayed losses. The link between, the position of those exit points and the corresponding at birth, orbits and drifts is analysed also. It is shown that such relation is rather independent of beam energy and plasma density and is mainly related to the magnetic configuration characteristics. This study is a needed intermediate step to the analysis of impacts of those ions on the vacuum vessel of TJ-II. (Author) 2 refs

  9. A Study on the Design of PM Exited Transverse Flux Linear Motor for Ropeless Elevator

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Do Hyun; Bang, Deok Je; Kim, Jong Moo; Jeong, Yeon Ho [Korea Electrotechnology Research Institute (Korea); Kim, Moon Hwan [Silla University (Korea)

    2000-03-01

    The topological investigations regarding magnetic circuit geometry and winding form of the transverse flux machine have brought up a variety of constructable arrangements with different features for several types of application[1, 2]. Here with, a novel PM-exited linear motor with inner mover, based on the transverse flux configuration leads to a considerable increase in power density for moving part. In this study we designed PM-exited transverse flux linear motor for ropeless elevator, whose output power density is higher and weight is lighter than conventional linear synchronous motors, When the designed motor in this study is applied to ropeless elevator, it is possible to increase power density more than 400% comparing with PM exited linear synchronous motor. The result of this study can be utilized for ropeless elevator or gearless direct linear moving system with high output[3]. (author). 8 refs., 9 figs., 4 tabs.

  10. Educational Inequalities in Exit from Paid Employment among Dutch Workers: The Influence of Health, Lifestyle and Work.

    Science.gov (United States)

    Robroek, Suzan J W; Rongen, Anne; Arts, Coos H; Otten, Ferdy W H; Burdorf, Alex; Schuring, Merel

    2015-01-01

    Individuals with lower socioeconomic status are at increased risk of involuntary exit from paid employment. To give sound advice for primary prevention in the workforce, insight is needed into the role of mediating factors between socioeconomic status and labour force participation. Therefore, it is aimed to investigate the influence of health status, lifestyle-related factors and work characteristics on educational differences in exit from paid employment. 14,708 Dutch employees participated in a ten-year follow-up study during 1999-2008. At baseline, education, self-perceived health, lifestyle (smoking, alcohol, sports, BMI) and psychosocial (demands, control, rewards) and physical work characteristics were measured by questionnaire. Employment status was ascertained monthly based on tax records. The relation between education, health, lifestyle, work-characteristics and exit from paid employment through disability benefits, unemployment, early retirement and economic inactivity was investigated by competing risks regression analyses. The mediating effects of these factors on educational differences in exit from paid employment were tested using a stepwise approach. Lower educated workers were more likely to exit paid employment through disability benefits (SHR:1.84), unemployment (SHR:1.74), and economic inactivity (SHR:1.53) but not due to early retirement (SHR:0.92). Poor or moderate health, an unhealthy lifestyle, and unfavourable work characteristics were associated with disability benefits and unemployment, and an unhealthy lifestyle with economic inactivity. Educational differences in disability benefits were explained for 40% by health, 31% by lifestyle, and 12% by work characteristics. For economic inactivity and unemployment, up to 14% and 21% of the educational differences could be explained, particularly by lifestyle-related factors. There are educational differences in exit from paid employment, which are partly mediated by health, lifestyle and work

  11. Construction of an easy-to-use CRISPR-Cas9 system by patching a newly designed EXIT circuit.

    Science.gov (United States)

    Tang, Qiang; Lou, Chunbo; Liu, Shuang-Jiang

    2017-01-01

    Plasmid-borne genetic editing tools, including the widely used CRISPR-Cas9 system, have greatly facilitated bacterial programming to obtain novel functionalities. However, the lack of effective post-editing plasmid elimination methods impedes follow-up genetic manipulation or application. Conventional strategies including exposure to physical and chemical treatments, or exploiting temperature-sensitive replication origins have several drawbacks (e.g., they are limited for efficiency and are time-consuming). Therefore, the demand is apparent for easy and rapid elimination of the tool plasmids from their bacterial hosts after genetic manipulation. To bridge this gap, we designed a novel EXIT circuit with the homing endonuclease, which can be exploited for rapid and efficient elimination of various plasmids with diverse replication origins. As a proof of concept, we validated the EXIT circuit in Escherichia coli by harnessing homing endonuclease I- Sce I and its cleavage site. When integrated into multiple plasmids with different origins, the EXIT circuit allowed them to be eliminated from the host cells, simultaneously. By combining the widely used plasmid-borne CRISPR-Cas9 system and the EXIT circuit, we constructed an easy-to-use CRISPR-Cas9 system that eliminated the Cas9- and the single-guide RNA (sgRNA)-encoding plasmids in one-step. Within 3 days, we successfully constructed an atrazine-degrading E. coli strain, thus further demonstrating the advantage of this new CRISPR-Cas9 system for bacterial genome editing. Our novel EXIT circuit, which exploits the homing endonuclease I- Sce I, enables plasmid(s) with different replication origins to be eliminated from their host cells rapidly and efficiently. We also developed an easy-to-use CRISPR-Cas9 system with the EXIT circuit, and this new system can be widely applied to bacterial genome editing.

  12. R6/2 Huntington's disease mice develop early and progressive abnormal brain metabolism and seizures.

    Science.gov (United States)

    Cepeda-Prado, Efrain; Popp, Susanna; Khan, Usman; Stefanov, Dimitre; Rodríguez, Jorge; Menalled, Liliana B; Dow-Edwards, Diana; Small, Scott A; Moreno, Herman

    2012-05-09

    A hallmark feature of Huntington's disease pathology is the atrophy of brain regions including, but not limited to, the striatum. Though MRI studies have identified structural CNS changes in several Huntington's disease (HD) mouse models, the functional consequences of HD pathology during the progression of the disease have yet to be investigated using in vivo functional MRI (fMRI). To address this issue, we first established the structural and functional MRI phenotype of juvenile HD mouse model R6/2 at early and advanced stages of disease. Significantly higher fMRI signals [relative cerebral blood volumes (rCBVs)] and atrophy were observed in both age groups in specific brain regions. Next, fMRI results were correlated with electrophysiological analysis, which showed abnormal increases in neuronal activity in affected brain regions, thus identifying a mechanism accounting for the abnormal fMRI findings. [(14)C] 2-deoxyglucose maps to investigate patterns of glucose utilization were also generated. An interesting mismatch between increases in rCBV and decreases in glucose uptake was observed. Finally, we evaluated the sensitivity of this mouse line to audiogenic seizures early in the disease course. We found that R6/2 mice had an increased susceptibility to develop seizures. Together, these findings identified seizure activity in R6/2 mice and show that neuroimaging measures sensitive to oxygen metabolism can be used as in vivo biomarkers, preceding the onset of an overt behavioral phenotype. Since fMRI-rCBV can also be obtained in patients, we propose that it may serve as a translational tool to evaluate therapeutic responses in humans and HD mouse models.

  13. Embracing the Exit: Assessment, Trust, and the Teaching of Writing

    Science.gov (United States)

    Eng, Joseph

    2006-01-01

    Historically, the Composition Program at Eastern Washington University (EWU), a comprehensive university in Cheney, WA, required a single essay sample from each composition student as the final exit exam; in practice, a student passed or failed the course based on an in-class argumentative essay, written in three consecutive class periods. Such a…

  14. Calculation of midplane dose for total body irradiation from entrance and exit dose MOSFET measurements.

    Science.gov (United States)

    Satory, P R

    2012-03-01

    This work is the development of a MOSFET based surface in vivo dosimetry system for total body irradiation patients treated with bilateral extended SSD beams using PMMA missing tissue compensators adjacent to the patient. An empirical formula to calculate midplane dose from MOSFET measured entrance and exit doses has been derived. The dependency of surface dose on the air-gap between the spoiler and the surface was investigated by suspending a spoiler above a water phantom, and taking percentage depth dose measurements (PDD). Exit and entrances doses were measured with MOSFETs in conjunction with midplane doses measured with an ion chamber. The entrance and exit doses were combined using an exponential attenuation formula to give an estimate of midplane dose and were compared to the midplane ion chamber measurement for a range of phantom thicknesses. Having a maximum PDD at the surface simplifies the prediction of midplane dose, which is achieved by ensuring that the air gap between the compensator and the surface is less than 10 cm. The comparison of estimated midplane dose and measured midplane dose showed no dependence on phantom thickness and an average correction factor of 0.88 was found. If the missing tissue compensators are kept within 10 cm of the patient then MOSFET measurements of entrance and exit dose can predict the midplane dose for the patient.

  15. An optimization method for the distance between exits of buildings considering uncertainties based on arbitrary polynomial chaos expansion

    NARCIS (Netherlands)

    Xie, Qimiao; Wang, Jinhui; Lu, Shouxiang; Hensen, J.L.M.

    2016-01-01

    The distance between exits is an important design parameter in fire safety design of buildings. In order to find the optimal distance between exits under uncertainties with a low computational cost, the surrogate model (i.e. approximation model) of evacuation time is constructed by the arbitrary

  16. Glaucoma and Driving: On-Road Driving Characteristics

    Science.gov (United States)

    Wood, Joanne M.; Black, Alex A.; Mallon, Kerry; Thomas, Ravi; Owsley, Cynthia

    2016-01-01

    Purpose To comprehensively investigate the types of driving errors and locations that are most problematic for older drivers with glaucoma compared to those without glaucoma using a standardized on-road assessment. Methods Participants included 75 drivers with glaucoma (mean = 73.2±6.0 years) with mild to moderate field loss (better-eye MD = -1.21 dB; worse-eye MD = -7.75 dB) and 70 age-matched controls without glaucoma (mean = 72.6 ± 5.0 years). On-road driving performance was assessed in a dual-brake vehicle by an occupational therapist using a standardized scoring system which assessed the types of driving errors and the locations where they were made and the number of critical errors that required an instructor intervention. Driving safety was rated on a 10-point scale. Self-reported driving ability and difficulties were recorded using the Driving Habits Questionnaire. Results Drivers with glaucoma were rated as significantly less safe, made more driving errors, and had almost double the rate of critical errors than those without glaucoma. Driving errors involved lane positioning and planning/approach, and were significantly more likely to occur at traffic lights and yield/give-way intersections. There were few between group differences in self-reported driving ability. Conclusions Older drivers with glaucoma with even mild to moderate field loss exhibit impairments in driving ability, particularly during complex driving situations that involve tactical problems with lane-position, planning ahead and observation. These results, together with the fact that these drivers self-report their driving to be relatively good, reinforce the need for evidence-based on-road assessments for evaluating driving fitness. PMID:27472221

  17. Glaucoma and Driving: On-Road Driving Characteristics.

    Directory of Open Access Journals (Sweden)

    Joanne M Wood

    Full Text Available To comprehensively investigate the types of driving errors and locations that are most problematic for older drivers with glaucoma compared to those without glaucoma using a standardized on-road assessment.Participants included 75 drivers with glaucoma (mean = 73.2±6.0 years with mild to moderate field loss (better-eye MD = -1.21 dB; worse-eye MD = -7.75 dB and 70 age-matched controls without glaucoma (mean = 72.6 ± 5.0 years. On-road driving performance was assessed in a dual-brake vehicle by an occupational therapist using a standardized scoring system which assessed the types of driving errors and the locations where they were made and the number of critical errors that required an instructor intervention. Driving safety was rated on a 10-point scale. Self-reported driving ability and difficulties were recorded using the Driving Habits Questionnaire.Drivers with glaucoma were rated as significantly less safe, made more driving errors, and had almost double the rate of critical errors than those without glaucoma. Driving errors involved lane positioning and planning/approach, and were significantly more likely to occur at traffic lights and yield/give-way intersections. There were few between group differences in self-reported driving ability.Older drivers with glaucoma with even mild to moderate field loss exhibit impairments in driving ability, particularly during complex driving situations that involve tactical problems with lane-position, planning ahead and observation. These results, together with the fact that these drivers self-report their driving to be relatively good, reinforce the need for evidence-based on-road assessments for evaluating driving fitness.

  18. Glaucoma and Driving: On-Road Driving Characteristics.

    Science.gov (United States)

    Wood, Joanne M; Black, Alex A; Mallon, Kerry; Thomas, Ravi; Owsley, Cynthia

    2016-01-01

    To comprehensively investigate the types of driving errors and locations that are most problematic for older drivers with glaucoma compared to those without glaucoma using a standardized on-road assessment. Participants included 75 drivers with glaucoma (mean = 73.2±6.0 years) with mild to moderate field loss (better-eye MD = -1.21 dB; worse-eye MD = -7.75 dB) and 70 age-matched controls without glaucoma (mean = 72.6 ± 5.0 years). On-road driving performance was assessed in a dual-brake vehicle by an occupational therapist using a standardized scoring system which assessed the types of driving errors and the locations where they were made and the number of critical errors that required an instructor intervention. Driving safety was rated on a 10-point scale. Self-reported driving ability and difficulties were recorded using the Driving Habits Questionnaire. Drivers with glaucoma were rated as significantly less safe, made more driving errors, and had almost double the rate of critical errors than those without glaucoma. Driving errors involved lane positioning and planning/approach, and were significantly more likely to occur at traffic lights and yield/give-way intersections. There were few between group differences in self-reported driving ability. Older drivers with glaucoma with even mild to moderate field loss exhibit impairments in driving ability, particularly during complex driving situations that involve tactical problems with lane-position, planning ahead and observation. These results, together with the fact that these drivers self-report their driving to be relatively good, reinforce the need for evidence-based on-road assessments for evaluating driving fitness.

  19. An entry and exit model on the energy-saving investment strategy with real options

    International Nuclear Information System (INIS)

    Lin, Tyrone T.; Huang, S.-L.

    2010-01-01

    This paper presents an improved decision model based on the real options approach presented by for the firms that have not yet established energy-saving equipment under the entry and exit strategies. Furthermore, the proposed model takes account of the inevitable equipment renewal and the occurrence of unexpected events under the Poisson jump process. The timing for terminating an investment when continuous operations of that business are unprofitable is also explored to realize the optimal timing of implementing the energy-saving strategy. The future discounted benefit B follows the geometric Brownian motion with the Poisson jump process and the replacement of investment equipment. A numerical analysis is followed by a sensitivity study of various parameters to better realize their impacts on the entry and exit thresholds. The results show that for the jump case, the higher probability of occurrence of unfavorable events will result in a higher entry threshold and lower exit threshold. Investors are forced to request higher benefit thresholds to cover the higher probability of losses brought by unfavorable events.

  20. Which consequences for the USA of its exit from the Paris agreement? An economic, commercial and geopolitical view

    International Nuclear Information System (INIS)

    Hache, Emmanuel; Bourcet, Clemence; Palle, Angelique

    2017-06-01

    The authors discuss economic impacts which its exit from the Paris agreement may have on the US at the medium and long terms, and also the possible geopolitical reconfigurations which may result from this exit at the international level. Possible economic impacts are discussed in terms of loss of competitiveness, of image and of leadership in the international innovation dynamics. The authors also notice and comment that this exit process has already started in January 2017 with the exit form the Transpacific Partnership Treaty. They question the possible emergence of new leaders for international climatic issues, and discuss the consequences of this loss of political and moral influence of the US, and of China be this leader. The authors comment the domestic situation for the US in terms of political debate between Democrats and Republicans, but also by considering the fact that the environment policy is a power issue between local and federal authorities in the US

  1. Formation of vortex pairs with hinged rigid flaps at the nozzle exit

    Science.gov (United States)

    Das, Prashant; Govardhan, Raghuraman; Arakeri, Jaywant

    2013-11-01

    Biological flows related to aquatic propulsion using pulsed jets, or flow through the valves in a human heart, have received considerable attention in the last two decades. Both these flows are associated with starting jets that occur through biological tissue/membranes that are flexible. Motivated by these flows, we explore in the present work, the effect of passive flexibility of the nozzle exit on vortex generation from a starting jet. The starting jet is generated using a two-dimensional piston cylinder mechanism, the cross-section of the cylinder being rectangular with large aspect ratio. The fluid is pushed out of this cylinder or channel using a computer controlled piston. We introduce flexibility at the channel exit by hinging rigid flaps, which are initially parallel to the channel. The hinge used is such that it provides negligible stiffness or damping, thus allowing for the maximum opening of the flaps due to fluid forces. Using this system, we study both the flap kinematics and the vorticity dynamics downstream of the channel exit. Visualizations show large flap motions as the piston starts and this dramatically changes the vorticity distribution downstream of the flaps, with the formation of up to three different kinds of vortex pairs. This idealized configuration opens new opportunities to look at the effect of flexibility in such biological flows.

  2. Oxidative Stress and Huntington's Disease: The Good, The Bad, and The Ugly.

    Science.gov (United States)

    Kumar, Amit; Ratan, Rajiv R

    2016-10-01

    Redox homeostasis is crucial for proper cellular functions, including receptor tyrosine kinase signaling, protein folding, and xenobiotic detoxification. Under basal conditions, there is a balance between oxidants and antioxidants. This balance facilitates the ability of oxidants, such as reactive oxygen species, to play critical regulatory functions through a direct modification of a small number of amino acids (e.g. cysteine) on signaling proteins. These signaling functions leverage tight spatial, amplitude, and temporal control of oxidant concentrations. However, when oxidants overwhelm the antioxidant capacity, they lead to a harmful condition of oxidative stress. Oxidative stress has long been held to be one of the key players in disease progression for Huntington's disease (HD). In this review, we will critically review this evidence, drawing some intermediate conclusions, and ultimately provide a framework for thinking about the role of oxidative stress in the pathophysiology of HD.

  3. Cognitive and behavioral changes in Huntington disease before diagnosis.

    Science.gov (United States)

    Paulsen, Jane S; Miller, Amanda C; Hayes, Terry; Shaw, Emily

    2017-01-01

    Phenotypic manifestations of Huntington disease (HD) can be detected at least 15 years prior to the time when a motor diagnosis is given. Advances in clinical care and future research will require consistent use of HD definitions and HD premanifest (prodromal) stages being used across clinics, sites, and countries. Cognitive and behavioral (psychiatric) changes in HD are summarized and implications for ongoing advancement in our knowledge of prodromal HD are suggested. The earliest detected cognitive changes are observed in the Symbol Digit Modalities Test, Stroop Interference, Stroop Color and Word Test-interference condition, and Trail Making Test. Cognitive changes in the middle and near motor diagnostic stages of prodromal HD involve nearly every cognitive test administered and the greatest changes over time (i.e., slopes) are found in those prodromal HD participants who are nearest to motor diagnosis. Psychiatric changes demonstrate significant worsening over time and remain elevated compared with healthy controls throughout the prodromal disease course. Psychiatric and behavior changes in prodromal HD are much lower than that obtained using cognitive assessment, although the psychiatric and behavioral changes represent symptoms most debilitating to independent capacity and wellness. Copyright © 2017 Elsevier B.V. All rights reserved.

  4. Exit Options in Corporate Finance: Liquidity versus Incentives

    OpenAIRE

    Aghion, Philippe; Bolton, P.; Tirole, J.

    2004-01-01

    This paper provides a first study of the optimal design of active monitors' exit options in a problem involving a demand for liquidity and costly monitoring of the issuer. Optimal incentives to monitor the issuer may involve restricting the monitor's right to sell her claims on the firm's cash-flow early. But the monitor will then require a liquidity premium for holding such an illiquid claim. In general, therefore, there will be a trade off between incentives and liquidity. The paper highlig...

  5. Water Entry and Exit of Horizontal Cylinder in Free Surface Flow

    International Nuclear Information System (INIS)

    Hafsia, Zouhaier; Maalel, Khlifa; Mnasri, Chokri; Mohamed, Omri

    2009-01-01

    This paper describes two-dimensional numerical simulations of the water entry and exit of horizontal circular cylinder at constant velocity. The deformation of free surface is described by Navier-Stokes (N S) equations of incompressible and viscous fluid with additional transport equation of the volume-of-fluid (VOF). The motion of the cylinder is modeled by the associated momentum source term implemented in the Phoenicis (Parabolic Hyperbolic Or Elliptic Numerical Integration Code Series) code. The domain is discretized by a fixed Cartesian grid using a finite volume method and the cylinder is represented and cut cell method. The simulated results are compared with the numerical results of Lin (2007). This comparison shows good agreement in terms of free surface evolution for water exit and sinking. However, for water entry, the jet flow simulated by Lin is not reproduced. The free surface deformation around the cylinder in downward direction is accurately predicted

  6. Some Features of Aerodynamics of Cyclonic Chamber with Free Exit

    Directory of Open Access Journals (Sweden)

    A. N. Orekhov

    2007-01-01

    Full Text Available The paper cites results of an experimental research in aerodynamics of a cyclonic chamber with a free exit that has a large relative length. Distributions of aerodynamic stream characteristics depending on geometry of working volume of the cyclonic chamber are given in the paper. Calculative dependences are proposed in the paper.

  7. Diving, Jumping and Drinking: instabilities during water entry and exit

    Science.gov (United States)

    Jung, Sunghwan

    2017-11-01

    All organisms interact with fluids in one way or another, and some have presumably adapted their behaviors or features in response to fluid-mechanical forces. Particularly, fluid forces are of great importance when organisms or their body parts move in and out of water. In this talk, I will discuss three problems in which fluid mechanics principles affect form and function of animals. The first problem is how several seabirds (e.g. Gannets and Boobies) dive into water at up to 24 m/s without any injuries. This study examines the effects of their beak shape and dense feathers during water entry to reduce or spread the impact force on the body. The second problem is how animals jump out of water, from plankton to whales. Some aquatic animals generate enough force to exit the water surface as an effective method of capturing prey or escaping from predators. Finally, I will discuss about lapping animals (e.g. dog and cat) as a combined water entry and exit. During the tongue-lapping, associated fluid forces and pinch-off instability will be discussed.

  8. Steam exit flow design for aft cavities of an airfoil

    Science.gov (United States)

    Storey, James Michael; Tesh, Stephen William

    2002-01-01

    Turbine stator vane segments have inner and outer walls with vanes extending therebetween. The inner and outer walls have impingement plates. Steam flowing into the outer wall passes through the impingement plate for impingement cooling of the outer wall surface. The spent impingement steam flows into cavities of the vane having inserts for impingement cooling the walls of the vane. The steam passes into the inner wall and through the impingement plate for impingement cooling of the inner wall surface and for return through return cavities having inserts for impingement cooling of the vane surfaces. A skirt or flange structure is provided for shielding the steam cooling impingement holes adjacent the inner wall aerofoil fillet region of the nozzle from the steam flow exiting the aft nozzle cavities. Moreover, the gap between the flash rib boss and the cavity insert is controlled to minimize the flow of post impingement cooling media therebetween. This substantially confines outflow to that exiting via the return channels, thus furthermore minimizing flow in the vicinity of the aerofoil fillet region that may adversely affect impingement cooling thereof.

  9. Care of patients with Huntington's disease in South America: a survey

    Directory of Open Access Journals (Sweden)

    Ricardo Oliveira Horta Maciel

    2013-06-01

    Full Text Available Huntington's disease (HD is a rare neurodegenerative disease with a multitude of symptoms, which requires access to specialized multidisciplinary care for adequate management. The aim of this study was to survey the characteristics of care in various HD centers in South America (SA. Methods A questionnaire was sent to 24 centers involved in the care for HD patients in SA. Results Of the total 24 centers, 19 (79.2% are academic units. The majority of centers (62.5% are general movement disorders clinics. Multidisciplinary care is available in 19 (79.2% centers and in 20 (83.3% care is provided free of charge. Genetic testing and counseling are available in 25 and 66.6% of centers, respectively. The majority of centers (83.3% have no institutional support for end-stage care. Conclusions Although HD centers in SA are committed to providing multidisciplinary care, access to genetic counseling and end-stage care are lacking in most centers.

  10. Is gardening a stimulating activity for people with advanced Huntington's disease?

    Science.gov (United States)

    Spring, Josephine A; Viera, Marc; Bowen, Ceri; Marsh, Nicola

    2014-11-01

    This study evaluated adapted gardening as an activity for people with advanced Huntington's disease (HD) and explored its therapeutic aspects. Visitors and staff completed a questionnaire and participated in structured interviews to capture further information, whereas a pictorial questionnaire was designed for residents with communication difficulties. Staff reported that gardening was a constructive, outdoor activity that promoted social interaction, physical activity including functional movement and posed cognitive challenges. Half the staff thought the activity was problem free and a third used the garden for therapy. Visitors used the garden to meet with residents socially. Despite their disabilities, HD clients enjoyed growing flourishing flowers and vegetables, labelling plants, being outside in the sun and the quiet of the garden. The garden is valued by all three groups. The study demonstrates the adapted method of gardening is a stimulating and enjoyable activity for people with advanced HD. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  11. Gardening with Huntington's disease clients--creating a programme of winter activities.

    Science.gov (United States)

    Spring, Josephine Anne; Baker, Mark; Dauya, Loreane; Ewemade, Ivie; Marsh, Nicola; Patel, Prina; Scott, Adrienne; Stoy, Nicholas; Turner, Hannah; Viera, Marc; Will, Diana

    2011-01-01

    A programme of garden-related indoor activities was developed to sustain a gardening group for people with mid to late stage Huntington's disease during the winter. The activities were devised by the horticulturist, working empirically, involving the services occupational therapist, physiotherapist, occupational therapy art technician, computer room, recreation and leisure staff. The programme was strongly supported by the nursing and care staff. Feedback on the effectiveness of the activities was sought from the clients, team members and unit staff. The clients' interest in gardening was sustained by a multidisciplinary programme of indoor growing and using plant products in creative activities, computing and group projects. The clients enjoyed all activities except one that they said lacked contact with plants. The inexpensive programme of activities enabled creativity and self-expression, stimulated social contact and helped with therapeutic goals of the clients. In addition, it engaged the multi-disciplinary team and the unit staff, was practical and enhanced the environment.

  12. Relationship of core exit-temperature noise to thermal-hydraulic conditions in PWRs

    International Nuclear Information System (INIS)

    Sweeney, F.J.; Upadhyaya, B.R.

    1983-01-01

    Core exit thermocouple temperature noise and neutron detector noise measurements were performed at the Loss of Fluid Test Facility (LOFT) reactor and a Westinghouse, 1148 MW(e) PWR to relate temperature noise to core thermal-hydraulic conditions. The noise analysis results show that the RMS of the temperature noise increases linearly with increasing core δT at LOFT and the commercial PWR. Out-of-core test loop temperature noise has shown similar behavior. The phase angle between core exit temperature noise and in-core or ex-core neutron noise is directly related to the core coolant flow velocity. However, if the thermocouple response time is slow, compared to the coolant transit time between the sensors, velocities inferred from the phase angle are lower than measured coolant flow velocities

  13. The Ribosomal Protein uL22 Modulates the Shape of the Protein Exit Tunnel

    DEFF Research Database (Denmark)

    Wekselman, Itai; Zimmerman, Ella; Davidovich, Chen

    2017-01-01

    Erythromycin is a clinically useful antibiotic that binds to an rRNA pocket in the ribosomal exit tunnel. Commonly, resistance to erythromycin is acquired by alterations of rRNA nucleotides that interact with the drug. Mutations in the β hairpin of ribosomal protein uL22, which is rather distal...... of the β hairpin of the mutated uL22 toward the interior of the exit tunnel, triggering a cascade of structural alterations of rRNA nucleotides that propagate to the erythromycin binding pocket. Our findings support recent studies showing that the interactions between uL22 and specific sequences within...

  14. S-phase Synchronization Facilitates the Early Progression of Induced-Cardiomyocyte Reprogramming through Enhanced Cell-Cycle Exit.

    Science.gov (United States)

    Bektik, Emre; Dennis, Adrienne; Pawlowski, Gary; Zhou, Chen; Maleski, Danielle; Takahashi, Satoru; Laurita, Kenneth R; Deschênes, Isabelle; Fu, Ji-Dong

    2018-05-04

    Direct reprogramming of fibroblasts into induced cardiomyocytes (iCMs) holds a great promise for regenerative medicine and has been studied in several major directions. However, cell-cycle regulation, a fundamental biological process, has not been investigated during iCM-reprogramming. Here, our time-lapse imaging on iCMs, reprogrammed by Gata4, Mef2c, and Tbx5 (GMT) monocistronic retroviruses, revealed that iCM-reprogramming was majorly initiated at late-G1- or S-phase and nearly half of GMT-reprogrammed iCMs divided soon after reprogramming. iCMs exited cell cycle along the process of reprogramming with decreased percentage of 5-ethynyl-20-deoxyuridine (EdU)⁺/α-myosin heavy chain (αMHC)-GFP⁺ cells. S-phase synchronization post-GMT-infection could enhance cell-cycle exit of reprogrammed iCMs and yield more GFP high iCMs, which achieved an advanced reprogramming with more expression of cardiac genes than GFP low cells. However, S-phase synchronization did not enhance the reprogramming with a polycistronic-viral vector, in which cell-cycle exit had been accelerated. In conclusion, post-infection synchronization of S-phase facilitated the early progression of GMT-reprogramming through a mechanism of enhanced cell-cycle exit.

  15. Association between keeping home records of catheter exit-site and incidence of peritoneal dialysis-related infections.

    Science.gov (United States)

    Iida, Hidekazu; Kurita, Noriaki; Fujimoto, Shino; Kamijo, Yuka; Ishibashi, Yoshitaka; Fukuma, Shingo; Fukuhara, Shunichi

    2018-04-01

    To prevent peritoneal dialysis (PD)-related infection, components of self-catheter care have been emphasized. However, studies on the effectiveness of home recording for the prevention of PD-related infections are limited. This study aimed to examine the association between keeping home records of catheter exit site and incidence of PD-related infections. Home record books were submitted by patients undergoing PD. The proportion of days on which exit-site home recording was carried out for 120 days (0-100%) was obtained. The patients were divided into the frequent home recording group (≥ 40.5%; median value) and the infrequent home recording group (home recording groups for PD-related infection were 1.58 (95% confidence interval [CI], 0.72-3.46) in the univariate analysis and 1.49 (95% CI, 0.65-3.42) in the multivariate analysis. The IRRs of the frequent versus infrequent home recording groups for composite of surgery to create a new exit site and removal of PD catheter were 0.55 (95% CI, 0.78-3.88) and 0.35 (95% CI, 0.06-1.99), respectively. This study could not prove that keeping home records of patients' catheter exit site is associated with a lower incidence of PD-related infections.

  16. All rights reserved and www.bioline.org.br/ja Estimation of exit ...

    African Journals Online (AJOL)

    ADOWIE PERE

    and www.bioline.org.br/ja. Estimation of exit temperatures in the isentropic compression of real gases. 1 ..... other thermodynamic properties can be calculated within the framework of ... of negative compressibility) has no physical significance.

  17. Estimation of exit temperatures in the isentropic compression of real ...

    African Journals Online (AJOL)

    This paper presents the estimation of exit temperatures in the isentropic compression of real gases based on the Peng-Robinson equation of state and entropy balance method. The methods were applied to Ar, N2, CH4, CO2, C2H4 and C2H6. Data obtained revealed that isentropic exponent method provides useful results ...

  18. Relationship between Amount of Exited Blood During Wet-cupping with Patient’s Individual Conditions and the Time of Doing it

    Directory of Open Access Journals (Sweden)

    Ghazanfari M

    2017-12-01

    Full Text Available Aims Wet-cupping as a therapeutic method has been recommended to be done on certain days based on authentic sources of traditional medicine in Iran. The purpose of this study was to determine the relationship between amount of exited blood during wet-cupping with patient’s individual conditions and the time it was performed. Instruments & Methods In this descriptive cross-sectional study, 391 men who were referred to a cupping center in Saveh city in the spring of 2016, were selected by simple sampling and wet-cupping in the position between the two scapula was performed. The research instrument was a questionnaire including demographic characteristics, date of reference (day, month and year, age, height, weight and amount of exited blood during the wet-cupping in gram. Data were analyzed by SPSS 16 software and using chi-square test, paired t test and one-way analysis of variance. Findings The means of age, weight and body mass index were significantly related to the mean of amount of exited blood during wet-cupping (p0.05, but the amount of exited blood was higher in people with previous history of wet-cupping than those without previous history (p<0.001. Also, in people with hypertension, the amount of exited blood was higher than other treatment groups (p<0.05. Conclusion Individual characteristics such as age, weight, body mass index, cause of referral and previous history of wet-cupping affect the amount of exited blood during wet-cupping, but the time of wet-cupping (day and month does not affect the amount of exited blood.

  19. Helping Survivors of Human Trafficking: A Systematic Review of Exit and Postexit Interventions.

    Science.gov (United States)

    Dell, Nathaniel A; Maynard, Brandy R; Born, Kara R; Wagner, Elizabeth; Atkins, Bonnie; House, Whitney

    2017-01-01

    Human trafficking is a global problem and results in deleterious psychological, social, and physical effects on the lives of those who are trafficked; however, it is not clear how to best intervene with survivors. The purpose of this review was to synthesize the evidence of exit and postexit intervention programs for survivors of human trafficking to inform practice and research. Systematic review methods were used to search, select, and extract data from published and unpublished experimental, quasi-experimental, and preexperimental studies that assessed the effects of any exit or postexit interventions for victims of human trafficking. The authors searched eight databases, reviewed bibliographies, and conducted forward citation searches from relevant reports and prior reviews to find studies authored between 2005 and 2015. The search yielded six eligible studies that included 155 female and 6 male survivors from four countries. Interventions were diverse, with three using a trauma-informed approach. Authors measured a myriad of outcomes, including mental health, social network, community reintegration, and employment; however, the quality of most studies was poor. Evidence of effects of exit and postexit interventions is sparse, and much of the research is poorly designed and executed; however, the needs of trafficking survivors are complex and effective interventions are desperately needed. Implications for practice and research are discussed.

  20. Radiation degradation of aromatic pollutants exit in wastewater and ph dependence

    CERN Document Server

    Takriti, S

    2002-01-01

    The effect of gamma radiation on the degradation of phenol (hydroxybenzene), resorcinol (1,3 dihydroxybenzen) and hydroquinone (1,4 dihydroxybenzen) exit in waste water was investigated. The concentrations of these pollutants as well as the irradiated solution ph were studied. The results showed that the phenol is very resistance against the radiation doses comparing the other phenol compounds. Phenol was also a product of radiolysis of resorcinol and hydroquinone. On the other hand, the acid phase of the irradiation sample increased the degradation rate of pollutants. Spectrophotometer (UV-VIS) and chromatography (HPLC) were used to monitor the analysis of the radiation product solution. The results illustrated the existing of many substances such as organic alcohol, aldehyde, ketone and acidic functional groups as a final radiation products. The degradation of benzene, monochlorobenzene (CB) and 1,2 dichlorobenzene (1,2 DCB) exit in waste water by gamma irradiation was investigated. The effect of the irradi...

  1. Identifying Method of Drunk Driving Based on Driving Behavior

    Directory of Open Access Journals (Sweden)

    Xiaohua Zhao

    2011-05-01

    Full Text Available Drunk driving is one of the leading causes contributing to traffic crashes. There are numerous issues that need to be resolved with the current method of identifying drunk driving. Driving behavior, with the characteristic of real-time, was extensively researched to identify impaired driving behaviors. In this paper, the drives with BACs above 0.05% were defined as drunk driving state. A detailed comparison was made between normal driving and drunk driving. The experiment in driving simulator was designed to collect the driving performance data of the groups. According to the characteristics analysis for the effect of alcohol on driving performance, seven significant indicators were extracted and the drunk driving was identified by the Fisher Discriminant Method. The discriminant function demonstrated a high accuracy of classification. The optimal critical score to differentiate normal from drinking state was found to be 0. The evaluation result verifies the accuracy of classification method.

  2. In vivo dosimetry with thermoluminescent dosimeters in radiotherapy: entrance and exit doses

    International Nuclear Information System (INIS)

    Alves, C.; Lopes, M.C.

    2000-01-01

    In vivo dosimetry, by entrance and exit dose measurements, is a vital part of a radiotherapy quality assurance program. The uncertainty associated with dose delivery is internationally accepted to be within 5% or inferior depending on the tumor pathology. Thermoluminescent dosimetry is one of the dosimetric techniques used to verify the agreement between delivered and prescribed doses. Nevertheless, it requires a very accurate calibration methodology. We have used LiF chips (4.5 mm diameter and 0.8 mm thick) calibrated towards a PTW ionization chamber of 0.3 cc, in three photon energies: Co-60, 4 and 6 MeV. The TLD reader used was a Rialto 688 from NE Technology and the annealing oven the Eurotherm type 815. The calibration methodology relies on the experimental determination of individual correction factors and on a correction factor derived from a control group of dosimeters. The exit and entrance dose measurements are performed in quite different situations. To be able to achieve those two quantities with TLD, these should be independently calibrated according to the measurement conditions. Alternatively, we can use a single calibration, in entrance dose, and convert the result to the exit dose value by introducing some correction factors. These corrections are related to the different measurement depths and to the different backscattering contributions. We have proved that within an acceptable error we can perform a single calibration and adopt the correction factors which are energy and field size dependent. (author)

  3. Effects of inlet distortion on gas turbine combustion chamber exit temperature profiles

    Science.gov (United States)

    Maqsood, Omar Shahzada

    Damage to a nozzle guide vane or blade, caused by non-uniform temperature distributions at the combustion chamber exit, is deleterious to turbine performance and can lead to expensive and time consuming overhaul and repair. A test rig was designed and constructed for the Allison 250-C20B combustion chamber to investigate the effects of inlet air distortion on the combustion chamber's exit temperature fields. The rig made use of the engine's diffuser tubes, combustion case, combustion liner, and first stage nozzle guide vane shield. Rig operating conditions simulated engine cruise conditions, matching the quasi-non-dimensional Mach number, equivalence ratio and Sauter mean diameter. The combustion chamber was tested with an even distribution of inlet air and a 4% difference in airflow at either side. An even distribution of inlet air to the combustion chamber did not create a uniform temperature profile and varying the inlet distribution of air exacerbated the profile's non-uniformity. The design of the combustion liner promoted the formation of an oval-shaped toroidal vortex inside the chamber, creating localized hot and cool sections separated by 90° that appeared in the exhaust. Uneven inlet air distributions skewed the oval vortex, increasing the temperature of the hot section nearest the side with the most mass flow rate and decreasing the temperature of the hot section on the opposite side. Keywords: Allison 250, Combustion, Dual-Entry, Exit Temperature Profile, Gas Turbine, Pattern Factor, Reverse Flow.

  4. Firm Size and Short-Term Dynamics in Aggregate Entry and Exit

    NARCIS (Netherlands)

    Manjon, M.C.

    2004-01-01

    Much of the research on industry dynamics focuses on the interdependence between the sectorial rates of entry and exit.This paper argues that the size of firms and the reaction-adjustment period are important conditions missed in this literature.I illustrate the effects of this omission using data

  5. Numerical identification of blade exit angle effect on the performance for a multistage centrifugal pump impeller

    Directory of Open Access Journals (Sweden)

    Babayigit Osman

    2015-01-01

    Full Text Available Nowadays, single and multistage centrifugal pumps are widely used in industrial and mining enterprises. One of the most important components of a centrifugal pump is the impeller. The performance characteristics are related to the pump comprising the head and the overall efficiency rely a great deal on the impeller geometry. In this work, effects of blade exit angle change on hydraulic efficiency of a multi stage pump impeller are investigated via Ansys-Fluent computational fluid dynamics software for constant width impeller entrance and exit gates, blade numbers and blade thickness. Firstly, the flow volume of a centrifugal pump impeller is generated and then mesh structure is formed for the full impeller flow volume. Secondly, rotational periodic flow model are adopted in order to examine the effect of periodic flow assumption on the performance predictions. Corresponding to the available experimental data, inlet mass flow rate, outlet static pressure and rotation of impeller are taken as 0.02m3s-1, 450 kPa and 2950 rpm, respectively for the water fluid. No slip boundary condition is exposed to all solid of surface in the flow volume. The continuity and Navier-Stokes equations with the k-ε turbulence model and the standard wall functions are used. During the study, numerical analyses are conducted for the blade exit angle values of 18°, 20°, 25°, 30° and 35°. In consequence of the performed analyses, it is determined that hydraulic efficiency of the pump impeller value is changed between 81.0-84.6%. The most convenient blade exit angle that yields 84.6% hydraulic efficiency at is 18°. The obtained results show that the blade exit angle range has an impact on the centrifugal pump performance describing the pump head and the hydraulic efficiency.

  6. Environmental regulations and plant exit: A logit analysis based on established panel data

    Energy Technology Data Exchange (ETDEWEB)

    Bioern, E; Golombek, R; Raknerud, A

    1995-12-01

    This publication uses a model to study the relationship between environmental regulations and plant exit. It has the main characteristics of a multinomial qualitative response model of the logit type, but also has elements of a Markov chain model. The model uses Norwegian panel data for establishments in three manufacturing sectors with high shares of units which have been under strict environmental regulations. In two of the sectors, the exit probability of non-regulated establishments is about three times higher than for regulated ones. It is also found that the probability of changing regulation status from non-regulated to regulated depends significantly on economic factors. In particular, establishments with weak profitability are the most likely to become subject to environmental regulation. 12 refs., 2 figs., 6 tabs.

  7. Self-rated driving and driving safety in older adults.

    Science.gov (United States)

    Ross, Lesley A; Dodson, Joan E; Edwards, Jerri D; Ackerman, Michelle L; Ball, Karlene

    2012-09-01

    Many U.S. states rely on older adults to self-regulate their driving and determine when driving is no longer a safe option. However, the relationship of older adults' self-rated driving in terms of actual driving competency outcomes is unclear. The current study investigates self-rated driving in terms of (1) systematic differences between older adults with high (good/excellent) versus low (poor/fair/average) self-ratings, and (2) the predictive nature of self-rated driving to adverse driving outcomes in older adults (n=350; mean age 73.9, SD=5.25, range 65-91). Adverse driving outcomes included self-reported incidences of (1) being pulled over by the police, (2) receiving a citation, (3) receiving a recommendation to cease or limit driving, (4) crashes, and (5) state-reported crashes. Results found that older drivers with low self-ratings reported more medical conditions, less driving frequency, and had been given more suggestions to stop/limit their driving; there were no other significant differences between low and high self-raters. Logistic regression revealed older drivers were more likely to have a state-reported crash and receive a suggestion to stop or limit driving. Men were more likely to report all adverse driving outcomes except for receiving a suggestion to stop or limit driving. Regarding self-rated driving, older adults with high ratings were 66% less likely (OR=0.34, 95% CI=0.14-0.85) to have received suggestions to limit or stop driving after accounting for demographics, health and driving frequency. Self-ratings were not predictive of other driving outcomes (being pulled over by the police, receiving a citation, self-reported crashes, or state-reported crashes, ps>0.05). Most older drivers (85.14%) rated themselves as either good or excellent drivers regardless of their actual previous citation or crash rates. Self-rated driving is likely not related to actual driving proficiency as indicated by previous crash involvement in older adults

  8. Retrospectively assessed physical work environment during working life and risk of sickness absence and labour market exit among older workers

    DEFF Research Database (Denmark)

    Sundstrup, Emil; Hansen, Åse Marie; Mortensen, Erik Lykke

    2018-01-01

    OBJECTIVE: To determine the prospective association between retrospectively assessed physical work environment during working life and prospectively assessed sickness absence and labour market exit among older workers. METHODS: Using Cox regression analyses we estimated the 4-year to 6-year...... and exposure to several factors in the physical work environment, especially heavy lifting, were important for labour market exit and sickness absence. This study underscores the importance of reducing physical work exposures throughout the working life course for preventing sickness absence and premature exit...... from the labour market....

  9. The Drive-Wise Project: Driving Simulator Training increases real driving performance in healthy older drivers

    Directory of Open Access Journals (Sweden)

    Gianclaudio eCasutt

    2014-05-01

    Full Text Available Background: Age-related cognitive decline is often associated with unsafe driving behavior. We hypothesized that 10 active training sessions in a driving simulator increase cognitive and on-road driving performance. In addition, driving simulator training should outperform cognitive training.Methods: Ninety-one healthy active drivers (62 – 87 years were randomly assigned to either (1 a driving simulator training group, (2 an attention training group (vigilance and selective attention, or (3 a control group. The main outcome variables were on-road driving and cognitive performance. Seventy-seven participants (85% completed the training and were included in the analyses. Training gains were analyzed using a multiple regression analysis with planned comparisons.Results: The driving simulator training group showed an improvement in on-road driving performance compared to the attention training group. In addition, both training groups increased cognitive performance compared to the control group. Conclusion: Driving simulator training offers the potential to enhance driving skills in older drivers. Compared to the attention training, the simulator training seems to be a more powerful program for increasing older drivers’ safety on the road.

  10. Quantifying groundwater dependency of riparian surface hydrologic features using the exit gradient

    Science.gov (United States)

    This study examines groundwater exit gradients as a way to quantify groundwater interactions with surface water. We calibrated high resolution groundwater models for the basin fill sediments in the lower Calapooia watershed, Oregon, using data collected between 1928--2000. The e...

  11. The relationship between substance use and exit security on psychiatric wards

    NARCIS (Netherlands)

    Simpson, A.; Bowers, L.; Allan, T.; Haglund, K.; Muir-Cochrane, E.; Nijman, H.L.I.; Merwe, M. van der

    2011-01-01

    Aim.  In this paper we report on the rates of drug/alcohol use on acute psychiatric wards in relation to levels and intensity of exit security measures. Background.  Many inpatient wards have become permanently locked, with staff concerned about the risk of patients leaving the ward and harming

  12. Estimates of the first Dirichlet eigenvalue from exit time moment spectra

    DEFF Research Database (Denmark)

    Hurtado, Ana; Markvorsen, Steen; Palmer, Vicente

    2013-01-01

    We compute the first Dirichlet eigenvalue of a geodesic ball in a rotationally symmetric model space in terms of the moment spectrum for the Brownian motion exit times from the ball. This expression implies an estimate as exact as you want for the first Dirichlet eigenvalue of a geodesic ball...

  13. Electrical drives for direct drive renewable energy systems

    CERN Document Server

    Mueller, Markus

    2013-01-01

    Wind turbine gearboxes present major reliability issues, leading to great interest in the current development of gearless direct-drive wind energy systems. Offering high reliability, high efficiency and low maintenance, developments in these direct-drive systems point the way to the next generation of wind power, and Electrical drives for direct drive renewable energy systems is an authoritative guide to their design, development and operation. Part one outlines electrical drive technology, beginning with an overview of electrical generators for direct drive systems. Principles of electrical design for permanent magnet generators are discussed, followed by electrical, thermal and structural generator design and systems integration. A review of power electronic converter technology and power electronic converter systems for direct drive renewable energy applications is then conducted. Part two then focuses on wind and marine applications, beginning with a commercial overview of wind turbine drive systems and a...

  14. Self-rated Driving and Driving Safety in Older Adults

    OpenAIRE

    Ross, Lesley A.; Dodson, Joan; Edwards, Jerri D.; Ackerman, Michelle L.; Ball, Karlene

    2012-01-01

    Many U.S. states rely on older adults to self-regulate their driving and determine when driving is no longer a safe option. However, the relationship of older adults’ self-rated driving in terms of actual driving competency outcomes is unclear. The current study investigates self-rated driving in terms of (1) systematic differences between older adults with high (good/excellent) versus low (poor/fair/average) self-ratings, and (2) the predictive nature of self-rated driving to adverse driving...

  15. Huntington's disease-like and ataxia syndromes: identification of a family with a de novo SCA17/TBP mutation

    DEFF Research Database (Denmark)

    Bech, Sara; Petersen, Thor; Nørremølle, Anne

    2010-01-01

    The autosomal dominant spinocerebellar ataxias, commonly referred to as SCAs, are clinically and genetically heterogeneous neurodegenerative disorders. Twenty-eight genetic subtypes have been identified, of which 7 are caused by expansion of a CAG trinucleotide repeat that encodes a polyglutamine....... The patient's mother and father both carried normal range repeats, 38/38 and 33/39 respectively. Analysis of the repeat structures revealed that the expansion had occurred upon expansion of the longer paternal allele. We conclude that, however rare, SCA17 must be considered as a cause of Huntington's disease...

  16. Características del habla, el lenguaje y la deglución en la enfermedad de Huntington

    OpenAIRE

    Camargo-Mendoza, Maryluz; Castillo-Triana, Nicolás; Fandiño-Cardona, Juan Miguel; Mateus-Moreno, Angélica; Moreno-Martínez, Mariana

    2017-01-01

    Resumen La enfermedad de Huntington (EH) ha sido descrita como una afección de causa genética producida por una mutación en la repetición de la secuencia de nucleótidos CAG (citosina-adenina-guanina). Según el estadio que curse la enfermedad, las personas pueden presentar dificultades en el habla, el lenguaje y la deglución. El propósito de este artículo es exponer con detalle dichas dificultades, así como su tratamiento fonoaudiológico. Se destaca que en el habla se encuentran característica...

  17. A two-stage compound parabolic concentrator system with a large entrance over the exit aperture ratio

    International Nuclear Information System (INIS)

    Angelescu, Tatiana; Radu, A. A.

    2000-01-01

    Certain optical designs in the field of high energy gamma ray astronomy components of the Cherenkov light, collected by the mirror of telescope, be concentrated on the photo-cathodes of the photomultiplier tubes, with the help of the light collectors having large entrance and small exit apertures. Mathematical restrictions imposed by the design of the compound parabolic concentrator (CPC) implied that for a given cut-off angle and an entrance aperture, the exit aperture of the CPC should not exceed a limit value. If this value is larger than the active diameter of the photocathode, an additional concentrator must be added to the system in order to transfer the light collected, from the exit aperture of the compound parabolic concentrator to the photocathode of the photomultiplier tube. Different designs of a two-stage system composed by a a hollow compound parabolic concentrator and a solid, dielectric filled concentrator are evaluated in this paper, from the point of view of optical efficiency and manufacturability. (authors)

  18. Central Banks Exit Strategies in Theory and Practice. The Case of the Polish National Bank's Policy

    Directory of Open Access Journals (Sweden)

    Anna Szelągowska

    2015-07-01

    Full Text Available This paper makes an attempt in discussing the pros and cons of central banks exiting from unconventional monetary policy strategies. Having in mind the latest international economic research concerning the optimal entrance and exit strategies of the zero interest rate policy, it is worth discussing the results of the contemporary central banks in preserving the financial system and supporting the real economy. The main aim of this paper is the assessment of the Polish central bank's low rate interest rates policy effectiveness and to find out its influence on the economy. The following research problem is discussed: should central banks use the exit strategy from zero interest rates and if yes, why and when. This task requires to put forward the following research hypothesis: too late implementation of the cycle of low interest rates by the National Bank of Poland does not help improvement of the Polish economy situation.

  19. Constitutive upregulation of chaperone-mediated autophagy in Huntington's disease.

    Science.gov (United States)

    Koga, Hiroshi; Martinez-Vicente, Marta; Arias, Esperanza; Kaushik, Susmita; Sulzer, David; Cuervo, Ana Maria

    2011-12-14

    Autophagy contributes to the removal of prone-to-aggregate proteins, but in several instances these pathogenic proteins have been shown to interfere with autophagic activity. In the case of Huntington's disease (HD), a congenital neurodegenerative disorder resulting from mutation in the huntingtin protein, we have previously described that the mutant protein interferes with the ability of autophagic vacuoles to recognize cytosolic cargo. Growing evidence supports the existence of cross talk among autophagic pathways, suggesting the possibility of functional compensation when one of them is compromised. In this study, we have identified a compensatory upregulation of chaperone-mediated autophagy (CMA) in different cellular and mouse models of HD. Components of CMA, namely the lysosome-associated membrane protein type 2A (LAMP-2A) and lysosomal-hsc70, are markedly increased in HD models. The increase in LAMP-2A is achieved through both an increase in the stability of this protein at the lysosomal membrane and transcriptional upregulation of this splice variant of the lamp-2 gene. We propose that CMA activity increases in response to macroautophagic dysfunction in the early stages of HD, but that the efficiency of this compensatory mechanism may decrease with age and so contribute to cellular failure and the onset of pathological manifestations.

  20. Huntington's disease in Greece: the experience of 14 years.

    Science.gov (United States)

    Panas, M; Karadima, G; Vassos, E; Kalfakis, N; Kladi, A; Christodoulou, K; Vassilopoulos, D

    2011-12-01

    A large scale genetic and epidemiological study of Huntington's disease (HD) was carried out in Greece from January 1995 to December 2008. Diagnostic testing was carried out in 461 symptomatic individuals, while 256 were tested for presymptomatic purposes. The diagnosis of HD with a CAG expansion ≥ 36 was confirmed in 278 symptomatic individuals. The prevalence of HD in Greece was estimated at approximately 2.5 to 5.4:100,000, while the mean minimum incidence was estimated at 2.2 to 4.4 per million per year. The molecular diagnosis of HD was confirmed in the majority of patients (84.4%) sent for confirmation. The false-positive cases 15.6% were characterized by the absence of a family history of HD and the presence of an atypical clinical picture. The uptake of predictive testing for HD was 8.6%. A prenatal test was requested in six pregnancies. The findings of our study do not differ significantly from those of similar studies from other European countries despite the relative genetic isolation of Greece. Of interest is the identification of clusters of HD in Greece. The presence or absence of a family history of HD should be interpreted cautiously, during the diagnostic process. © 2010 John Wiley & Sons A/S.

  1. Reduction of IFNα and IL-10 in central nervous system and increase in peripheral IL-8 in transgenic porcine Huntington´s disease model

    Czech Academy of Sciences Publication Activity Database

    Kovářová, Hana; Valeková, Ivona; Jarkovská, Karla; Kotrčová, Eva; Motlík, Jan; Gadher, S. J.

    2015-01-01

    Roč. 78, Suppl 2 (2015), s. 10-11 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124 Institutional support: RVO:67985904 Keywords : porcine Huntington ´s disease model * IFNα * IL-10 Subject RIV: EB - Genetics ; Molecular Biology

  2. Care of patients with Huntington's disease in South America: a survey Cuidado de pacientes com doença de Huntington na América do Sul: um inquérito

    Directory of Open Access Journals (Sweden)

    Ricardo Oliveira Horta Maciel

    2013-06-01

    Full Text Available Huntington's disease (HD is a rare neurodegenerative disease with a multitude of symptoms, which requires access to specialized multidisciplinary care for adequate management. The aim of this study was to survey the characteristics of care in various HD centers in South America (SA. Methods A questionnaire was sent to 24 centers involved in the care for HD patients in SA. Results Of the total 24 centers, 19 (79.2% are academic units. The majority of centers (62.5% are general movement disorders clinics. Multidisciplinary care is available in 19 (79.2% centers and in 20 (83.3% care is provided free of charge. Genetic testing and counseling are available in 25 and 66.6% of centers, respectively. The majority of centers (83.3% have no institutional support for end-stage care. Conclusions Although HD centers in SA are committed to providing multidisciplinary care, access to genetic counseling and end-stage care are lacking in most centers. A doença de Huntington (DH é uma doença neurodegenerativa rara que requer tratamento multidisciplinar especializado para manejo adequado. O objetivo do presente trabalho foi pesquisar as características da assistência à saúde em centros de DH na América do Sul (AS. Métodos Um questionário foi enviado para 24 centros envolvidos no cuidado de pacientes com DH na AS. Resultados Dos 24 centros, 19 (79,2% são unidades acadêmicas. A maioria (62,5% são clínicas de distúrbios dos movimentos. Cuidado multidisciplinar é disponível em 19 (79,2% dos centros e em 20 (83,3%, o tratamento é gratuito. O teste e o aconselhamento genético estão disponíveis em 25 e 66,6% dos centros, respectivamente. Não há suporte institucional para cuidado terminal em 83,3% dos centros. Conclusões Apesar dos centros de DH na AS terem compromisso com o provimento de cuidados multidisciplinares, o acesso a aconselhamento genético e a tratamento na fase terminal são falhos na maioria dos centros.

  3. "Now My Old Self Is Thin": Stigma Exits after Weight Loss

    Science.gov (United States)

    Granberg, Ellen M.

    2011-01-01

    In this article, I employ a structural symbolic interaction framework to examine the processes by which persons can exit a stigmatized identity. Using the empirical example of weight loss, I analyze how individuals evolve from an identity as "fat" and stigmatized to one that is "normal" with respect to weight and free from identification with…

  4. Voice, exit and efficacy : Dealing with perceived neighbourhood decline without moving out

    NARCIS (Netherlands)

    Van der Land, M.; Doff, W.

    2010-01-01

    Residents of deprived urban neighbourhoods with a changing population often experience an increase in insecurity. If they judge the change as decline, they are likely to become less satisfied with their residential situation and exhibit coping tactics. This paper combines Hirschman’s Exit, Voice and

  5. Labour market entries and exits of women from different origin countries in the UK.

    Science.gov (United States)

    Khoudja, Yassine; Platt, Lucinda

    2018-01-01

    Labour force participation rates of women differ strongly by ethnic origin. Even though existing research using cross-sectional studies has demonstrated that part of these differences can be attributed to compositional differences in human capital, household conditions and gender attitudes, residual 'ethnic effects' typically remain. To further our understanding of women's labour market behaviour across ethnic groups, we use a large-scale longitudinal study and apply a dynamic perspective to examine how far relevant life-course events in addition to individual characteristics, gender attitudes and religiosity contribute to the explanation of ethnic differences in women's labour force entries and exits in the UK. Our findings show that, adjusting for all these factors, Indian and Caribbean women do not differ from White majority women in their labour force entry and exit probabilities but that Pakistani and Bangladeshi women are less likely to enter and more likely to exit the labour market, whereas Black African women have higher entry rates. We also find that relations between life-course events and labour market transitions differ by ethnic group. Most notably, Pakistani and Bangladeshi women's labour market transitions are less sensitive to child-bearing and Caribbean women's transitions less sensitive to partnership changes than other women's. Copyright © 2017 Elsevier Inc. All rights reserved.

  6. Liver function in Huntington's disease assessed by blood biochemical analyses in a clinical setting

    DEFF Research Database (Denmark)

    Nielsen, Signe Marie Borch; Vinther-Jensen, Tua; Nielsen, Jørgen E.

    2016-01-01

    dysfunction are seen. Blood levels of a wide range of hormones, metabolites and proteins have been analyzed in HD patients, identifying several changes associated with the disease. However, a comprehensive panel of liver function tests (LFT) has not been performed. We investigated a cohort of manifest......Huntington's disease (HD) is a dominantly inherited, progressive neurological disorder caused by a CAG repeat elongation in the huntingtin gene. In addition to motor-, psychiatric- A nd cognitive dysfunction, peripheral disease manifestations in the form of metabolic changes and cellular...... and premanifest HD gene-expansion carriers and controls, using a clinically applied panel of LFTs. Here, we demonstrate that the level of alkaline phosphatase is increased in manifest HD gene-expansion carriers compared to premanifest HD gene-expansion carriers and correlate with increased disease severity...

  7. Early grey matter changes in structural covariance networks in Huntington's disease.

    Science.gov (United States)

    Coppen, Emma M; van der Grond, Jeroen; Hafkemeijer, Anne; Rombouts, Serge A R B; Roos, Raymund A C

    2016-01-01

    Progressive subcortical changes are known to occur in Huntington's disease (HD), a hereditary neurodegenerative disorder. Less is known about the occurrence and cohesion of whole brain grey matter changes in HD. We aimed to detect network integrity changes in grey matter structural covariance networks and examined relationships with clinical assessments. Structural magnetic resonance imaging data of premanifest HD ( n  = 30), HD patients (n = 30) and controls (n = 30) was used to identify ten structural covariance networks based on a novel technique using the co-variation of grey matter with independent component analysis in FSL. Group differences were studied controlling for age and gender. To explore whether our approach is effective in examining grey matter changes, regional voxel-based analysis was additionally performed. Premanifest HD and HD patients showed decreased network integrity in two networks compared to controls. One network included the caudate nucleus, precuneous and anterior cingulate cortex (in HD p  covariance might be a sensitive approach to reveal early grey matter changes, especially for premanifest HD.

  8. Dementia & Driving

    Science.gov (United States)

    ... have to give up driving. Many people associate driving with self-reliance and freedom; the loss of driving privileges ... familiar roads and avoid long distances. Avoid heavy traffic and heavily traveled roads. Avoid driving at night and in bad weather. Reduce the ...

  9. Cardiovascular disease, diabetes and early exit from paid employment in Europe; the impact of work-related factors.

    Science.gov (United States)

    Kouwenhoven-Pasmooij, T A; Burdorf, A; Roos-Hesselink, J W; Hunink, M G M; Robroek, S J W

    2016-07-15

    The aims of the study were to examine (i) the association between cardiovascular disease (CVD) or diabetes and exit from paid employment via disability benefits, unemployment, early retirement or other exit routes; and (ii) the impact of work-related factors on exit from paid employment among individuals with CVD or diabetes. Respondents of the longitudinal Survey of Health and Retirement in Europe (SHARE) were included if they were aged >50years, had paid employment at baseline, and a known employment status after 2 or 6years (n=5182). A baseline-interview provided information on the presence of diagnosed CVD and diabetes, and physical and psychosocial work-related factors. During follow-up interviews information on work status was collected. Multinomial regression analyses were used to investigate the association between CVD, diabetes and exit from paid employment, and the impact of work-related factors. Workers with CVD or diabetes had significantly increased probabilities of disability benefits (OR 2.50, 95% CI 1.69-3.70) and early retirement (OR 1.34, 95% CI 1.05-1.74), but a comparable probability of unemployment (OR 1.10, 95% CI 0.71-1.71). Regarding disability benefits, individuals who had a stroke had the highest probability (OR 3.48, 95% CI 1.31-9.23). Perceived high job demands with low rewards or with low control at work further increased the probability of early exit among individuals with CVD or diabetes. Our study shows a prominent role of CVD and diabetes in premature losses to the workforce, and it shows that optimizing psychosocial work-related factors could be beneficial in people with CVD or diabetes. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  10. Perturbations in the p53/miR-34a/SIRT1 pathway in the R6/2 Huntington's disease model

    DEFF Research Database (Denmark)

    Reynolds, Regina Hertfelder; Petersen, Maria Hvidberg; Willert, Cecilie Wennemoes

    2018-01-01

    The three factors, p53, the microRNA-34 family and Sirtuin 1 (SIRT1), interact in a positive feedback loop involved in cell cycle progression, cellular senescence and apoptosis. Each factor in this triad has roles in metabolic regulation, maintenance of mitochondrial function, and regulation...... of brain-derived neurotrophic factor (BDNF). Thus, this regulatory network holds potential importance for the pathophysiology of Huntington's disease (HD), an inherited neurodegenerative disorder in which both mitochondrial dysfunction and impaired neurotrophic signalling are observed. We investigated...

  11. Total recognition discriminability in Huntington's and Alzheimer's disease.

    Science.gov (United States)

    Graves, Lisa V; Holden, Heather M; Delano-Wood, Lisa; Bondi, Mark W; Woods, Steven Paul; Corey-Bloom, Jody; Salmon, David P; Delis, Dean C; Gilbert, Paul E

    2017-03-01

    Both the original and second editions of the California Verbal Learning Test (CVLT) provide an index of total recognition discriminability (TRD) but respectively utilize nonparametric and parametric formulas to compute the index. However, the degree to which population differences in TRD may vary across applications of these nonparametric and parametric formulas has not been explored. We evaluated individuals with Huntington's disease (HD), individuals with Alzheimer's disease (AD), healthy middle-aged adults, and healthy older adults who were administered the CVLT-II. Yes/no recognition memory indices were generated, including raw nonparametric TRD scores (as used in CVLT-I) and raw and standardized parametric TRD scores (as used in CVLT-II), as well as false positive (FP) rates. Overall, the patient groups had significantly lower TRD scores than their comparison groups. The application of nonparametric and parametric formulas resulted in comparable effect sizes for all group comparisons on raw TRD scores. Relative to the HD group, the AD group showed comparable standardized parametric TRD scores (despite lower raw nonparametric and parametric TRD scores), whereas the previous CVLT literature has shown that standardized TRD scores are lower in AD than in HD. Possible explanations for the similarity in standardized parametric TRD scores in the HD and AD groups in the present study are discussed, with an emphasis on the importance of evaluating TRD scores in the context of other indices such as FP rates in an effort to fully capture recognition memory function using the CVLT-II.

  12. Plasma homovanillic acid and prolactin in Huntington's disease.

    Science.gov (United States)

    Markianos, Manolis; Panas, Marios; Kalfakis, Nikos; Vassilopoulos, Dimitrios

    2009-05-01

    Dopaminergic activity is expected to be altered in patients with Huntington's disease (HD) and be related to factors like duration and severity of illness or patients' specific symptomatology like dementia, depression, or psychotic features. We assessed plasma homovanillic acid (pHVA) and plasma prolactin (pPRL), two correlates of dopaminergic activity, in 116 subjects with CAG repeats expansion in the HD gene, 26 presymptomatic (18 females) and 90 with overt symptomatology (43 females). Patients were evaluated using the Unified HD Rating Scale and the Total Functional Capacity Scale. Presence of dementia, depression, and psychotic features were also assessed. The age range of the patients was 22-83 years, duration of illness from 0.5 to 27 years, and CAG repeat number from 34 to 66. A group of 60 age and sex matched healthy subjects served as control group. Plasma PRL in subjects at risk and in neuroleptic-free patients, evaluated separately for males and females, did not differ from controls. Plasma HVA levels did not differ from controls in the group of presymptomatic subjects, but were significantly higher in the patients group. This increase was positively associated mainly with severity of illness and functional capacity of the patients, and not with presence of depression or dementia. Plasma HVA levels may be proven to be a peripheral index of disease progression. Reducing dopaminergic activity may have not only symptomatic, but also neuroprotective effects in HD.

  13. Somatostatin receptor 1 and 5 double knockout mice mimic neurochemical changes of Huntington's disease transgenic mice.

    Directory of Open Access Journals (Sweden)

    Padmesh S Rajput

    Full Text Available Selective degeneration of medium spiny neurons and preservation of medium sized aspiny interneurons in striatum has been implicated in excitotoxicity and pathophysiology of Huntington's disease (HD. However, the molecular mechanism for the selective sparing of medium sized aspiny neurons and vulnerability of projection neurons is still elusive. The pathological characteristic of HD is an extensive reduction of the striatal mass, affecting caudate putamen. Somatostatin (SST positive neurons are selectively spared in HD and Quinolinic acid/N-methyl-D-aspartic acid induced excitotoxicity, mimic the model of HD. SST plays neuroprotective role in excitotoxicity and the biological effects of SST are mediated by five somatostatin receptor subtypes (SSTR1-5.To delineate subtype selective biological responses we have here investigated changes in SSTR1 and 5 double knockout mice brain and compared with HD transgenic mouse model (R6/2. Our study revealed significant loss of dopamine and cAMP regulated phosphoprotein of 32 kDa (DARPP-32 and comparable changes in SST, N-methyl-D-aspartic acid receptors subtypes, calbindin and brain nitric oxide synthase expression as well as in key signaling proteins including calpain, phospho-extracellular-signal-regulated kinases1/2, synapsin-IIa, protein kinase C-α and calcineurin in SSTR1/5(-/- and R6/2 mice. Conversely, the expression of somatostatin receptor subtypes, enkephalin and phosphatidylinositol 3-kinases were strain specific. SSTR1/5 appears to be important in regulating NMDARs, DARPP-32 and signaling molecules in similar fashion as seen in HD transgenic mice.This is the first comprehensive description of disease related changes upon ablation of G- protein coupled receptor gene. Our results indicate that SST and SSTRs might play an important role in regulation of neurodegeneration and targeting this pathway can provide a novel insight in understanding the pathophysiology of Huntington's disease.

  14. Map showing selected surface-water data for the Huntington 30 x 60-minute quadrangle, Utah

    Science.gov (United States)

    Price, Don

    1984-01-01

    This is one of a series of maps that describe the geology and related natural resources of the Huntington 30 x 60-minute quadrangle, Utah. Streamflow records used to compile this map were collected by the U.S. Geological Survey in cooperation with the Utah Department of Natural Resources, Division of Water Rights, and the Utah Department of Transportation. The principal runoff-producing area shown on the map was delineated from a work map (scale 1:250,000) compiled to estimate water yields in Utah (Bagley and others, 1964). Sources of information about recorded floods resulting from cloudbursts included Woolley (1946) and Butler and Marsell (1972); sources of information about the chemical quality of streamflow included Mundorff (1972) and Mundorff and Thompson (1982).

  15. Modulation at Age of Onset in Tunisian Huntington Disease Patients: Implication of New Modifier Genes

    Directory of Open Access Journals (Sweden)

    Dorra Hmida-Ben Brahim

    2014-01-01

    Full Text Available Huntington’s disease (HD is an autosomal dominant neurodegenerative disorder. The causative mutation is an expansion of more than 36 CAG repeats in the first exon of IT15 gene. Many studies have shown that the IT15 interacts with several modifier genes to regulate the age at onset (AO of HD. Our study aims to investigate the implication of CAG expansion and 9 modifiers in the age at onset variance of 15 HD Tunisian patients and to establish the correlation between these modifiers genes and the AO of this disease. Despite the small number of studied patients, this report consists of the first North African study in Huntington disease patients. Our results approve a specific effect of modifiers genes in each population.

  16. Aerial gamma ray and magnetic survey, Huntington quadrangle: Ohio, West Virginia and Kentucky. Final report

    International Nuclear Information System (INIS)

    1981-04-01

    The Huntington quadrangle of Kentucky, Ohio, and West Virginia covers 7250 square miles of the easternmost Midwestern Physiographic Province. Paleozoic exposures dominate the surface. These Paleozoics deepen toward the east from approximately 500 feet to a maximum depth of 8000 feet. Precambrian basement is thought to underlie the entire area. No known uranium deposits exist in the area. One hundred anomalies were found using the standard statistical analysis. Some high uranium concentration anomalies that may overlie the stratigraphic equivalent of the Devonian-Mississippian New Albany or Chattanooga Shales may represent significant levels of naturally occurring uranium. Future studies should concentrate on this unit. Magnetic data are largely in concurrence with existing structural interpretations but suggest some complexities in the underlying Precambrian

  17. Development of a portable system of grazing exit X-Ray fluorescence applied to environmental and biological studies

    Energy Technology Data Exchange (ETDEWEB)

    Santos, Ramon S.; Oliveira, Davi F.; Anjos, Marcelino J. [Universidade do Estado do Rio de Janeiro (UERJ), RJ (Brazil). Instituto de Fisica; Assis, Joaquim T., E-mail: ramonziosp@yahoo.com.br, E-mail: davi.oliveira@uerj.br, E-mail: marcelin@uerj.br, E-mail: joaquim.iprj@gmail.com [Universidade do Estado do Rio de Janeiro (UERJ), Nova Friburgo, RJ (Brazil). Instituto Politécnico

    2017-07-01

    In this study was developed a portable system of grazing exit X-ray fluorescence (geometric 90° - 0°) that will be applied in environmental studies (aerosol samples) and biological studies. GE-XRF portable system is formed by a mini X-ray tube of low power (anode of Au, maximum voltage and current of 50 kV and 200 μA, respectively) and a SiPIN detector (model XR-100CR of the Amptek). The reflectors used as sample support (sampler carrier) were quartz discs with 25.4 mm diameter and 3.0 mm thickness. The grazing exit angle was experimentally determined by measuring a cooper solution (10 μg.g{sup -1}). The system of GE-XRF proved to be quite stable and reproducible. It was calculated the sensibility curve of the system using multielement solutions. The accuracy of the system was checked using multielement reference solution as standard reference material. The relative errors between measured and certified values are in the range of 4 to 19%. The first results showed a background was drastically reduced at grazing exit angles, enabling trace elemental analysis. This paper shows that it is possible to produce a portable system of grazing exit X-ray fluorescence compact, efficient, low-cost and easy-to-handle instrumentation using a low-power X-ray tube and a SiPIN compact detector. (author)

  18. A qualitative exploration of driving stress and driving discourtesy.

    Science.gov (United States)

    Scott-Parker, B; Jones, C M; Rune, K; Tucker, J

    2018-05-31

    Driving courtesy, and conversely driving discourtesy, recently has been of great interest in the public domain. In addition, there has been increasing recognition of the negative impact of stress upon the individual's health and wellbeing, with a plethora of interventions aimed at minimising stress more generally. The research literature regarding driving dis/courtesy, in comparison, is scant, with a handful of studies examining the dis/courteous driving behaviour of road users, and the relationship between driving discourtesy and driving stress. To examine courteous and discourteous driving experiences, and to explore the impact of stress associated with such driving experiences. Thirty-eight drivers (20 females) from the Sunshine Coast region volunteered to participate in one of four 1-1.5 h focus groups. Content analysis used the verbatim utterances captured via an Mp3 device. Three themes pertaining to stressful and discourteous interactions were identified. Theme one pertained to the driving context: road infrastructure (eg, roundabouts, roadwork), vehicles (eg, features), location (eg, country vs city, unfamiliar areas), and temporal aspects (eg, holidays). Theme two pertained to other road users: their behaviour (eg, tailgating, merging), and unknown factors (eg, illicit and licit drug use). Theme three pertained to the self as road user: their own behaviours (eg, deliberate intimidation), and their emotions (eg, angry reaction to other drivers, being in control). Driving dis/courtesy and driving stress is a complex phenomenon, suggesting complex intervention efforts are required. Driving discourtesy was reported as being highly stressful, therefore intervention efforts which encourage driving courtesy and which foster emotional capacity to cope with stressful circumstances appear warranted. Copyright © 2018. Published by Elsevier Ltd.

  19. Understandings of psychological difficulties in people with the Huntington's disease gene and their expectations of psychological therapy.

    Science.gov (United States)

    Theed, Rachael; Eccles, Fiona J R; Simpson, Jane

    2018-06-01

    This study sought to investigate how people who had tested positive for the Huntington's disease (HD) gene mutation understood and experienced psychological distress and their expectations of psychological therapy. A qualitative methodology was adopted involving semi-structured interviews and interpretative phenomenological analysis (IPA). A total of nine participants (five women and four men) who had opted to engage in psychological therapy were recruited and interviewed prior to the start of this particular psychological therapeutic intervention. Interviews were transcribed verbatim and analysed using IPA whereby themes were analysed within and across transcripts and classified into superordinate themes. Three superordinate themes were developed: Attributing psychological distress to HD: 'you're blaming everything on that now'; Changes in attributions of distress over time: 'in the past you'd just get on with it'; and Approaching therapy with an open mind, commitment, and hope: 'a light at the end of the tunnel'. Understandings of psychological distress in HD included biological and psychological explanations, with both often being accepted simultaneously by the same individual but with biomedical accounts generally dominating. Individual experience seemed to reflect a dynamic process whereby people's understanding and experience of their distress changed over time. Psychological therapy was accepted as a positive alternative to medication, providing people with HD with hope that their psychological well-being could be enhanced. People with the Huntington's disease gene mutation have largely biomedical understandings of their psychological distress. This largely biomedical understanding does not, however, preclude them for being interested in the potential gains resulting from psychological therapy. The mechanisms of psychological therapy should be explained in detail before therapy and explored along with current attributions of distress. © 2017 The British

  20. Revelation of the IFN alpha, IL-10, IL-8 and IL-1 beta as promising biomarkers reflecting immuno-pathological mechanisms in porcine Huntington's disease model

    Czech Academy of Sciences Publication Activity Database

    Valeková, Ivona; Jarkovská, Karla; Kotrčová, Eva; Bucci, J.; Ellederová, Zdeňka; Juhás, Štefan; Motlík, Jan; Gadher, S. J.; Kovářová, Hana

    2016-01-01

    Roč. 293, č. 2 (2016), s. 71-81 ISSN 0165-5728 R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) LO1609 Institutional support: RVO:67985904 Keywords : porcine Huntington´s disease * immune response * cytokines * central nervous system * serum * biomarkers Subject RIV: FH - Neurology Impact factor: 2.720, year: 2016

  1. Effect of ITE and nozzle exit cone erosion on specific impulse of solid rocket motors

    Science.gov (United States)

    Smith-Kent, Randall; Ridder, Jeffrey P.; Loh, Hai-Tien; Abel, Ralph

    1993-06-01

    Specific impulse loss due to the use of a slowly eroding integral throat entrance, or a throat insert, with a faster eroding nozzle exit cone is studied. It is suggested that an oblique shock wave produced by step-off erosion results in loss of specific impulse. This is studied by use of a shock capturing CFD method. The shock loss predictions for first-stage Peacekeeper and Castor 25 motors are found to match the trends of the test data. This work suggests that a loss mechanism, previously unaccounted, should be considered in the specific impulse prediction procedure for nozzles with step-off exit cone erosion.

  2. Detection of early behavioral markers of Huntington's disease in R6/2 mice employing an automated social home cage

    DEFF Research Database (Denmark)

    Rudenko, Olga; Tkach, Vadim; Berezin, Vladimir

    2009-01-01

    developed behavior screening system, the IntelliCage, allows automated testing of mouse behavior in the home cage employing individual recognition of animals living in social groups. The present study validates the ability of the IntelliCage system to detect behavioral and cognitive dysfunction in R6/2 mice......Huntington's disease (HD) is an autosomal-dominant neurodegenerative disorder, for which no known cure or effective treatment exists. To facilitate the search for new potential treatments of HD, an automated system for analyzing the behavior of transgenic HD mice is urgently needed. A recently...

  3. Dose-Dependent Lowering of Mutant Huntingtin Using Antisense Oligonucleotides in Huntington Disease Patients.

    Science.gov (United States)

    van Roon-Mom, Willeke M C; Roos, Raymund A C; de Bot, Susanne T

    2018-04-01

    On December 11 of 2017, Ionis Pharmaceuticals published a press release announcing dose-dependent reductions of mutant huntingtin protein in their HTTRx Phase 1/2a study in Huntington disease (HD) patients. The results from this Ionis trial have gained much attention from the patient community and the oligonucleotide therapeutics field, since it is the first trial targeting the cause of HD, namely the mutant huntingtin protein, using antisense oligonucleotides (ASOs). The press release also states that the primary endpoints of the study (safety and tolerability) were met, but does not contain data. This news follows the approval of another therapeutic ASO nusinersen (trade name Spinraza) for a neurological disease, spinal muscular atrophy, by the U.S. Food and Drug Administration and European Medicines Agency, in 2016 and 2017, respectively. Combined, this offers hope for the development of the HTTRx therapy for HD patients.

  4. Electric drives

    CERN Document Server

    Boldea, Ion

    2005-01-01

    ENERGY CONVERSION IN ELECTRIC DRIVESElectric Drives: A DefinitionApplication Range of Electric DrivesEnergy Savings Pay Off RapidlyGlobal Energy Savings Through PEC DrivesMotor/Mechanical Load MatchMotion/Time Profile MatchLoad Dynamics and StabilityMultiquadrant OperationPerformance IndexesProblemsELECTRIC MOTORS FOR DRIVESElectric Drives: A Typical ConfigurationElectric Motors for DrivesDC Brush MotorsConventional AC MotorsPower Electronic Converter Dependent MotorsEnergy Conversion in Electric Motors/GeneratorsPOWER ELECTRONIC CONVERTERS (PECs) FOR DRIVESPower Electronic Switches (PESs)The

  5. A firm-level dataset for analyzing entry, exit, employment and R&D expenditures in the UK: 1997-2012.

    Science.gov (United States)

    Ugur, Mehmet; Trushin, Eshref; Solomon, Edna

    2016-09-01

    This data article is related to the research article entitled "Inverted-U relationship between R&D intensity and survival: Evidence on scale and complementarity effects in UK data" (Ugur et al., In press) [1]. It describes the trends in R&D expenditures, employment of R&D personnel and firm entry and exit rates in the UK from 1998 to 2012. We also provide statistics on net employment creation and net R&D investments due to firm entry and exits. In addition, we compute the correlation coefficients between entry and exit rates at the two digit industry level so as to examine whether the correlations are contemporaneous or inter-temporal. Finally, we provide information about the underlying dataset to which secure access is available through UK Data Service Archive 7716 at http://dx.doi.org/10.5255/UKDA-SN-7716-1.

  6. Older drivers with cognitive impairment: Perceived changes in driving skills, driving-related discomfort and self-regulation of driving

    DEFF Research Database (Denmark)

    Meng, A.; Siren, A.; Teasdale, Thomas William

    2013-01-01

    The results of a previous study indicate that in general, older drivers who recognise cognitive problems show realistic self-assessment of changes in their driving skills and that driving-related discomfort may function as an indirect monitoring of driving ability, contributing to their safe...... drivers may recognise cognitive problems, they tend not to recognise changes to their driving, which may reflect reluctance to acknowledge the impact of cognitive impairment on their driving. Furthermore, the results suggest that driving-related discomfort plays an important role in the self......-regulation of driving among cognitively impaired older drivers. However, it is less clear what triggers driving-related discomfort among cognitively impaired older drivers indicating that it may be a less reliable aspect of their self-monitoring of driving ability....

  7. VCE-003.2, a novel cannabigerol derivative, enhances neuronal progenitor cell survival and alleviates symptomatology in murine models of Huntington's disease.

    Science.gov (United States)

    Díaz-Alonso, Javier; Paraíso-Luna, Juan; Navarrete, Carmen; Del Río, Carmen; Cantarero, Irene; Palomares, Belén; Aguareles, José; Fernández-Ruiz, Javier; Bellido, María Luz; Pollastro, Federica; Appendino, Giovanni; Calzado, Marco A; Galve-Roperh, Ismael; Muñoz, Eduardo

    2016-07-19

    Cannabinoids have shown to exert neuroprotective actions in animal models by acting at different targets including canonical cannabinoid receptors and PPARγ. We previously showed that VCE-003, a cannabigerol (CBG) quinone derivative, is a novel neuroprotective and anti-inflammatory cannabinoid acting through PPARγ. We have now generated a non-thiophilic VCE-003 derivative named VCE-003.2 that preserves the ability to activate PPARγ and analyzed its neuroprotective activity. This compound exerted a prosurvival action in progenitor cells during neuronal differentiation, which was prevented by a PPARγ antagonist, without affecting neural progenitor cell proliferation. In addition, VCE-003.2 attenuated quinolinic acid (QA)-induced cell death and caspase-3 activation and also reduced mutant huntingtin aggregates in striatal cells. The neuroprotective profile of VCE-003.2 was analyzed using in vivo models of striatal neurodegeneration induced by QA and 3-nitropropionic acid (3NP) administration. VCE-003.2 prevented medium spiny DARPP32(+) neuronal loss in these Huntington's-like disease mice models improving motor deficits, reactive astrogliosis and microglial activation. In the 3NP model VCE-003.2 inhibited the upregulation of proinflammatory markers and improved antioxidant defenses in the brain. These data lead us to consider VCE-003.2 to have high potential for the treatment of Huntington's disease (HD) and other neurodegenerative diseases with neuroinflammatory traits.

  8. Drive Stands

    Data.gov (United States)

    Federal Laboratory Consortium — The Electrical Systems Laboratory (ESL)houses numerous electrically driven drive stands. A drive stand consists of an electric motor driving a gearbox and a mounting...

  9. Do specialists exit the firm outsourcing its R&D?

    DEFF Research Database (Denmark)

    Wang, Wenjing

    Do specialists exit the firm increasingly outsourcing its research and development (R&D) work? Although this question is critical in understanding how R&D outsourcing links to innovation performance, the answer is not yet clear. This paper proposes that the optimal level of firm's internal...... employment of R&D specialists decreases with the deepening of R&D outsourcing but increases with the broadening of R&D outsourcing. These relations can be inferred from previous empirical studies as well as our theoretical analysis, and are supported by the empirical evidence from estimations of correlated...

  10. An item response theory analysis of the Executive Interview and development of the EXIT8: A Project FRONTIER Study.

    Science.gov (United States)

    Jahn, Danielle R; Dressel, Jeffrey A; Gavett, Brandon E; O'Bryant, Sid E

    2015-01-01

    The Executive Interview (EXIT25) is an effective measure of executive dysfunction, but may be inefficient due to the time it takes to complete 25 interview-based items. The current study aimed to examine psychometric properties of the EXIT25, with a specific focus on determining whether a briefer version of the measure could comprehensively assess executive dysfunction. The current study applied a graded response model (a type of item response theory model for polytomous categorical data) to identify items that were most closely related to the underlying construct of executive functioning and best discriminated between varying levels of executive functioning. Participants were 660 adults ages 40 to 96 years living in West Texas, who were recruited through an ongoing epidemiological study of rural health and aging, called Project FRONTIER. The EXIT25 was the primary measure examined. Participants also completed the Trail Making Test and Controlled Oral Word Association Test, among other measures, to examine the convergent validity of a brief form of the EXIT25. Eight items were identified that provided the majority of the information about the underlying construct of executive functioning; total scores on these items were associated with total scores on other measures of executive functioning and were able to differentiate between cognitively healthy, mildly cognitively impaired, and demented participants. In addition, cutoff scores were recommended based on sensitivity and specificity of scores. A brief, eight-item version of the EXIT25 may be an effective and efficient screening for executive dysfunction among older adults.

  11. Comparing Expert and Novice Driving Behavior in a Driving Simulator

    Directory of Open Access Journals (Sweden)

    Hiran B. Ekanayake

    2014-02-01

    Full Text Available This paper presents a study focused on comparing driving behavior of expert and novice drivers in a mid-range driving simulator with the intention of evaluating the validity of driving simulators for driver training. For the investigation, measurements of performance, psychophysiological measurements, and self-reported user experience under different conditions of driving tracks and driving sessions were analyzed. We calculated correlations between quantitative and qualitative measures to enhance the reliability of the findings. The experiment was conducted involving 14 experienced drivers and 17 novice drivers. The results indicate that driving behaviors of expert and novice drivers differ from each other in several ways but it heavily depends on the characteristics of the task. Moreover, our belief is that the analytical framework proposed in this paper can be used as a tool for selecting appropriate driving tasks as well as for evaluating driving performance in driving simulators.

  12. An Elimination of Resonance in Electric Drives

    Directory of Open Access Journals (Sweden)

    Michal Malek

    2011-01-01

    Full Text Available Flexible couplings together with resonance phenomenon are present mainly in the field of servodrives where high accuracy and dynamic requirements are crucial. When dynamics doesn’t correlate with mechanical system design, unwanted frequencies in the system are exited. Sometimes we haven’t conditions (whether material or space to design mechanical system with resonant frequencies too high to be exited. In that case we must choose compensating methods which can eliminate these phenomenons. This paper is dedicated to them.

  13. Cognitive problems, self-rated changes in driving skills, driving-related discomfort and self-regulation of driving in old drivers

    DEFF Research Database (Denmark)

    Meng, Annette; Siren, Anu Kristiina

    2012-01-01

    Ageing in general is associated with functional decline that may have an adverse effect on driving. Nevertheless, older drivers have been found to show good judgement and to self-regulate their driving, which may enable them to continue driving safely despite functional decline. The process...... of the self-monitoring of driving ability and the awareness of functional decline, and its association with the self-regulation of driving is, however, not fully understood. The aim of the present study was to examine the perceived changes in driving skills, the discomfort experienced in driving, and the self......-related discomfort is an important factor affecting the self-regulation of driving. Finally, the findings indicate that driving-related discomfort functions as an indirect self-monitoring of driving ability and may contribute to the safe driving performance of Danish older drivers....

  14. MO-E-BRB-04: Real-Time Exit-Fluence Delivery Validation

    Energy Technology Data Exchange (ETDEWEB)

    Siebers, J. [University of Virginia Health System (United States)

    2015-06-15

    Recent high profile reports of technical failures and human errors causing severe radiation- induced injuries and deaths come in support of the sustained efforts to ensure patient safety in the delivery of radiation treatments. In addition, highly conformal radiation therapies and escalated fraction doses mandate increased and sustained accuracy of the entire radiotherapy process. Consequently, and as a Result of AAPM and ASTRO led efforts patient specific quality assurance for specialized radiation treatments such as IMRT, SRS/SBRT and Arc Therapy had become a three-tier process: Pre-treatment, during treatment, and post treatment patient specific QA. Traditional patient QA consists of pre-treatment data transfer integrity dosimetric verifications and during-treatment geometric verifications. However, as treatment adaptation becomes closer to deployment in the clinics, during treatment validation via exit detectors had become a realistic QA option, permitting plan assessment in near real time. Post-treatment, machine logs allow comparisons of a range of mechanical parameters. A combination of these techniques could be used in evaluating inter-fraction, and intra-fraction delivery over a long time period such as an year, to evaluate the significant errors per site, per treatment technique. This type of data mining over longer periods of time provides the potential to recognize suboptimal radiation treatments, while allowing to identify systematic, possibly significant errors. This would allow creation of a data base of realized errors, small and large in dosimetry that could be for process or equipment improvement. This educational symposium will describe and review patient QA techniques, results, and strategies for patient specific quality assurance. Learning Objectives: review the goals of pre-treatment QA for various specialized procedures review methods and means for pre-treatment QA, limitations and tolerances review the scenarios where Varian/Tomo Log files

  15. MO-E-BRB-04: Real-Time Exit-Fluence Delivery Validation

    International Nuclear Information System (INIS)

    Siebers, J.

    2015-01-01

    Recent high profile reports of technical failures and human errors causing severe radiation- induced injuries and deaths come in support of the sustained efforts to ensure patient safety in the delivery of radiation treatments. In addition, highly conformal radiation therapies and escalated fraction doses mandate increased and sustained accuracy of the entire radiotherapy process. Consequently, and as a Result of AAPM and ASTRO led efforts patient specific quality assurance for specialized radiation treatments such as IMRT, SRS/SBRT and Arc Therapy had become a three-tier process: Pre-treatment, during treatment, and post treatment patient specific QA. Traditional patient QA consists of pre-treatment data transfer integrity dosimetric verifications and during-treatment geometric verifications. However, as treatment adaptation becomes closer to deployment in the clinics, during treatment validation via exit detectors had become a realistic QA option, permitting plan assessment in near real time. Post-treatment, machine logs allow comparisons of a range of mechanical parameters. A combination of these techniques could be used in evaluating inter-fraction, and intra-fraction delivery over a long time period such as an year, to evaluate the significant errors per site, per treatment technique. This type of data mining over longer periods of time provides the potential to recognize suboptimal radiation treatments, while allowing to identify systematic, possibly significant errors. This would allow creation of a data base of realized errors, small and large in dosimetry that could be for process or equipment improvement. This educational symposium will describe and review patient QA techniques, results, and strategies for patient specific quality assurance. Learning Objectives: review the goals of pre-treatment QA for various specialized procedures review methods and means for pre-treatment QA, limitations and tolerances review the scenarios where Varian/Tomo Log files

  16. A study on surveillance equipment at the exit/entry control point of nuclear facility

    International Nuclear Information System (INIS)

    Park, C. S.; Kim, D. Y.; Cha, H. L.; Kim, H. D.; Hong, J. S.

    1999-01-01

    Exit/Entry control is an essential measure at both entrances of the protected area and vital area of nuclear facility at which physical protection is required under the relevant laws and regulations. Especially, when there are heavy traffics of personnel and process equipment in those areas, automated surveillance devices have to be introduced to timely and efficiently screen out internal and external adversaries from achieving their goals of stealing of nuclear material and/or sabotage of the facility. The major portion of this study involves with integration and processing of signals from radiation detector, metal detector, and image monitor. This integrated device together with positive personal identification device which will be reinforced in near future would contribute to the establishment of total exit/entry control point of nuclear facility

  17. Embodied emotion impairment in Huntington's Disease.

    Science.gov (United States)

    Trinkler, Iris; Devignevielle, Sévérine; Achaibou, Amal; Ligneul, Romain V; Brugières, Pierre; Cleret de Langavant, Laurent; De Gelder, Beatrice; Scahill, Rachael; Schwartz, Sophie; Bachoud-Lévi, Anne-Catherine

    2017-07-01

    Theories of embodied cognition suggest that perceiving an emotion involves somatovisceral and motoric re-experiencing. Here we suggest taking such an embodied stance when looking at emotion processing deficits in patients with Huntington's Disease (HD), a neurodegenerative motor disorder. The literature on these patients' emotion recognition deficit has recently been enriched by some reports of impaired emotion expression. The goal of the study was to find out if expression deficits might be linked to a more motoric level of impairment. We used electromyography (EMG) to compare voluntary emotion expression from words to emotion imitation from static face images, and spontaneous emotion mimicry in 28 HD patients and 24 matched controls. For the latter two imitation conditions, an underlying emotion understanding is not imperative (even though performance might be helped by it). EMG measures were compared to emotion recognition and to the capacity to identify and describe emotions using alexithymia questionnaires. Alexithymia questionnaires tap into the more somato-visceral or interoceptive aspects of emotion perception. Furthermore, we correlated patients' expression and recognition scores to cerebral grey matter volume using voxel-based morphometry (VBM). EMG results replicated impaired voluntary emotion expression in HD. Critically, voluntary imitation and spontaneous mimicry were equally impaired and correlated with impaired recognition. By contrast, alexithymia scores were normal, suggesting that emotion representations on the level of internal experience might be spared. Recognition correlated with brain volume in the caudate as well as in areas previously associated with shared action representations, namely somatosensory, posterior parietal, posterior superior temporal sulcus (pSTS) and subcentral sulcus. Together, these findings indicate that in these patients emotion deficits might be tied to the "motoric level" of emotion expression. Such a double

  18. Results of the radiological survey at Exit 4, Interstate 90, Albany, New York (AL212)

    International Nuclear Information System (INIS)

    Marley, J.L.; Carrier, R.F.

    1988-02-01

    A number of properties in the Albany/Colonie area have been identified as being potentially contaminated with uranium originating from the former National Lead Company's uranium forming plant in Colonie, New York. The property at Exit 4 between Yardboro Avenue and Interstate 90 in Albany, New York was the subject of a radiological investigation initiated April 27, 1987. The area of survey of the property of the State of New York was the north side of the right-of-way of Interstate 90 (I-90) at Exit 4 (the Slingerlands exit) from the fence along the south side of Yardboro Avenue to the berm of the west-bound lanes of I-90. The survey, starting at the I-90 bridge over Central Avenue continued west for approximately 570 m. The width of the area ranged from /approximately/10 m to 195 m. To perform the survey, a network of sectors forming a grid was established, beginning with a series of basing points measured at /approximately/23-m intervals at the fence along Yardboro Avenue. Sectors were set by projection of lines from the basing points, perpendicular to the fence, to the berm of I-90. Then parallel lines at 23-m intervals were set. 12 refs., 6 figs., 4 tabs

  19. The Effects of the Specialization of Private Equity Firms on their Exit Strategy

    DEFF Research Database (Denmark)

    Rigamonti, Damiana; Cefis, Elena; Meoli, Michele

    2016-01-01

    We investigate how industry and stage specialization of Private Equity (PE) firms affect the likelihood to exit investments by means of trade sales, Initial Public Offerings (IPOs), or Secondary Buyouts (SBOs). Our empirical analysis relies on competing risks models. Using a sample of 818 Leverag...

  20. Evidence for a modifier of onset age in Huntington disease linked to the HD gene in 4p16

    Science.gov (United States)

    Djoussé, Luc; Knowlton, Beth; Hayden, Michael R.; Almqvist, Elisabeth W.; Brinkman, Ryan R.; Ross, Christopher A.; Margolis, Russel L.; Rosenblatt, Adam; Durr, Alexandra; Dode, Catherine; Morrison, Patrick J.; Novelletto, Andrea; Frontali, Marina; Trent, Ronald J. A.; McCusker, Elizabeth; Gómez-Tortosa, Estrella; Mayo Cabrero, David; Jones, Randi; Zanko, Andrea; Nance, Martha; Abramson, Ruth K.; Suchowersky, Oksana; Paulsen, Jane S.; Harrison, Madaline B.; Yang, Qiong; Cupples, L. Adrienne; Mysore, Jayalakshmi; Gusella, James F.; MacDonald, Marcy E.

    2007-01-01

    Huntington disease (HD) is a neurodegenerative disorder caused by the abnormal expansion of CAG repeats in the HD gene on chromosome 4p16.3. A recent genome scan for genetic modifiers of age at onset of motor symptoms (AO) in HD suggests that one modifier may reside in the region close to the HD gene itself. We used data from 535 HD participants of the New England Huntington cohort and the HD MAPS cohort to assess whether AO was influenced by any of the three markers in the 4p16 region: MSX1 (Drosophila homeo box homologue 1, formerly known as homeo box 7, HOX7), Δ2642 (within the HD coding sequence), and BJ56 (D4S127). Suggestive evidence for an association was seen between MSX1 alleles and AO, after adjustment for normal CAG repeat, expanded repeat, and their product term (model P value 0.079). Of the variance of AO that was not accounted for by HD and normal CAG repeats, 0.8% could be attributed to the MSX1 genotype. Individuals with MSX1 genotype 3/3 tended to have younger AO. No association was found between Δ2642 (P=0.44) and BJ56 (P=0.73) and AO. This study supports previous studies suggesting that there may be a significant genetic modifier for AO in HD in the 4p16 region. Furthermore, the modifier may be present on both HD and normal chromosomes bearing the 3 allele of the MSX1 marker. PMID:15029481

  1. Disruption of astrocyte-neuron cholesterol cross talk affects neuronal function in Huntington's disease.

    Science.gov (United States)

    Valenza, M; Marullo, M; Di Paolo, E; Cesana, E; Zuccato, C; Biella, G; Cattaneo, E

    2015-04-01

    In the adult brain, neurons require local cholesterol production, which is supplied by astrocytes through apoE-containing lipoproteins. In Huntington's disease (HD), such cholesterol biosynthesis in the brain is severely reduced. Here we show that this defect, occurring in astrocytes, is detrimental for HD neurons. Astrocytes bearing the huntingtin protein containing increasing CAG repeats secreted less apoE-lipoprotein-bound cholesterol in the medium. Conditioned media from HD astrocytes and lipoprotein-depleted conditioned media from wild-type (wt) astrocytes were equally detrimental in a neurite outgrowth assay and did not support synaptic activity in HD neurons, compared with conditions of cholesterol supplementation or conditioned media from wt astrocytes. Molecular perturbation of cholesterol biosynthesis and efflux in astrocytes caused similarly altered astrocyte-neuron cross talk, whereas enhancement of glial SREBP2 and ABCA1 function reversed the aspects of neuronal dysfunction in HD. These findings indicate that astrocyte-mediated cholesterol homeostasis could be a potential therapeutic target to ameliorate neuronal dysfunction in HD.

  2. The Influence of Psychographic Variables on the Theory of Exit, Voice, and Loyalty of Customer Complaints Behaviour in Banks

    OpenAIRE

    Preko Alexander; Agbanu Kwami Samuel

    2015-01-01

    This paper analyses Customer Complaints Behaviour (CCB) using psychographic factors and the Theory of Exit, Voice and Loyalty. Prior studies on the continent of Africa have explored customer dissatisfaction, service failure, complaints handling and complaint attitudes, but not in association with psychographic factors. This research gap is addressed with a new conceptual understanding that integrates psychographic variables and the Theory of Exit, Voice and Loyalty in a single ...

  3. R2 & NE: NAVTEQ 2011 Q3 Exit Points for the United States, including Puerto Rico and the US Virgin Islands in SDC Format

    Data.gov (United States)

    U.S. Environmental Protection Agency — The EXITS layer contains point representations of the locations of exits, typically on controlled access roads such as motorways and freeways. Controlled access...

  4. D-β-hydroxybutyrate is protective in mouse models of Huntington's disease.

    Directory of Open Access Journals (Sweden)

    Soyeon Lim

    Full Text Available Abnormalities in mitochondrial function and epigenetic regulation are thought to be instrumental in Huntington's disease (HD, a fatal genetic disorder caused by an expanded polyglutamine track in the protein huntingtin. Given the lack of effective therapies for HD, we sought to assess the neuroprotective properties of the mitochondrial energizing ketone body, D-β-hydroxybutyrate (DβHB, in the 3-nitropropionic acid (3-NP toxic and the R6/2 genetic model of HD. In mice treated with 3-NP, a complex II inhibitor, infusion of DβHB attenuates motor deficits, striatal lesions, and microgliosis in this model of toxin induced-striatal neurodegeneration. In transgenic R6/2 mice, infusion of DβHB extends life span, attenuates motor deficits, and prevents striatal histone deacetylation. In PC12 cells with inducible expression of mutant huntingtin protein, we further demonstrate that DβHB prevents histone deacetylation via a mechanism independent of its mitochondrial effects and independent of histone deacetylase inhibition. These pre-clinical findings suggest that by simultaneously targeting the mitochondrial and the epigenetic abnormalities associated with mutant huntingtin, DβHB may be a valuable therapeutic agent for HD.

  5. Diffusion tensor imaging of brain white matter in Huntington gene mutation individuals

    Directory of Open Access Journals (Sweden)

    Roberta Arb Saba

    Full Text Available ABSTRACT Objective To evaluate the role of the involvement of white matter tracts in huntingtin gene mutation patients as a potential biomarker of the progression of the disease. Methods We evaluated 34 participants (11 symptomatic huntingtin gene mutation, 12 presymptomatic huntingtin gene mutation, and 11 controls. We performed brain magnetic resonance imaging to assess white matter integrity using diffusion tensor imaging, with measurement of fractional anisotropy. Results We observed a significant decrease of fractional anisotropy in the cortical spinal tracts, corona radiate, corpus callosum, external capsule, thalamic radiations, superior and inferior longitudinal fasciculus, and inferior frontal-occipital fasciculus in the Huntington disease group compared to the control and presymptomatic groups. Reduction of fractional anisotropy is indicative of a degenerative process and axonal loss. There was no statistically significant difference between the presymptomatic and control groups. Conclusion White matter integrity is affected in huntingtin gene mutation symptomatic individuals, but other studies with larger samples are required to assess its usefulness in the progression of the neurodegenerative process.

  6. DrivingSense: Dangerous Driving Behavior Identification Based on Smartphone Autocalibration

    Directory of Open Access Journals (Sweden)

    Chunmei Ma

    2017-01-01

    Full Text Available Since pervasive smartphones own advanced computing capability and are equipped with various sensors, they have been used for dangerous driving behaviors detection, such as drunk driving. However, sensory data gathered by smartphones are noisy, which results in inaccurate driving behaviors estimations. Some existing works try to filter noise from sensor readings, but usually only the outlier data are filtered. The noises caused by hardware of the smartphone cannot be removed from the sensor reading. In this paper, we propose DrivingSense, a reliable dangerous driving behavior identification scheme based on smartphone autocalibration. We first theoretically analyze the impact of the sensor error on the vehicle driving behavior estimation. Then, we propose a smartphone autocalibration algorithm based on sensor noise distribution determination when a vehicle is being driven. DrivingSense leverages the corrected sensor parameters to identify three kinds of dangerous behaviors: speeding, irregular driving direction change, and abnormal speed control. We evaluate the effectiveness of our scheme under realistic environments. The results show that DrivingSense, on average, is able to detect the driving direction change event and abnormal speed control event with 93.95% precision and 90.54% recall, respectively. In addition, the speed estimation error is less than 2.1 m/s, which is an acceptable range.

  7. The Impact of High School Exit Exams on Graduation Rates and Achievement

    Science.gov (United States)

    Caves, Katherine; Balestra, Simone

    2018-01-01

    The authors examined the short- and long-term effects of high school exit exams (HSEEs) on graduation rates and achievement using an interrupted time series approach. There is a positive overall effect of HSEE introduction for graduation rate trends, which is heterogeneous over time. HSEEs have a negative impact on graduation rates in the year of…

  8. High School Exit Exams and Dropout in an Era of Increased Accountability

    Science.gov (United States)

    Hemelt, Steven W.; Marcotte, Dave E.

    2013-01-01

    A key form of student-level accountability is the requirement for students to pass high school exit exams (HSEEs) in order to receive a diploma. In this paper, we examine the impact of HSEEs on dropout during a period when these exams became more common and rigorous. Further, we study whether offering alternate pathways to graduation for students…

  9. Real-time multi-function entry / exit management system

    International Nuclear Information System (INIS)

    Hiyama, Kazuhisa; Kurosawa, Akihiko; Asano, Norikazu; Onoue, Ryuji; Eguchi, Shohei; Hanawa, Nobuhiro; Hori, Naohiko; Ueda, Hisao; Kanda, Hiroaki

    2012-01-01

    In order to prevent radiation accident and its expansion, more integrated management system is required to safety management for radiation workers in the nuclear facilities. Therefore, JAEA (Japan Atomic Energy Agency) and HAM (Hitachi Aloka Medical, Ltd) have developed innovative real-time multi-function entry/exit management system which managed worker's exposed dose and position under the joint developed patent. This system is sharing worker's data among workers and server manager who is inside of or outside of building, such as worker's positing, health condition and exposed dose. It consists of mobile equipments, receivers, LAN, and servers system. This report summarizes the system to be installed in the JMTR. (author)

  10. Driving Style Analysis Using Primitive Driving Patterns With Bayesian Nonparametric Approaches

    OpenAIRE

    Wang, Wenshuo; Xi, Junqiang; Zhao, Ding

    2017-01-01

    Analysis and recognition of driving styles are profoundly important to intelligent transportation and vehicle calibration. This paper presents a novel driving style analysis framework using the primitive driving patterns learned from naturalistic driving data. In order to achieve this, first, a Bayesian nonparametric learning method based on a hidden semi-Markov model (HSMM) is introduced to extract primitive driving patterns from time series driving data without prior knowledge of the number...

  11. Embedded system design to control the entry and exit of vehicles online, at the main access of ESPOCH

    Directory of Open Access Journals (Sweden)

    Javier J. Gavilanes

    2018-02-01

    Full Text Available In this research a embedded real-time system was developed by using Raspberry Pi3 (a reduced board computer, which is an equipment with a camera placed in strategic points of the mechanic arms at the main entrance and exit of Escuela Superior Politécnica de Chimborazo, this equipment captures images of vehicles that enter and exit the campus and the information is extracted through the implementation of a segmentation algorithm written in Python programming language and the collaboration of artificial vision bookstores offered by OpenCV, processing techniques were applied to extract the vehicle plate from the location scenery. Then, an Optical Character Recognition (OCR algorithm also known as K-Nearest Neighbours (KNN was applied, which after a training phase is able to identify letters and numbers on the automobile plates, the information is stored in the entrance database and it is deleted when the automobile exits the campus.

  12. A time-compressed simulated geomagnetic storm influences the nest-exiting flight angles of the stingless bee Tetragonisca angustula

    Science.gov (United States)

    Esquivel, D. M. S.; Corrêa, A. A. C.; Vaillant, O. S.; de Melo, V. Bandeira; Gouvêa, G. S.; Ferreira, C. G.; Ferreira, T. A.; Wajnberg, E.

    2014-03-01

    Insects have been used as models for understanding animal orientation. It is well accepted that social insects such as honeybees and ants use different natural cues in their orientation mechanism. A magnetic sensitivity was suggested for the stingless bee Schwarziana quadripunctata, based on the observation of a surprising effect of a geomagnetic storm on the nest-exiting flight angles. Stimulated by this result, in this paper, the effects of a time-compressed simulated geomagnetic storm (TC-SGS) on the nest-exiting flight angles of another stingless bee, Tetragonisca angustula, are presented. Under an applied SGS, either on the horizontal or vertical component of the geomagnetic field, both nest-exiting flight angles, dip and azimuth, are statistically different from those under geomagnetic conditions. The angular dependence of ferromagnetic resonance (FMR) spectra of whole stingless bees shows the presence of organized magnetic nanoparticles in their bodies, which indicates this material as a possible magnetic detector.

  13. PPARGC1A/PGC-1α, TFEB and enhanced proteostasis in Huntington disease: Defining regulatory linkages between energy production and protein–organelle quality control

    OpenAIRE

    La Spada, Albert R.

    2012-01-01

    Huntington disease (HD) results from CAG repeats that encode expanded polyglutamine tracts in the HTT/huntingtin protein. HD belongs to a large category of inherited and sporadic neurodegenerative disorders in which production of a misfolded protein initiates the pathogenic cascade. Previous studies have shown that misfolded proteins become resistant to cellular protein turnover pathways by eluding and disabling the ubiquitin-proteasome system (UPS) and autophagy-lysosome pathway. Based upon ...

  14. Journal of Biosciences | Indian Academy of Sciences

    Indian Academy of Sciences (India)

    Entsar Saheb1 2 Wendy Trzyna3 John Bush1. Biology Department, University of Arkansas at Little Rock, 2801 South University Ave., Little Rock, AR 72204-1099, USA; Biology Department, University of Baghdad, Baghdad, Iraq; Department of Biological Sciences, Marshall University, One John Marshall Drive, Huntington, ...

  15. Control rod drive

    International Nuclear Information System (INIS)

    Okutani, Tetsuro.

    1988-01-01

    Purpose: To provide a simple and economical control rod drive using a control circuit requiring no pulse circuit. Constitution: Control rods in a BWR type reactor are driven by hydraulic pressure and inserted or withdrawn in the direction of applying the hydraulic pressure. The direction of the hydraulic pressure is controlled by a direction control valve. Since the driving for the control rod is extremely important in view of the operation, a self diagnosis function is disposed for rapid inspection of possible abnormality. In the present invention, two driving contacts are disposed each by one between the both ends of a solenoid valve of the direction control valve for driving the control rod and the driving power source, and diagnosis is conducted by alternately operating them. Therefore, since it is only necessary that the control circuit issues a driving instruction only to one of the two driving contacts, the pulse circuit is no more required. Further, since the control rod driving is conducted upon alignment of the two driving instructions, the reliability of the control rod drive can be improved. (Horiuchi, T.)

  16. UC3M Emergencies : health center search, first aid, defibrillators, emergency exits, last warnings and torch (iOS platform)

    OpenAIRE

    Martín Civiac, María

    2013-01-01

    This document contains Maria Martin’s bachelor thesis. The issues that this project wants to solve are emergencies-related: health centersand emergency exits location,emergency call, first aid instructions and information and latest emergency alerts at Carlos III University. It has been detected that members of Carlos III University currently have difficulties to find health centers, emergency exits and first aid information. Furthermore, the University itself did not have any mechanism to pu...

  17. Assessment of the exit defects in carbon fibre-reinforced plastic plates caused by drilling

    Energy Technology Data Exchange (ETDEWEB)

    Zhang Houjiang; Zhang Liangchi [Sydney Univ. (Australia). Dept. of Mechanical and Mechatronic Engineering; Chen Wuyi; Chen Dingchang [Beijing Univ. of Aeronautics and Astronautics, BJ (China). Dept. of Manufacturing Engineering

    2001-07-01

    This paper investigates the formation of the exit defects in carbon fibre-reinforced plates and characterizes their features in terms of drilling conditions. It was found that spalling and fuzzing are the major mechanisms of exit defects. The spalling, consisting of a main region and a secondary region, is caused by chisel and cutting edge actions, in which the former plays a key role. The fuzzing, however, exists in the cutting region where the included angle between the fibre direction of the surface layer and that of the cutting speed is acute. A severer spalling damage corresponds to a high spindle speed, a large feed rate and a great thrust force. Some empirical relationships, including a dimensionless formula, were developed for assessing the characteristic dimension of the spalling damage based on the known drilling conditions. (orig.)

  18. Dimensions of driving anger and their relationships with aberrant driving.

    Science.gov (United States)

    Zhang, Tingru; Chan, Alan H S; Zhang, Wei

    2015-08-01

    The purpose of this study was to investigate the relationship between driving anger and aberrant driving behaviours. An internet-based questionnaire survey was administered to a sample of Chinese drivers, with driving anger measured by a 14-item short Driving Anger Scale (DAS) and the aberrant driving behaviours measured by a 23-item Driver Behaviour Questionnaire (DBQ). The results of Confirmatory Factor Analysis demonstrated that the three-factor model (hostile gesture, arrival-blocking and safety-blocking) of the DAS fitted the driving anger data well. The Exploratory Factor Analysis on DBQ data differentiated four types of aberrant driving, viz. emotional violation, error, deliberate violation and maintaining progress violation. For the anger-aberration relation, it was found that only "arrival-blocking" anger was a significant positive predictor for all four types of aberrant driving behaviours. The "safety-blocking" anger revealed a negative impact on deliberate violations, a finding different from previously established positive anger-aberration relation. These results suggest that drivers with different patterns of driving anger would show different behavioural tendencies and as a result intervention strategies may be differentially effective for drivers of different profiles. Copyright © 2015 Elsevier Ltd. All rights reserved.

  19. Age at onset in Huntington's disease: replication study on the association of HAP1.

    Science.gov (United States)

    Karadima, Georgia; Dimovasili, Christina; Koutsis, Georgios; Vassilopoulos, Demetris; Panas, Marios

    2012-11-01

    In recent years two association studies investigating the HAP1 T441M (rs4523977) polymorphism as a potential modifying factor of the age at onset (AAO) of Huntington's disease (HD), have been reported. Initially evidence for association was found between the M441 risk allele and the AAO. Subsequently, a second study, although failing to replicate these findings, found evidence for association between the same risk allele and AAO of motor symptoms (mAAO). In the present study, the role of the HAP1 T441M polymorphism as a modifier of the AAO in HD was investigated in a cohort of 298 Greek HD patients. In this cohort the CAG repeat number accounted for 55% of the variance in AAO. No association was found between the HAP1 T441M polymorphism and the AAO of HD. © 2012 Elsevier Ltd. All rights reserved.

  20. Optimization of exit-plane waves restored from HRTEM through-focal series

    International Nuclear Information System (INIS)

    Erni, Rolf; Rossell, Marta D.; Nakashima, Philip N.H.

    2010-01-01

    Atomic-resolution transmission electron microscopy has largely benefited from the implementation of aberration correctors in the imaging part of the microscope. Though the dominant geometrical axial aberrations can in principle be corrected or suitably adjusted, the impact of higher-order aberrations, which are mainly due to the implementation of non-round electron optical elements, on the imaging process remains unclear. Based on a semi-empirical criterion, we analyze the impact of residual aperture aberrations on the quality of exit-plane waves that are retrieved from through-focal series recorded using an aberration-corrected and monochromated instrument which was operated at 300 kV and enabled for an information transfer of ∼0.05 nm. We show that the impact of some of the higher-order aberrations in retrieved exit-plane waves can be balanced by a suitable adjustment of symmetry equivalent lower-order aberrations. We find that proper compensation and correction of 1st and 2nd order aberrations is critical, and that the required accuracy is difficult to achieve. This results in an apparent insensitivity towards residual higher-order aberrations. We also investigate the influence of the detector characteristics on the image contrast. We find that correction for the modulation transfer function results in a contrast gain of up to 40%.