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...

Learning about Huntington's Disease

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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 ...

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.

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...

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.

Clinical and genetic study of a juvenile-onset Huntington disease

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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

Investigation of the subsurface features of the basement complex of ...

African Journals Online (AJOL)

3D seismic reflection survey was recently carried out within the Zaria area of the basement complex of northern Nigeria, in order to investigate the complexity of the subsurface features within the basement. The geology of the survey area was characterized by gneisses and low grade meta-sedimentary rocks that form the ...

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.

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...

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.

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....

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.

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

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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.

Major Superficial White Matter Abnormalities in Huntington's Disease

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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

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

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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.

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...

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

The Lusi eruption site: insights from surface and subsurface investigations

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Mazzini, A.

2017-12-01

The Indonesian Lusi eruption has been spewing boiling water, gas, and sediments since the 29th of May 2006. Initially, numerous aligned eruptions sites appeared along the Watukosek fault system (WFS) that was reactivated after the Yogyakarta earthquake occurring the 27th of May in the Java Island. Within weeks several villages were submerged by boiling mud. The most prominent eruption site was named Lusi. To date Lusi is still active and an area of 7 km2is covered by mud. Since its birth Lusi erupted with a pulsating behaviour. In the framework of the ERC grant "Lusi Lab" we conducted several years of monitoring and regional investigations coupling surface sampling and subsurface imaging in the region around Lusi. Ambient noise tomography studies, obtained with a local network of 31 stations, revealed for the first time subsurface images of the Lusi region and the adjacent Arjuno-Welirang (AW) volcanic complex. Results show that below the AW volcanic complex are present 5km deep magma chambers that are connected, through a defined corridor, with the roots of the Lusi eruption site. The Lusi subsurface shows the presence of a defined vertical hydrothermal plume that extends to at least 5km. Chemical analyses of the seeping fluids sampled from 1) the Lusi plume (using a specifically designed drone), 2) the region around Lusi, and 3) the fumaroles and the hydro thermal springs of AW, revealed striking similarities. More specifically a mantellic signature of the Lusi fluids confirms the scenario that Lusi represents a magmatic-driven hydrothermal system hosted in sedimentary basin. Seismic profiles interpretation, surface mapping, and fluid sampling show that the WFS, connecting AW and extending towards the NE of Java, acted as a preferential pathway for the igneous intrusion and fluids migration towards the subsurface. Petrography and dating of the clasts erupted at Lusi record high temperatures and indicate that the roots of the active conduit extend to at least 5km

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

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

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 ...

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...

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...

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

A technical investigation on tools and concepts for sustainable management of the subsurface in The Netherlands.

Science.gov (United States)

Griffioen, Jasper; van Wensem, Joke; Oomes, Justine L M; Barends, Frans; Breunese, Jaap; Bruining, Hans; Olsthoorn, Theo; Stams, Alfons J M; van der Stoel, Almer E C

2014-07-01

In response to increasing use of the subsurface, there is a need to modernise policies on sustainable use of the subsurface. This holds in particular for the densely populated Netherlands. We aimed to analyse current practice of subsurface management and the associated pressure points and to establish a conceptual overview of the technical issues related to sustainable management of the subsurface. Case studies on the exploitation of subsurface resources (including spatial use of the subsurface) were analysed, examining social relevance, environmental impact, pressure points and management solutions. The case studies ranged from constructing underground garages to geothermal exploitation. The following issues were identified for the technological/scientific aspects: site investigation, suitability, risk assessment, monitoring and measures in the event of failure. Additionally, the following general issues were identified for the administrative aspects: spatial planning, option assessment, precaution, transparency, responsibility and liability. These issues were explored on their technological implications within the framework of sustainable management of the subsurface. This resulted into the following key aspects: (1) sustainability assessment, (2) dealing with uncertainty and (3) policy instruments and governance. For all three aspects, different options were identified which might have a legal, economic or ethical background. The technological implications of these backgrounds have been identified. A set of recommendations for sustainable management of the subsurface resources (incl. space) was established: (1) management should be driven by scarcity, (2) always implement closed loop monitoring when the subsurface activities are high-risk, (3) when dealing with unknown features and heterogeneity, apply the precautionary principle, (4) responsibility and liability for damage must be set out in legislation and (5) sustainability should be incorporated in all

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

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....

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

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.

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,

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

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.

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

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.

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.

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.

Microbial activity in the terrestrial subsurface

International Nuclear Information System (INIS)

Kaiser, J.P.; Bollag, J.M.

1990-01-01

Little is known about the layers under the earth's crust. Only in recent years have techniques for sampling the deeper subsurface been developed to permit investigation of the subsurface environment. Prevailing conditions in the subsurface habitat such as nutrient availability, soil composition, redox potential, permeability and a variety of other factors can influence the microflora that flourish in a given environment. Microbial diversity varies between geological formations, but in general sandy soils support growth better than soils rich in clay. Bacteria predominate in subsurface sediments, while eukaryotes constitute only 1-2% of the microorganisms. Recent investigations revealed that most uncontaminated subsurface soils support the growth of aerobic heteroorganotrophic bacteria, but obviously anaerobic microorganisms also exist in the deeper subsurface habitat. The microorganisms residing below the surface of the earth are capable of degrading both natural and xenobiotic contaminants and can thereby adapt to growth under polluted conditions. (author) 4 tabs, 77 refs

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

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...

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

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.

ESTIMATION OF NEAR SUBSURFACE COAL FIRE GAS EMISSIONS BASED ON GEOPHYSICAL INVESTIGATIONS

Science.gov (United States)

Chen-Brauchler, D.; Meyer, U.; Schlömer, S.; Kus, J.; Gundelach, V.; Wuttke, M.; Fischer, C.; Rueter, H.

2009-12-01

Spontaneous and industrially caused subsurface coal fires are worldwide disasters that destroy coal resources, cause air pollution and emit a large amount of green house gases. Especially in developing countries, such as China, India and Malaysia, this problem has intensified over the last 15 years. In China alone, 10 to 20 million tons of coal are believed to be lost in uncontrolled coal fires. The cooperation of developing countries and industrialized countries is needed to enforce internationally concerted approaches and political attention towards the problem. The Clean Development Mechanism (CDM) under the framework of the Kyoto Protocol may provide an international stage for financial investment needed to fight the disastrous situation. A Sino-German research project for coal fire exploration, monitoring and extinction applied several geophysical approaches in order to estimate the annual baseline especially of CO2 emissions from near subsurface coal fires. As a result of this project, we present verifiable methodologies that may be used in the CDM framework to estimate the amount of CO2 emissions from near subsurface coal fires. We developed three possibilities to approach the estimation based on (1) thermal energy release, (2) geological and geometrical determinations as well as (3) direct gas measurement. The studies involve the investigation of the physical property changes of the coal seam and bedrock during different burning stages of a underground coal fire. Various geophysical monitoring methods were applied from near surface to determine the coal volume, fire propagation, temperature anomalies, etc.

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

[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

Theoretical and experimental investigations of ferrofluids for guiding and detecting liquids in the subsurface. FY 1997 annual report

Energy Technology Data Exchange (ETDEWEB)

Moridis, G.J.; Borglin, S.E.; Oldenburg, C.M.; Becker, A.

1998-03-01

Ferrofluids are stable colloidal suspensions of magnetic particles in various carrier liquids with high saturation magnetizations, which can be manipulated in virtually any fashion, defying gravitational or viscous forces in response to external magnetic fields. In this report, the authors review the results of their investigation of the potential of ferrofluids (1) to accurately and effectively guide reactants (for in-situ treatment) or barrier liquids (low-viscosity permeation grouts) to contaminated target zones in the subsurface using electromagnetic forces, and (2) to trace the movement and position of liquids injected in the subsurface using geophysical methods. They investigate the use of ferrofluids to enhance the efficiency of in-situ treatment and waste containment through (a) accurate guidance and delivery of reagent liquids to the desired subsurface contamination targets and/or (b) effective sweeping of the contaminated zone as ferrofluids move from the application point to an attracting magnet/collection point. They also investigate exploiting the strong magnetic signature of ferrofluids to develop a method for monitoring of liquid movement and position during injection using electromagnetic methods. The authors demonstrated the ability to induce ferrofluid movement in response to a magnetic field, and measured the corresponding magnetopressure. They demonstrated the feasibility of using conventional magnetometry for detecting subsurface zones of various shapes containing ferrofluids for tracing liquids injected for remediation or barrier formation. Experiments involving spherical, cylindrical and horizontal slabs showed a very good agreement between predictions and measurements.

Theoretical and experimental investigations of ferrofluids for guiding and detecting liquids in the subsurface. FY 1997 annual report

International Nuclear Information System (INIS)

Moridis, G.J.; Borglin, S.E.; Oldenburg, C.M.; Becker, A.

1998-03-01

Ferrofluids are stable colloidal suspensions of magnetic particles in various carrier liquids with high saturation magnetizations, which can be manipulated in virtually any fashion, defying gravitational or viscous forces in response to external magnetic fields. In this report, the authors review the results of their investigation of the potential of ferrofluids (1) to accurately and effectively guide reactants (for in-situ treatment) or barrier liquids (low-viscosity permeation grouts) to contaminated target zones in the subsurface using electromagnetic forces, and (2) to trace the movement and position of liquids injected in the subsurface using geophysical methods. They investigate the use of ferrofluids to enhance the efficiency of in-situ treatment and waste containment through (a) accurate guidance and delivery of reagent liquids to the desired subsurface contamination targets and/or (b) effective sweeping of the contaminated zone as ferrofluids move from the application point to an attracting magnet/collection point. They also investigate exploiting the strong magnetic signature of ferrofluids to develop a method for monitoring of liquid movement and position during injection using electromagnetic methods. The authors demonstrated the ability to induce ferrofluid movement in response to a magnetic field, and measured the corresponding magnetopressure. They demonstrated the feasibility of using conventional magnetometry for detecting subsurface zones of various shapes containing ferrofluids for tracing liquids injected for remediation or barrier formation. Experiments involving spherical, cylindrical and horizontal slabs showed a very good agreement between predictions and measurements

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.

Investigation of the subsurface environment at the Idaho National Engineering Laboratory Radioactive Waste Management Complex

International Nuclear Information System (INIS)

Russell, B.F.; Mizell, S.A.; Hull, L.C.; Smith, T.H.; Lewis, B.D.; Barraclough, J.T.; Humphrey, T.G.

1984-01-01

A comprehensive, 10-year plan to investigate radionuclide migration in the subsurface at the Radioactive Waste Management Complex (RWMC) has been prepared and initiated (in FY-84). The RWMC Subsurface Investigation is designed to address two objectives set forth by the DOE Idaho Operations Office: (1) determine the extent of radionuclide migration, if any, from the buried waste, and (2) develop and calibrate a computer model to simulate long-term radionuclide migration. At the RWMC, the Snake River Plain Aquifer underlies about 177 m of partially saturated, fractured basalts and thin sedimentary units. Three sedimentary units, accounting for no more than 20 m of the partially saturated thickness, appear to be continuous throughout the area. Thinner sedimentary units are discontinuous. Low-level waste and (prior to 1970) transuranic waste have been buried in the surficial sediments at the RWMC. The first burials took place in 1952. Due to the complicated disposal system, a comprehensive review of state-of-the-art vadose zone monitoring instrumentation and techniques, an analysis of conceptual migration pathways, and an evaluation of potential hazard from buried radionuclides were conducted to guide preparation of the investigation plan. The plan includes an overview of the RWMC facility, subsurface work conducted to date at the RWMC and other DOE laboratory facilities, an evaluation and selection of the methods and studies to be used, a radionuclide hazard evaluation, a cost analysis, and external peer review results. In addition, an Appendix contains the details for each method/study to be employed. 4 references, 5 figures, 1 table

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

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.

Time-Lapse Electrical Resistivity Investigations for Imaging the Grouting Injection in Shallow Subsurface Cavities

Directory of Open Access Journals (Sweden)

Muhammad Farooq

2014-01-01

Full Text Available The highway of Yongweol-ri, Muan-gun, south-western part of the South Korean Peninsula, is underlain by the abandoned of subsurface cavities, which were discovered in 2005. These cavities lie at shallow depths with the range of 5∼15 meters below the ground surface. Numerous subsidence events have repeatedly occurred in the past few years, damaging infrastructure and highway. As a result of continuing subsidence issues, the Korean Institute of Geosciences and Mineral Resources (KIGAM was requested by local administration to resolve the issue. The KIGAM used geophysical methods to delineate subsurface cavities and improve more refined understanding of the cavities network in the study area. Cement based grouting has been widely employed in the construction industry to reinforce subsurface ground. In this research work, time-lapse electrical resistivity surveys were accomplished to monitor the grouting injection in the subsurface cavities beneath the highway, which have provided a quasi-real-time monitoring for modifying the subsurface cavities related to ground reinforcement, which would be difficult with direct methods. The results obtained from time-lapse electrical resistivity technique have satisfactory imaged the grouting injection experiment in the subsurface cavities beneath the highway. Furthermore, the borehole camera confirmed the presence of grouting material in the subsurface cavities, and hence this procedure increases the mechanical resistance of subsurface cavities below the highway.

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...

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...

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.

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.

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...

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...

Investigation of subsurface fatigue crack in PEEK shaft under one-point rolling contact by using 2.5D layer observation method

Directory of Open Access Journals (Sweden)

KOIKE Hitonobu

2017-01-01

Full Text Available Subsurface fatigue cracks under rolling contact area of the PEEK shaft against an alumina bearing’s ball were investigated for application of frictional part in mechanical element in special situations such as chemical environments. In order to explore the flaking process of the PEEK shaft, the rolling contact fatigue tests were carried out by using a one-point radial loading rolling contact machine. The flaking occurred on the rolling track of the PEEK shaft at approximate 4⨉105 fatigue cycles. The subsurface fatigue crack propagation was investigated by using 2.5-Dimension layer observation method. The flaking was caused by the propagations of surface cracks and subsurface shear cracks, and the flaking shape was half-ellipse. Moreover, beach marks as fatigue crack propagation in the flaking were observed.

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...

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.

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.

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

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.

[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.

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.

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.

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...

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 ...

Hydraulic characterization of rocky subsurface using field infiltrometer measurements coupled with hydrogeophysical investigations

Science.gov (United States)

Caputo, M. C.; de Carlo, L.; de Benedictis, F.; Vurro, M.

2009-04-01

The shallow and/or karstic and fractured aquifers are among the most important water resources. At the same time, they are particularly vulnerable to contamination. A detailed scientific knowledge of the behavior of these aquifers is essential for the development of sustainable groundwater management. Different investigation methods have been developed with the aim to characterize the subsurface and to monitor the flow and solute transport in these hydrogeology systems. This study presents the results of an investigation method, that combine large infiltrometer measurements with elettrical resistivity profiles, carried out in two different experimental sites characterized by different hydrogeology systems. One site, close to Altamura a city in the South of Italy, is represented from karstic and fractured limestone that overlays the deep aquifer. This area has been affected by sludge waste deposits derived from municipal and industrial wastewater treatment plants. The second site, close to San Pancrazio Salentino town in Southern Italy also, is represented from a quarry of calcarenite that has been used as a dump of sludge of mycelium producted from pharmaceutical industry. In both these cases the waste disposal have caused soil-subsoil contamination. Knowledge of the flow rate of the unsaturated zone percolation is needed to investigate the vertical migration of pollutants and the vulnerability of the aquifers. In this study, subsurface electrical resistivity measurements were used to visualize the infiltration of water in the subsoil due to unsaturated water flow. Simultaneously, the vertical flow was investigated by measuring water levels during infiltrometer tests carried out using a large adjustable ring infiltrometer, designed to be installed in the field directly on the outcrop of rock. In addition electrical resistivity azimuthal surveys have been conducted to detect principal fractures strike directions that cause preferential flow. The results obtained

Positron emission tomographic scan investigations of Huntington's disease: cerebral metabolic correlates of cognitive function

International Nuclear Information System (INIS)

Berent, S.; Giordani, B.; Lehtinen, S.; Markel, D.; Penney, J.B.; Buchtel, H.A.; Starosta-Rubinstein, S.; Hichwa, R.; Young, A.B.

1988-01-01

Fifteen drug-free patients with early to mid-stage Huntington's disease (HD) were evaluated with positron emission tomographic (PET) scans of 18 F-2-fluoro-2-deoxy-D-glucose uptake and quantitative measures of neurological function, learning, memory, and general intelligence. In comparison with a group of normal volunteers, the HD patients showed lower metabolism in both caudate (p less than 0.001) and putamen (p less than 0.001) on PET scans. A significant and positive relationship was found between neuropsychological measures of verbal learning and memory and caudate metabolism in the patient group but not in the normal group. Visual-spatial learning did not reflect a similar pattern, but performance intelligence quotient was positively related to both caudate and putamen metabolism in the HD group. Vocabulary level was unrelated to either brain structure. Discussion focuses on these and other observed brain-behavior relationships and on the implications of these findings for general behaviors such as those involved in coping and adaptation

A technical investigation on tools and concepts for sustainable management of the subsurface in The Netherlands

NARCIS (Netherlands)

Griffioen, Jasper; van Wensem, Joke; Oomes, Justine L.M.; Barends, Frans; Breunese, Jaap; Bruining, Hans; Olsthoorn, Theo; Stams, Alfons J.M.; van der Stoel, Almer E.C.

2014-01-01

In response to increasing use of the subsurface, there is a need to modernise policies on sustainable use of the subsurface. This holds in particular for the densely populated Netherlands. We aimed to analyse current practice of subsurface management and the associated pressure points and to

A technical investigation on tools and concepts for sustainable management of the subsurface in The Netherlands.

NARCIS (Netherlands)

Griffioen, J.; Wensem, van J.; Oomes, J.L.; Barends, F.; Breunese, J.; Bruining, H.; Olsthoorn, T.; Stams, A.J.M.; Stoel, van der A.E.

2014-01-01

In response to increasing use of the subsurface, there is a need to modernise policies on sustainable use of the subsurface. This holds in particular for the densely populated Netherlands. We aimed to analyse current practice of subsurface management and the associated pressure points and to

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...

Trichloroethylene (TCE) in tree cores to complement a subsurface investigation on residential property near a former electroplating facility.

Science.gov (United States)

Wilcox, Jeffrey D; Johnson, Kathy M

2016-10-01

Tree cores were collected and analyzed for trichloroethylene (TCE) on a private property between a former electroplating facility in Asheville, North Carolina (USA), and a contaminated wetland/spring complex. TCE was detected in 16 of 31 trees, the locations of which were largely consistent with a "plume core" delineated by a more detailed subsurface investigation nearly 2 years later. Concentrations in tree cores and nearby soil borings were not correlated, perhaps due to heterogeneities in both geologic and tree root structure, spatial and temporal variability in transpiration rates, or interferences caused by other contaminants at the site. Several tree cores without TCE provided evidence for significantly lower TCE concentrations in shallow groundwater along the margins of the contaminated spring complex in an area with limited accessibility. This study demonstrates that tree core analyses can complement a more extensive subsurface investigation, particularly in residential or ecologically sensitive areas.

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 ...

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.

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...

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.

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...

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

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

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.

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 ...

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

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.

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.

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.

Investigating the Surface and Subsurface in Karstic Regions – Terrestrial Laser Scanning versus Low-Altitude Airborne Imaging and the Combination with Geophysical Prospecting

Directory of Open Access Journals (Sweden)

Nora Tilly

2017-08-01

Full Text Available Combining measurements of the surface and subsurface is a promising approach to understand the origin and current changes of karstic forms since subterraneous processes are often the initial driving force. A karst depression in south-west Germany was investigated in a comprehensive campaign with remote sensing and geophysical prospecting. This contribution has two objectives: firstly, comparing terrestrial laser scanning (TLS and low-altitude airborne imaging from an unmanned aerial vehicle (UAV regarding their performance in capturing the surface. Secondly, establishing a suitable way of combining this 3D surface data with data from the subsurface, derived by geophysical prospecting. Both remote sensing approaches performed satisfying and the established digital elevation models (DEMs differ only slightly. These minor discrepancies result essentially from the different viewing geometries and post-processing concepts, for example whether the vegetation was removed or not. Validation analyses against high-accurate DGPS-derived point data sets revealed slightly better results for the DEMTLS with a mean absolute difference of 0.03 m to 0.05 m and a standard deviation of 0.03 m to 0.07 m (DEMUAV: mean absolute difference: 0.11 m to 0.13 m; standard deviation: 0.09 m to 0.11 m. The 3D surface data and 2D image of the vertical cross section through the subsurface along a geophysical profile were combined in block diagrams. The data sets fit very well and give a first impression of the connection between surface and subsurface structures. Since capturing the subsurface with this method is limited to 2D and the data acquisition is quite time consuming, further investigations are necessary for reliable statements about subterraneous structures, how these may induce surface changes, and the origin of this karst depression. Moreover, geophysical prospecting can only produce a suspected image of the subsurface since the apparent resistivity is measured

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.

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.

Naval Arctic Research Laboratory (NARL) Subsurface Containment Berm Investigation

Science.gov (United States)

2015-10-01

Degree-Days CRREL Cold Regions Research and Engineering Laboratory ERDC U.S. Army Engineer Research and Development Center FWENC Foster Wheeler ...contract with the Navy, Foster Wheeler Environmental Corporation (FWENC) constructed a subsurface containment berm at the airfield of the Naval...659J91.61 ncURE 3- 3 NAVAl.. AACnC R(Sf.ARCH l,.ASORATORY POINT 9ARROW. AlASKA AS-BUILT CONTAINMENT BERM EXTENSION AND MONITORING WELLS FOSTER W

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.

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.

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…

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

Corrective Action Investigation Plan for the CNTA Subsurface Sites (CAU Number 443), Revision 1; FINAL

International Nuclear Information System (INIS)

1999-01-01

This Corrective Action Investigation Plan (CAIP) describes the U.S. Department of Energy's (DOE's) planned environmental investigation of the subsurface Central Nevada Test Area (CNTA) Corrective Action Unit (CAU) No. 443. The CNTA is located in Hot Creek Valley in Nye County, Nevada, adjacent to U.S. Highway 6, about 48 kilometers (km) (30 miles[mi]) north of Warm Springs, Nevada. The CNTA was the site of Project Faultless, a nuclear device detonated in the subsurface by the U.S. Atomic Energy Commission (AEC) in January 1968. The purposes of this test were to gauge the seismic effects of a relatively large, high-yield detonation completed in Hot Creek Valley (outside the Nevada Test Site) and to determine the suitability of the site for future large detonations. The yield of the Faultless test was between 200 kilotons and 1 megaton. Two similar tests were planned for the CNTA, but neither of them was completed. Based on the general definition of a corrective action investigation (CAI) from Section IV.14 of the Federal Facility Agreement and Consent Order (FFACO), the purpose of the CAI is ''to gather data sufficient to characterize the nature, extent, and rate of migration or potential rate of migration from releases or discharges of pollutants or contaminants and/or potential releases or discharges from corrective action units identified at the facilities''. For CNTA CAU 443 the concepts developed for the Underground Test Area (UGTA) CAUs will be applied on a limited scale. For the UGTA CAUs, ''the objective of the CAI process is to define boundaries around each UGTA CAU that establish areas that contain water that may be unsafe for domestic and municipal use,'' as stated in Appendix VI of the FFACO (1996). Based on this strategy the CAI for CAU 443 will start with modeling using existing data. New data collection activities are generally contingent upon the results of the modeling and may or may not be part of the CAI. Specific objectives of the CAI ar e as

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

A multi-modal geological investigation framework for subsurface modeling and kinematic monitoring of a slow-moving landslide complex in Colorado, United States

Science.gov (United States)

Lowry, B. W.; Zhou, W.; Smartgeo

2010-12-01

The Muddy Creek landslide complex is a large area of active and reactivating landslides that impact the operation of both a state highway and Paonia Reservoir in Gunnison County, Colorado, United States. Historically, the monitoring of this slide has been investigated using disparate techniques leading to protracted analysis and project knowledge attrition. We present an integrated, data-driven investigation framework that supports continued kinematic monitoring, document cataloging, and subsurface modeling of the landslide complex. A geospatial information system (GIS) was integrated with a visual programming based subsurface model to facilitate modular integration of monitoring data with borehole information. Subsurface modeling was organized by material type and activity state based on multiple sources of kinematic measurement. The framework is constructed to modularly integrate remotely sensed imagery and other spatial datasets such as ASTER, InSAR, and LiDAR derived elevation products as more precise datasets become available. The framework allows for terrestrial LiDAR survey error estimation, borehole siting, and placement of wireless sensor (GPS, accelerometers, geophysical ) networks for optimized spatial relevance and utility. Coordinated spatial referencing within the GIS facilitates geotechnical and hydrogeological modeling input generation and common display of modeling outputs. Kinematic data fusion techniques are accomplished with integration of instrumentation, surficial feature tracking, subsurface classification, and 3D interpolation. The framework includes dynamic decision support including landslide dam failure estimates, back-flooding scenario planning that can be accessed by multiple agencies and stakeholders.

Subsurface defects structural evolution in nano-cutting of single crystal copper

International Nuclear Information System (INIS)

Wang, Quanlong; Bai, Qingshun; Chen, Jiaxuan; Sun, Yazhou; Guo, Yongbo; Liang, Yingchun

2015-01-01

Highlights: • An innovative analysis method is adopted to analyze nano-cutting process accurately. • A characteristic SFT and stair-rod dislocation are found in subsurface defect layer. • The formation mechanism of stair-rod dislocation is investigated. • The local atomic structure of subsurface defects is introduced. - Abstract: In this work, molecular dynamics simulation is performed to study the subsurface defects structural distribution and its evolution during nano-cutting process of single crystal copper. The formation mechanism of chip and machined surface is interviewed by analyzing the dislocation evolution and atomic migration. The centro-symmetry parameter and spherical harmonics method are adopted to characterize the distribution and evolution of the subsurface defect structures and local atomic structures. The results show that stacking faults, dislocation loops, “V-shaped” dislocation loops, and plenty of point defects are formed during the machined surface being formed in shear-slip zone. In subsurface damage layers, stair-rod dislocation, stacking fault tetrahedra, atomic cluster defect, and vacancy defect are formed. And the formation mechanism of stair-rod dislocation is investigated by atomic-scale structure evolution. The local atomic structures of subsurface defects are icosahedrons, hexagonal close packed, body-centered cubic, and defect face center cubic, and the variations of local atomic structures are investigated

Subsurface defects structural evolution in nano-cutting of single crystal copper

Energy Technology Data Exchange (ETDEWEB)

Wang, Quanlong [School of Mechatronics Engineering, Harbin Institute of Technology, Harbin 150001 (China); Center for Precision Engineering, Harbin Institute of Technology, Harbin 150001 (China); Bai, Qingshun [School of Mechatronics Engineering, Harbin Institute of Technology, Harbin 150001 (China); Chen, Jiaxuan, E-mail: wangquanlong0@hit.edu.cn [Center for Precision Engineering, Harbin Institute of Technology, Harbin 150001 (China); Sun, Yazhou [School of Mechatronics Engineering, Harbin Institute of Technology, Harbin 150001 (China); Guo, Yongbo [Center for Precision Engineering, Harbin Institute of Technology, Harbin 150001 (China); Liang, Yingchun [School of Mechatronics Engineering, Harbin Institute of Technology, Harbin 150001 (China)

2015-07-30

Highlights: • An innovative analysis method is adopted to analyze nano-cutting process accurately. • A characteristic SFT and stair-rod dislocation are found in subsurface defect layer. • The formation mechanism of stair-rod dislocation is investigated. • The local atomic structure of subsurface defects is introduced. - Abstract: In this work, molecular dynamics simulation is performed to study the subsurface defects structural distribution and its evolution during nano-cutting process of single crystal copper. The formation mechanism of chip and machined surface is interviewed by analyzing the dislocation evolution and atomic migration. The centro-symmetry parameter and spherical harmonics method are adopted to characterize the distribution and evolution of the subsurface defect structures and local atomic structures. The results show that stacking faults, dislocation loops, “V-shaped” dislocation loops, and plenty of point defects are formed during the machined surface being formed in shear-slip zone. In subsurface damage layers, stair-rod dislocation, stacking fault tetrahedra, atomic cluster defect, and vacancy defect are formed. And the formation mechanism of stair-rod dislocation is investigated by atomic-scale structure evolution. The local atomic structures of subsurface defects are icosahedrons, hexagonal close packed, body-centered cubic, and defect face center cubic, and the variations of local atomic structures are investigated.

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

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.

Developing an eco-routing application.

Science.gov (United States)

2014-01-01

The study develops eco-routing algorithms and investigates and quantifies the system-wide impacts of implementing an eco-routing system. Two eco-routing algorithms are developed: one based on vehicle sub-populations (ECO-Subpopulation Feedback Assign...

Characteristics of Nitrogen Loss through Surface-Subsurface Flow on Red Soil Slopes of Southeast China

Science.gov (United States)

Zheng, Haijin; Liu, Zhao; Zuo, Jichao; Wang, Lingyun; Nie, Xiaofei

2017-12-01

Soil nitrogen (N) loss related to surface flow and subsurface flow (including interflow and groundwater flow) from slope lands is a global issue. A lysimetric experiment with three types of land cover (grass cover, GC; litter cover, LC; and bare land, BL) were carried out on a red soil slope land in southeast China. Total Nitrogen (TN) loss through surface flow, interflow and groundwater flow was observed under 28 natural precipitation events from 2015 to 2016. TN concentrations from subsurface flow on BL and LC plots were, on average, 2.7-8.2 and 1.5-4.4 times greater than TN concentrations from surface flow, respectively; the average concentration of TN from subsurface flow on GC was about 36-56% of that recorded from surface flow. Surface flow, interflow and groundwater flow contributed 0-15, 2-9 and 76-96%, respectively, of loss load of TN. Compared with BL, GC and LC intercepted 83-86% of TN loss through surface runoff; GC intercepted 95% of TN loss through subsurface flow while TN loss through subsurface flow on LC is 2.3 times larger than that on BL. In conclusion, subsurface flow especially groundwater flow is the dominant hydrological rout for N loss that is usually underestimated. Grass cover has the high retention of N runoff loss while litter mulch will increase N leaching loss. These findings provide scientific support to control N runoff loss from the red soil slope lands by using suitable vegetation cover and mulching techniques.

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.

Hydrological processes in the subsurface investigated by water isotopes and silica

Czech Academy of Sciences Publication Activity Database

Šanda, M.; Kulasová, Alena; Císlerová, M.

2009-01-01

Roč. 4, Sp. is. 2 (2009), S83-S92 ISSN 1801-5395 R&D Projects: GA ČR GA205/06/0375 Institutional research plan: CEZ:AV0Z20600510 Keywords : rainfall to runoff response * isotopes * geochemical tracers * streamflow generation * subsurface stormflow * groundwater recharge Subject RIV: DA - Hydrology ; Limnology

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

Wall Painting Investigation by Means of Non-invasive Terahertz Time-Domain Imaging (THz-TDI): Inspection of Subsurface Structures Buried in Historical Plasters

Science.gov (United States)

Dandolo, Corinna Ludovica Koch; Jepsen, Peter Uhd

2016-02-01

Characterization of subsurface features of wall paintings is important in conservation and technical art history as well as in building archaeology and architecture fields. In this study, an area of the apsidal wall painting of Nebbelunde Church (Rødby, Denmark) has been investigated by means of terahertz time-domain imaging (THz-TDI). Subsurface structures have been detected at different depths inside the lime-based plaster of the wall painting until approximately 1 cm from the surface. The surface morphology of the buried structures has been 3D imaged in detail, providing a substantial contribution in their characterization.

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...

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...

Border Collision Route to Quasiperiodicity: Numerical Investigation and Experimental Confirmation

DEFF Research Database (Denmark)

Zhusubaliyev, Zhanybai; Mosekilde, Erik; Maity, S.

2006-01-01

Numerical studies of higher-dimensional piecewise-smooth systems have recently shown how a torus can arise from a periodic cycle through a special type of border-collision bifurcation. The present article investigates this new route to quasiperiodicity in the two-dimensional piecewise-linear normal...... form map. We have obtained the chart of the dynamical modes for this map and showed that border-collision bifurcations can lead to the birth of a stable closed invariant curve associated with quasiperiodic or periodic dynamics. In the parameter regions leading to the existence of an invariant closed...... to quasiperiodicity through a border-collision bifurcation. ©2006 American Institute of Physics...

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.

Using GNSS-R techniques to investigate the near sub-surface of Mars with the Deep Space Network

Science.gov (United States)

Elliott, H. M.; Bell, D. J.; Jin, C.; Decrossas, E.; Asmar, S.; Lazio, J.; Preston, R. A.; Ruf, C. S.; Renno, N. O.

2017-12-01

Global Navigation Satellite Systems Reflectometry (GNSS-R) has shown that passive measurements using separate active sources can infer the soil moisture, snow pack depth and other quantities of scientific interest. Here, we expand upon this method and propose that a passive measurement of the sub-surface dielectric profile of Mars can be made by using multipath interference between reflections off the surface and subsurface dielectric discontinuities. This measurement has the ability to reveal changes in the soil water content, the depth of a layer of sand, thickness of a layer of ice, and even identify centimeter-scale layering which may indicate the presence of a sedimentary bed. We have created a numerical ray tracing model to understand the potential of using multipath interference techniques to investigate the sub-surface dielectric properties and structure of Mars. We have further verified this model using layered beds of sand and concrete in laboratory experiments and then used the model to extrapolate how this technique may be applied to future Mars missions. We will present new results demonstrating how to characterize a multipath interference patterns as a function of frequency and/or incidence angle to measure the thickness of a dielectric layer of sand or ice. Our results demonstrate that dielectric discontinuities in the subsurface can be measured using this passive sensing technique and it could be used to effectively measure the thickness of a dielectric layer in the proximity of a landed spacecraft. In the case of an orbiter, we believe this technique would be effective at measuring the seasonal thickness of CO2 ice in the Polar Regions. This is exciting because our method can produce similar results to traditional ground penetrating radars without the need to have an active radar transmitter in-situ. Therefore, it is possible that future telecommunications systems can serve as both a radio and a scientific instrument when used in conjunction with

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

Subsurface Investigation using 2D Resistivity and Ground Penetrating Radar at Teluk Kumbar, Penang

Science.gov (United States)

Teoh, YJ; Bruka, MA; Idris, NM; Ismail, NA; Muztaza, NM

2018-04-01

The objective of this study is to determine the structure and condition of the subsurface by using 2D resistivity and Ground Penetrating Radar (GPR) methods. The study was conducted at SK Sungai Batu, Teluk Kumbar, Penang Island. For 2D resistivity method, Wenner-Schlumberger array was used while for GPR, 250 MHz antenna was used at the site. The survey consists of 200m length survey line. GPR result shows that there is high intensity of EM. 2D resistivity result shows that the low resistivity region (200 Ωm to 340 Ωm) appears to be at the centre of the survey line from depth 7 m to 13 m. Meanwhile, the higher resistivity region (4000 Ωm to 6000 Ωm) may indicate the bedrock structure of the subsurface, which is the granitic rock. This region is bedrock which rested at depth 14 m and below. In conclusion, data obtained from GPR and 2D resistivity methods can be easily correlated to determine the features of the subsurface.

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.

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.

Modeling subsurface stormflow initiation in low-relief landscapes

Science.gov (United States)

Hopp, Luisa; Vaché, Kellie B.; Rhett Jackson, C.; McDonnell, Jeffrey J.

2015-04-01

Shallow lateral subsurface flow as a runoff generating mechanism at the hillslope scale has mostly been studied in steeper terrain with typical hillside angles of 10 - 45 degrees. These studies have shown that subsurface stormflow is often initiated at the interface between a permeable upper soil layer and a lower conductivity impeding layer, e.g. a B horizon or bedrock. Many studies have identified thresholds of event size and soil moisture states that need to be exceeded before subsurface stormflow is initiated. However, subsurface stormflow generation on low-relief hillslopes has been much less studied. Here we present a modeling study that investigates the initiation of subsurface stormflow on low-relief hillslopes in the Upper Coastal Plain of South Carolina, USA. Hillslopes in this region typically have slope angles of 2-5 degrees. Topsoils are sandy, underlain by a low-conductivity sandy clay loam Bt horizon. Subsurface stormflow has only been intercepted occasionally in a 120 m long trench, and often subsurface flow was not well correlated with stream signals, suggesting a disconnect between subsurface flow on the hillslopes and stream flow. We therefore used a hydrologic model to better understand which conditions promote the initiation of subsurface flow in this landscape, addressing following questions: Is there a threshold event size and soil moisture state for producing lateral subsurface flow? What role does the spatial pattern of depth to the impeding clay layer play for subsurface stormflow dynamics? We reproduced a section of a hillslope, for which high-resolution topographic data and depth to clay measurements were available, in the hydrologic model HYDRUS-3D. Soil hydraulic parameters were based on experimentally-derived data. The threshold analysis was first performed using hourly climate data records for 2009-2010 from the study site to drive the simulation. For this period also trench measurements of subsurface flow were available. In addition

An electrical resistivity-based method for investigation of subsurface structure

Science.gov (United States)

Alves Meira Neto, A.; Litwin, D.; Troch, P. A. A.; Ferre, T. P. A.

2017-12-01

Resolving the spatial distribution of soil porosity within the subsurface is of great importance for understanding flow and transport within heterogeneous media. Additionally, porosity patterns can be associated with the availability of water and carbon dioxide that will drive geochemical reactions and constrain microbiological growth. The use of controlled experimentation has the potential to circumvent problems related to the external and internal variability of natural systems, while also allowing a higher degree of observability. In this study, we suggest an ERT-based method of retrieving porosity fields based on the application of Archie's law associated with an experimental procedure that can be used in laboratory-scale studies. We used a 2 cubic meter soil lysimeter, equipped with 238 electrodes distributed along its walls for testing the method. The lysimeter serves as a scaled-down version of the highly monitored artificial hillslopes at the Landscape Evolution Observatory (LEO) located at Biosphere 2 - University of Arizona. The capability of the ERT system in deriving spatially distributed patterns of porosity with respect to its several sources of uncertainty was numerically evaluated. The results will be used to produce an optimal experimental design and for assessing the reliability of experimental results. This novel approach has the potential to further resolve subsurface heterogeneity within the LEO project, and highlight the use of ERT-derived results for hydro-bio-geochemical studies.

Subsurface Contamination Control

Energy Technology Data Exchange (ETDEWEB)

Y. Yuan

2001-12-12

There are two objectives of this report, ''Subsurface Contamination Control''. The first is to provide a technical basis for recommending limiting radioactive contamination levels (LRCL) on the external surfaces of waste packages (WP) for acceptance into the subsurface repository. The second is to provide an evaluation of the magnitude of potential releases from a defective WP and the detectability of the released contents. The technical basis for deriving LRCL has been established in ''Retrieval Equipment and Strategy for Wp on Pallet'' (CRWMS M and O 2000g, 6.3.1). This report updates the derivation by incorporating the latest design information of the subsurface repository for site recommendation. The derived LRCL on the external surface of WPs, therefore, supercede that described in CRWMS M and O 2000g. The derived LRCL represent the average concentrations of contamination on the external surfaces of each WP that must not be exceeded before the WP is to be transported to the subsurface facility for emplacement. The evaluation of potential releases is necessary to control the potential contamination of the subsurface repository and to detect prematurely failed WPs. The detection of failed WPs is required in order to provide reasonable assurance that the integrity of each WP is intact prior to MGR closure. An emplaced WP may become breached due to manufacturing defects or improper weld combined with failure to detect the defect, by corrosion, or by mechanical penetration due to accidents or rockfall conditions. The breached WP may release its gaseous and volatile radionuclide content to the subsurface environment and result in contaminating the subsurface facility. The scope of this analysis is limited to radioactive contaminants resulting from breached WPs during the preclosure period of the subsurface repository. This report: (1) documents a method for deriving LRCL on the external surfaces of WP for acceptance into the

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

Geophysical and geological investigations of subsurface reservoirs : case studies of Spitsbergen, Norway

Energy Technology Data Exchange (ETDEWEB)

Baelum, Karoline

2011-07-01

The thesis gives a description of the subsurface and outcrop geology at a number of localities on Svalbard through a selection of various geophysical and geological methods. The localities represent a series of geological settings of varying scale, from near surface paleokarst and glacial environments to large scale geological features such as fault zones, grabens and dolerite intrusions. The geophysical and geological methods deployed likewise represent both detailed small scale investigations such as Lidar, radar and geoelectric investigations on and near the surface, and seismic investigations covering larger areas to a depth of several kilometers. The overall aim for all the studies has been to better understand reservoir and cap rock/ice systems in a barren arctic desert characterized by a frozen ground that challenges common geophysical methods. The investigations undertaken in connection with this thesis cover several areas The first part addresses the Billefjorden fault zone (BFZ) with its eastern hanging wall classic rift-basin. This fault zone can be traced for more than 200 km as a lineament that runs almost the entire length of Spitsbergen, from Wijdefjorden in the north to Storfjorden in the south. The seismic data along with surface observations and Lidar scans illustrate the long and complicated history of the BFZ and associated basin, from the initial formation via linkage of reverse faults in the Devonian, through Carboniferous reactivation as a normal fault with adjacent rift-basin in an extensional tectonic regime, to finally Tertiary contraction seen as fault reactivation and basin inversion in connection with the formation of the west-coast fold and thrust-belt. Especially the development of the Carboniferous rift-basin is of interest. An integrated study by seismic and georadar mapping, and Lidar data interpretation combined with outcrop analysis of faults and sedimentary succession, have shed new, detailed information on the good sandstone

Geochemical and mineralogical investigation of uranium in multi-element contaminated, organic-rich subsurface sediment

International Nuclear Information System (INIS)

Qafoku, Nikolla P.; Gartman, Brandy N.; Kukkadapu, Ravi K.; Arey, Bruce W.; Williams, Kenneth H.; Mouser, Paula J.; Heald, Steve M.; Bargar, John R.; Janot, Noémie; Yabusaki, Steve; Long, Philip E.

2014-01-01

Highlights: • Subsurface naturally reduced zones (NRZ) contain U and other potential co-contaminants. • The NRZ has a remarkable assortment of chemically complex, potential U hosts. • Micron-scale, multi-contaminant areas were discovered in NRZ. • U(IV) occurs as biogenic UO 2 (82%), or biomass – bound monomeric U(IV) (18%). • NRZs may exhibit contaminant sink-source complex behavior. - Abstract: Subsurface regions of alluvial sediments characterized by an abundance of refractory or lignitic organic carbon compounds and reduced Fe and S bearing minerals, which are referred to as naturally reduced zones (NRZ), are present at the Integrated Field Research Challenge site in Rifle, CO (a former U mill site), and other contaminated subsurface sites. A study was conducted to demonstrate that the NRZ contains a variety of contaminants and unique minerals and potential contaminant hosts, investigate micron-scale spatial association of U with other co-contaminants, and determine solid phase-bounded U valence state and phase identity. The NRZ sediment had significant solid phase concentrations of U and other co-contaminants suggesting competing sorption reactions and complex temporal variations in dissolved contaminant concentrations in response to transient redox conditions, compared to single contaminant systems. The NRZ sediment had a remarkable assortment of potential contaminant hosts, such as Fe oxides, siderite, Fe(II) bearing clays, rare solids such as ZnS framboids and CuSe, and, potentially, chemically complex sulfides. Micron-scale inspections of the solid phase showed that U was spatially associated with other co-contaminants. High concentration, multi-contaminant, micron size (ca. 5–30 μm) areas of mainly U(IV) (53–100%) which occurred as biogenic UO 2 (82%), or biomass – bound monomeric U(IV) (18%), were discovered within the sediment matrix confirming that biotically induced reduction and subsequent sequestration of contaminant U(VI) via

Upscaling Surface and Subsurface Runoff Process Using a Travel Time Matching Strategy: Application to the Ohio River Basin

Science.gov (United States)

Zhao, Y.; Beighley, E.

2017-12-01

While hydrologic understanding gained from model assessment and sensitivity analyses continues to grow, computational efficiency is still a challenge for the hydrologic and hydraulic modeling community, especially at continental and global scales. This research presents a runoff flowpath travel-time matching method to upscale hydrologic response characteristics of surface and subsurface runoff from fine to coarse model resolutions. Five model resolutions are investigated in this study: 10, 32, 100, 320, 1000 km2, where model resolution represents the threshold areas used to define the underlying river network and catchment boundaries. Here, the 1 km2 mode resolution is set as the reference model. A case study in the Ohio River Basin (roughly 500,000 km2) is presented using a synthetic SCS 2-year flood event. The velocities of surface and subsurface runoff from Hillslope River Routing (HRR) model operating at 1 km2 resolution is determined on a high-performance computing cluster. Using these simulated velocities and 90-m Digital Elevation Model (DEM), pixel level velocities are determined separately for hillslopes (surface and subsurface) and channels. Cumulative Probability Distributions (CDFs) for surface and subsurface travel times based on the gridded 90-m velocities and conceptualized model units representing individual catchments in the HRR model are matched by adjusting surface roughness and subsurface hydraulic conductivity along HRR hillslopes in the courser model resolutions. The beta distribution is applied to approximate the CDF travel time to reduce pixel-level processing time for large model units. Simulated hydrographs at the outlet of the Ohio River Basin for the five coarser model resolutions are shown to have nearly identical peak discharge and time-to-peak discharge values as compared to the reference model. The proposed upscaling method can reduce the computation time by transferring the hydrologic characteristics captured at fine scales to

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.

Complete Subsurface Elemental Composition Measurements With PING

Science.gov (United States)

Parsons, A. M.

2012-01-01

The Probing In situ with Neutrons and Gamma rays (PING) instrument will measure the complete bulk elemental composition of the subsurface of Mars as well as any other solid planetary body. PING can thus be a highly effective tool for both detailed local geochemistry science investigations and precision measurements of Mars subsurface reSOurces in preparation for future human exploration. As such, PING is thus fully capable of meeting a majority of both ncar and far term elements in Challenge #1 presented for this conference. Measuring the ncar subsurface composition of Mars will enable many of the MEPAG science goals and will be key to filling an important Strategic Knowledge Gap with regard to In situ Resources Utilization (ISRU) needs for human exploration. [1, 2] PING will thus fill an important niche in the Mars Exploration Program.

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.

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

Investigations of subsurface flow constructed wetlands and associated geomaterial resources in the Akumal and Reforma regions, Quintana Roo, Mexico

Science.gov (United States)

Krekeler, Mark P. S.; Probst, Pete; Samsonov, Misha; Tselepis, Cynthia M.; Bates, William; Kearns, Lance E.; Maynard, J. Barry

2007-12-01

Subsurface flow constructed wetlands in the village of Akumal, Quintana Roo, Mexico were surveyed to determine the general status of the wetland systems and provide baseline information for long term monitoring and further study. Twenty subsurface flow wetlands were surveyed and common problems observed in the systems were overloading, poor plant cover, odor, and no secondary containment. Bulk mineral composition of aggregate from two subsurface flow constructed wetlands was determined to consist solely of calcite using bulk powder X-ray diffraction. Some soil structure is developed in the aggregate and aggregate levels in wetlands drop at an estimated rate between 3 and 10 cm/year for overloaded wetlands owing to dissolution. Mineral composition from fresh aggregate samples commonly is a mixture of calcite and aragonite. Trace amounts of Pb, Zn, Co, and Cr were observed in fresh aggregate. Coefficients of permeability ( k) varied from 0.006 to 0.027 cm/s with an average values being 0.016 cm/s. Grain size analysis of fresh aggregate samples indicates there are unimodal and multimodal size distributions in the samples with modes in the coarse and fine sand being common. Investigations of other geologic media from the Reforma region indicate that a dolomite with minor amounts of Fe-oxide and palygorskite is abundant and may be a better aggregate source that the current materials used. A Ca-montmorillonite bed was identified in the Reforma region as well and this unit is suitable to serve as a clay liner to prevent leaks for new and existing wetland systems. These newly discovered geologic resources should aid in the improvement of subsurface flow constructed wetlands in the region. Although problems do exist in these wetlands with respect to design, these systems represent a successful implementation of constructed wetlands at a community level in developing regions.

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.

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.

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.

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

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

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, ...

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.

Wall Painting Investigation by Means of Non-invasive Terahertz Time-Domain Imaging (THz-TDI): Inspection of Subsurface Structures Buried in Historical Plasters

DEFF Research Database (Denmark)

Dandolo, Corinna Ludovica Koch; Jepsen, Peter Uhd

2016-01-01

Characterization of subsurface features of wall paintings is important in conservation and technical art history as well as in building archaeology and architecture fields. In this study, an area of the apsidal wall painting of Nebbelunde Church (Rødby, Denmark) has been investigated by means of ...

Subsurface Ventilation System Description Document

Energy Technology Data Exchange (ETDEWEB)

Eric Loros

2001-07-25

The Subsurface Ventilation System supports the construction and operation of the subsurface repository by providing air for personnel and equipment and temperature control for the underground areas. Although the system is located underground, some equipment and features may be housed or located above ground. The system ventilates the underground by providing ambient air from the surface throughout the subsurface development and emplacement areas. The system provides fresh air for a safe work environment and supports potential retrieval operations by ventilating and cooling emplacement drifts. The system maintains compliance within the limits established for approved air quality standards. The system maintains separate ventilation between the development and waste emplacement areas. The system shall remove a portion of the heat generated by the waste packages during preclosure to support thermal goals. The system provides temperature control by reducing drift temperature to support potential retrieval operations. The ventilation system has the capability to ventilate selected drifts during emplacement and retrieval operations. The Subsurface Facility System is the main interface with the Subsurface Ventilation System. The location of the ducting, seals, filters, fans, emplacement doors, regulators, and electronic controls are within the envelope created by the Ground Control System in the Subsurface Facility System. The Subsurface Ventilation System also interfaces with the Subsurface Electrical System for power, the Monitored Geologic Repository Operations Monitoring and Control System to ensure proper and safe operation, the Safeguards and Security System for access to the emplacement drifts, the Subsurface Fire Protection System for fire safety, the Emplacement Drift System for repository performance, and the Backfill Emplacement and Subsurface Excavation Systems to support ventilation needs.

Subsurface Ventilation System Description Document

Energy Technology Data Exchange (ETDEWEB)

NONE

2000-10-12

The Subsurface Ventilation System supports the construction and operation of the subsurface repository by providing air for personnel and equipment and temperature control for the underground areas. Although the system is located underground, some equipment and features may be housed or located above ground. The system ventilates the underground by providing ambient air from the surface throughout the subsurface development and emplacement areas. The system provides fresh air for a safe work environment and supports potential retrieval operations by ventilating and cooling emplacement drifts. The system maintains compliance within the limits established for approved air quality standards. The system maintains separate ventilation between the development and waste emplacement areas. The system shall remove a portion of the heat generated by the waste packages during preclosure to support thermal goals. The system provides temperature control by reducing drift temperature to support potential retrieval operations. The ventilation system has the capability to ventilate selected drifts during emplacement and retrieval operations. The Subsurface Facility System is the main interface with the Subsurface Ventilation System. The location of the ducting, seals, filters, fans, emplacement doors, regulators, and electronic controls are within the envelope created by the Ground Control System in the Subsurface Facility System. The Subsurface Ventilation System also interfaces with the Subsurface Electrical System for power, the Monitored Geologic Repository Operations Monitoring and Control System to ensure proper and safe operation, the Safeguards and Security System for access to the emplacement drifts, the Subsurface Fire Protection System for fire safety, the Emplacement Drift System for repository performance, and the Backfill Emplacement and Subsurface Excavation Systems to support ventilation needs.

Subsurface Facility System Description Document

International Nuclear Information System (INIS)

Eric Loros

2001-01-01

The Subsurface Facility System encompasses the location, arrangement, size, and spacing of the underground openings. This subsurface system includes accesses, alcoves, and drifts. This system provides access to the underground, provides for the emplacement of waste packages, provides openings to allow safe and secure work conditions, and interfaces with the natural barrier. This system includes what is now the Exploratory Studies Facility. The Subsurface Facility System physical location and general arrangement help support the long-term waste isolation objectives of the repository. The Subsurface Facility System locates the repository openings away from main traces of major faults, away from exposure to erosion, above the probable maximum flood elevation, and above the water table. The general arrangement, size, and spacing of the emplacement drifts support disposal of the entire inventory of waste packages based on the emplacement strategy. The Subsurface Facility System provides access ramps to safely facilitate development and emplacement operations. The Subsurface Facility System supports the development and emplacement operations by providing subsurface space for such systems as ventilation, utilities, safety, monitoring, and transportation

Intelligent SUBsurface Quality : Intelligent use of subsurface infrastructure for surface quality

NARCIS (Netherlands)

Hooimeijer, F.L.; Kuzniecow Bacchin, T.; Lafleur, F.; van de Ven, F.H.M.; Clemens, F.H.L.R.; Broere, W.; Laumann, S.J.; Klaassen, R.G.; Marinetti, C.

2016-01-01

This project focuses on the urban renewal of (delta) metropolises and concentrates on the question how to design resilient, durable (subsurface) infrastructure in urban renewal projects using parameters of the natural system – linking in an efficient way (a) water cycle, (b) soil and subsurface

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.

Deep subsurface microbial processes

Science.gov (United States)

Lovley, D.R.; Chapelle, F.H.

1995-01-01

Information on the microbiology of the deep subsurface is necessary in order to understand the factors controlling the rate and extent of the microbially catalyzed redox reactions that influence the geophysical properties of these environments. Furthermore, there is an increasing threat that deep aquifers, an important drinking water resource, may be contaminated by man's activities, and there is a need to predict the extent to which microbial activity may remediate such contamination. Metabolically active microorganisms can be recovered from a diversity of deep subsurface environments. The available evidence suggests that these microorganisms are responsible for catalyzing the oxidation of organic matter coupled to a variety of electron acceptors just as microorganisms do in surface sediments, but at much slower rates. The technical difficulties in aseptically sampling deep subsurface sediments and the fact that microbial processes in laboratory incubations of deep subsurface material often do not mimic in situ processes frequently necessitate that microbial activity in the deep subsurface be inferred through nonmicrobiological analyses of ground water. These approaches include measurements of dissolved H2, which can predict the predominant microbially catalyzed redox reactions in aquifers, as well as geochemical and groundwater flow modeling, which can be used to estimate the rates of microbial processes. Microorganisms recovered from the deep subsurface have the potential to affect the fate of toxic organics and inorganic contaminants in groundwater. Microbial activity also greatly influences 1 the chemistry of many pristine groundwaters and contributes to such phenomena as porosity development in carbonate aquifers, accumulation of undesirably high concentrations of dissolved iron, and production of methane and hydrogen sulfide. Although the last decade has seen a dramatic increase in interest in deep subsurface microbiology, in comparison with the study of

Modular evaluation method for subsurface activities (MEMSA). A novel approach for integrating social acceptance in a permit decision-making process for subsurface activities

International Nuclear Information System (INIS)

Os, Herman W.A. van; Herber, Rien; Scholtens, Bert

2017-01-01

We investigate how the decision support system ‘Modular Evaluation Method Subsurface Activities’ (MEMSA) can help facilitate an informed decision-making process for permit applications of subsurface activities. To this end, we analyze the extent the MEMSA approach allows for a dialogue between stakeholders in a transparent manner. We use the exploration permit for the underground gas storage facility at the Pieterburen salt dome (Netherlands) as a case study. The results suggest that the MEMSA approach is flexible enough to adjust to changing conditions. Furthermore, MEMSA provides a novel way for identifying structural problems and possible solutions in permit decision-making processes for subsurface activities, on the basis of the sensitivity analysis of intermediate rankings. We suggest that the planned size of an activity should already be specified in the exploration phase, because this would allow for a more efficient use of the subsurface as a whole. We conclude that the host community should be involved to a greater extent and in an early phase of the permit decision-making process, for example, already during the initial analysis of the project area of a subsurface activity. We suggest that strategic national policy goals are to be re-evaluated on a regular basis, in the form of a strategic vision for the subsurface, to account for timing discrepancies between the realization of activities and policy deadlines, because this discrepancy can have a large impact on the necessity and therefore acceptance of a subsurface activity.

Modular evaluation method for subsurface activities (MEMSA). A novel approach for integrating social acceptance in a permit decision-making process for subsurface activities

Energy Technology Data Exchange (ETDEWEB)

Os, Herman W.A. van, E-mail: h.w.a.van.os@rug.nl [University of Groningen, Faculty of Mathematics and Natural Sciences, Geo-Energy, PO Box 800, 9700 AV Groningen (Netherlands); Herber, Rien, E-mail: rien.herber@rug.nl [University of Groningen, Faculty of Mathematics and Natural Sciences, Geo-Energy, PO Box 800, 9700 AV Groningen (Netherlands); Scholtens, Bert, E-mail: l.j.r.scholtens@rug.nl [University of Groningen, Faculty of Economics and Business, PO Box 800, 9700 AV Groningen (Netherlands)

2017-05-15

We investigate how the decision support system ‘Modular Evaluation Method Subsurface Activities’ (MEMSA) can help facilitate an informed decision-making process for permit applications of subsurface activities. To this end, we analyze the extent the MEMSA approach allows for a dialogue between stakeholders in a transparent manner. We use the exploration permit for the underground gas storage facility at the Pieterburen salt dome (Netherlands) as a case study. The results suggest that the MEMSA approach is flexible enough to adjust to changing conditions. Furthermore, MEMSA provides a novel way for identifying structural problems and possible solutions in permit decision-making processes for subsurface activities, on the basis of the sensitivity analysis of intermediate rankings. We suggest that the planned size of an activity should already be specified in the exploration phase, because this would allow for a more efficient use of the subsurface as a whole. We conclude that the host community should be involved to a greater extent and in an early phase of the permit decision-making process, for example, already during the initial analysis of the project area of a subsurface activity. We suggest that strategic national policy goals are to be re-evaluated on a regular basis, in the form of a strategic vision for the subsurface, to account for timing discrepancies between the realization of activities and policy deadlines, because this discrepancy can have a large impact on the necessity and therefore acceptance of a subsurface activity.

Biodegradation of crude oil in Arctic subsurface water from the Disko Bay (Greenland) is limited

DEFF Research Database (Denmark)

Scheibye, Katrine; Christensen, Jan H.; Johnsen, Anders R.

2017-01-01

Biological degradation is the main process for oil degradation in a subsurface oil plume. There is, however, little information on the biodegradation potential of Arctic, marine subsurface environments. We therefore investigated oil biodegradation in microcosms at 2 °C containing Arctic subsurfac...... for the C1-naphthalenes. To conclude, the marine subsurface microorganisms from the Disko Bay had the potential for biodegradation of n-alkanes and isoprenoids while the metabolically complex and toxic PACs and their alkylated homologs remained almost unchanged.......Biological degradation is the main process for oil degradation in a subsurface oil plume. There is, however, little information on the biodegradation potential of Arctic, marine subsurface environments. We therefore investigated oil biodegradation in microcosms at 2 °C containing Arctic subsurface...... seawater from the Disko Bay (Greenland) and crude oil at three concentrations of 2.5-10 mg/L. Within 71 days, the total petroleum hydrocarbon concentration decreased only by 18 ± 18% for an initial concentration of 5 mg/L. The saturated alkanes nC13-nC30 and the isoprenoids iC18-iC21 were biodegraded...

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...

Ultra-trace measurement of Dechloranes to investigate food as a route of human exposure.

Science.gov (United States)

L'Homme, Benjamin; Calaprice, Chiara; Calvano, Cosima Damiana; Zambonin, Carlo; Leardi, Riccardo; Focant, Jean-François

2015-11-01

Dechloranes, including Dechlorane Plus (syn- and anti-isomers), Dechlorane 602, Dechlorane 603, Dechlorane 604, Chlordene Plus, and Mirex are used as flame-retardants and were recently found in human serum of the European population. In order to investigate if food consumption would possibly be a significant route of exposure, we developed a method for the measurement of Dechloranes in food and feed. We showed that it was possible to extend the scope of the regular polychlorinated dibenzo-p-dioxins (PCDDs), polychlorinated dibenzofurans (PCDFs), dioxin like (DL-), and non-dioxin like (NDL-) regulated PCBs clean-up and fractionation procedure to Dechloranes and that no compound degradation occurred during the strong acidic treatments used for lipid digestion. Dechloranes were measured by gas chromatography coupled to triple quadrupole mass spectrometry (GC-QQQMS/MS). We optimized injection parameters by face centered experimental design (FCD). The electron ionization fragmentation was investigated to set appropriate multiple reaction monitoring (MRM) transitions. Instrumental and method limits of quantitation (iLOQs and mLOQs) were determined following EU guidelines for dioxin analyses in food. A total of 88 samples were analyzed to assess the prevalence of this route of exposure to humans. Average levels of the sum of Dechloranes ranged from 10 to 31pg/g fat, with the exception of fish, feed additives, and corn that were reported in pg/g wet weight at average levels of 9, 12, and 2pg/g ww. Based on Belgian food habits, a dietary intake was estimated to be 136pg/day. The relatively low reported levels indicate that other routes of human exposure should be considered. Copyright © 2015 Elsevier Ltd. All rights reserved.

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.

Site Recommendation Subsurface Layout

International Nuclear Information System (INIS)

C.L. Linden

2000-01-01

The purpose of this analysis is to develop a Subsurface Facility layout that is capable of accommodating the statutory capacity of 70,000 metric tons of uranium (MTU), as well as an option to expand the inventory capacity, if authorized, to 97,000 MTU. The layout configuration also requires a degree of flexibility to accommodate potential changes in site conditions or program requirements. The objective of this analysis is to provide a conceptual design of the Subsurface Facility sufficient to support the development of the Subsurface Facility System Description Document (CRWMS M andO 2000e) and the ''Emplacement Drift System Description Document'' (CRWMS M andO 2000i). As well, this analysis provides input to the Site Recommendation Consideration Report. The scope of this analysis includes: (1) Evaluation of the existing facilities and their integration into the Subsurface Facility design. (2) Identification and incorporation of factors influencing Subsurface Facility design, such as geological constraints, thermal loading, constructibility, subsurface ventilation, drainage control, radiological considerations, and the Test and Evaluation Facilities. (3) Development of a layout showing an available area in the primary area sufficient to support both the waste inventories and individual layouts showing the emplacement area required for 70,000 MTU and, if authorized, 97,000 MTU

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.

Change in ocean subsurface environment to suppress tropical cyclone intensification under global warming

Science.gov (United States)

Huang, Ping; Lin, I. -I; Chou, Chia; Huang, Rong-Hui

2015-01-01

Tropical cyclones (TCs) are hazardous natural disasters. Because TC intensification is significantly controlled by atmosphere and ocean environments, changes in these environments may cause changes in TC intensity. Changes in surface and subsurface ocean conditions can both influence a TC's intensification. Regarding global warming, minimal exploration of the subsurface ocean has been undertaken. Here we investigate future subsurface ocean environment changes projected by 22 state-of-the-art climate models and suggest a suppressive effect of subsurface oceans on the intensification of future TCs. Under global warming, the subsurface vertical temperature profile can be sharpened in important TC regions, which may contribute to a stronger ocean coupling (cooling) effect during the intensification of future TCs. Regarding a TC, future subsurface ocean environments may be more suppressive than the existing subsurface ocean environments. This suppressive effect is not spatially uniform and may be weak in certain local areas. PMID:25982028

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...

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

Method for retrospective estimation of absorbed dose in subsurface tissues when conducting works connected with the Chernobyl' NPP accident effect elimination (using experimental and calculated data)

International Nuclear Information System (INIS)

Panova, V.I.; Shaks, A.I.

1992-01-01

The method for retrospective estimation of doses in subsurface tissues at early time periods from the accident begin in the case, when gamma radiation dose rate values (radiation field cartogram) and a person irradiation conditions on contaminated territory (professional route) are known, is discussed

Uranium Biomineralization by Natural Microbial Phosphatase Activities in the Subsurface

Energy Technology Data Exchange (ETDEWEB)

Sobecky, Patricia A. [Univ. of Alabama, Tuscaloosa, AL (United States)

2015-04-06

In this project, inter-disciplinary research activities were conducted in collaboration among investigators at The University of Alabama (UA), Georgia Institute of Technology (GT), Lawrence Berkeley National Laboratory (LBNL), Brookhaven National Laboratory (BNL), the DOE Joint Genome Institute (JGI), and the Stanford Synchrotron Radiation Light source (SSRL) to: (i) confirm that phosphatase activities of subsurface bacteria in Area 2 and 3 from the Oak Ridge Field Research Center result in solid U-phosphate precipitation in aerobic and anaerobic conditions; (ii) investigate the eventual competition between uranium biomineralization via U-phosphate precipitation and uranium bioreduction; (iii) determine subsurface microbial community structure changes of Area 2 soils following organophosphate amendments; (iv) obtain the complete genome sequences of the Rahnella sp. Y9-602 and the type-strain Rahnella aquatilis ATCC 33071 isolated from these soils; (v) determine if polyphosphate accumulation and phytate hydrolysis can be used to promote U(VI) biomineralization in subsurface sediments; (vi) characterize the effect of uranium on phytate hydrolysis by a new microorganism isolated from uranium-contaminated sediments; (vii) utilize positron-emission tomography to label and track metabolically-active bacteria in soil columns, and (viii) study the stability of the uranium phosphate mineral product. Microarray analyses and mineral precipitation characterizations were conducted in collaboration with DOE SBR-funded investigators at LBNL. Thus, microbial phosphorus metabolism has been shown to have a contributing role to uranium immobilization in the subsurface.

Uranium Biomineralization by Natural Microbial Phosphatase Activities in the Subsurface

International Nuclear Information System (INIS)

Sobecky, Patricia A.

2015-01-01

In this project, inter-disciplinary research activities were conducted in collaboration among investigators at The University of Alabama (UA), Georgia Institute of Technology (GT), Lawrence Berkeley National Laboratory (LBNL), Brookhaven National Laboratory (BNL), the DOE Joint Genome Institute (JGI), and the Stanford Synchrotron Radiation Light source (SSRL) to: (i) confirm that phosphatase activities of subsurface bacteria in Area 2 and 3 from the Oak Ridge Field Research Center result in solid U-phosphate precipitation in aerobic and anaerobic conditions; (ii) investigate the eventual competition between uranium biomineralization via U-phosphate precipitation and uranium bioreduction; (iii) determine subsurface microbial community structure changes of Area 2 soils following organophosphate amendments; (iv) obtain the complete genome sequences of the Rahnella sp. Y9-602 and the type-strain Rahnella aquatilis ATCC 33071 isolated from these soils; (v) determine if polyphosphate accumulation and phytate hydrolysis can be used to promote U(VI) biomineralization in subsurface sediments; (vi) characterize the effect of uranium on phytate hydrolysis by a new microorganism isolated from uranium-contaminated sediments; (vii) utilize positron-emission tomography to label and track metabolically-active bacteria in soil columns, and (viii) study the stability of the uranium phosphate mineral product. Microarray analyses and mineral precipitation characterizations were conducted in collaboration with DOE SBR-funded investigators at LBNL. Thus, microbial phosphorus metabolism has been shown to have a contributing role to uranium immobilization in the subsurface.

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

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,...

Characterization of accumulated precipitates during subsurface iron removal

KAUST Repository

Van Halem, Doris

2011-01-01

The principle of subsurface iron removal for drinking water supply is that aerated water is periodically injected into the aquifer through a tube well. On its way into the aquifer, the injected O2-rich water oxidizes adsorbed Fe 2+, creating a subsurface oxidation zone. When groundwater abstraction is resumed, the soluble Fe 2+ is adsorbed and water with reduced Fe concentrations is abstracted for multiple volumes of the injection water. In this article, Fe accumulation deposits in the aquifer near subsurface treatment wells were identified and characterized to assess the sustainability of subsurface iron removal regarding clogging of the aquifer and the potential co-accumulation of other groundwater constituents, such as As. Chemical extraction of soil samples, with Acid-Oxalate and HNO3, showed that Fe had accumulated at specific depths near subsurface iron removal wells after 12 years of operation. Whether it was due to preferred flow paths or geochemical mineralogy conditions; subsurface iron removal clearly favoured certain soil layers. The total Fe content increased between 11.5 and 390.8 mmol/kg ds in the affected soil layers, and the accumulated Fe was found to be 56-100% crystalline. These results suggest that precipitated amorphous Fe hydroxides have transformed to Fe hydroxides of higher crystallinity. These crystalline, compact Fe hydroxides have not noticeably clogged the investigated well and/or aquifer between 1996 and 2008. The subsurface iron removal wells even need less frequent rehabilitation, as drawdown increases more slowly than in normal production wells. Other groundwater constituents, such as Mn, As and Sr were found to co-accumulate with Fe. Acid extraction and ESEM-EDX showed that Ca occurred together with Fe and by X-ray Powder Diffraction it was identified as calcite. © 2010 Elsevier Ltd. All rights reserved.

Characterization of accumulated precipitates during subsurface iron removal

International Nuclear Information System (INIS)

Halem, Doris van; Vet, Weren de; Verberk, Jasper; Amy, Gary; Dijk, Hans van

2011-01-01

Research highlights: → Accumulated iron was not found to clog the well or aquifer after 12 years of subsurface iron removal. → 56-100% of accumulated iron hydroxides were found to be crystalline. → Subsurface iron removal favoured certain soil layers, either due to hydraulics or mineralogy. → Other groundwater constituents, such as manganese and arsenic were found to co-accumulate with iron. - Abstract: The principle of subsurface iron removal for drinking water supply is that aerated water is periodically injected into the aquifer through a tube well. On its way into the aquifer, the injected O 2 -rich water oxidizes adsorbed Fe 2+ , creating a subsurface oxidation zone. When groundwater abstraction is resumed, the soluble Fe 2+ is adsorbed and water with reduced Fe concentrations is abstracted for multiple volumes of the injection water. In this article, Fe accumulation deposits in the aquifer near subsurface treatment wells were identified and characterized to assess the sustainability of subsurface iron removal regarding clogging of the aquifer and the potential co-accumulation of other groundwater constituents, such as As. Chemical extraction of soil samples, with Acid-Oxalate and HNO 3 , showed that Fe had accumulated at specific depths near subsurface iron removal wells after 12 years of operation. Whether it was due to preferred flow paths or geochemical mineralogy conditions; subsurface iron removal clearly favoured certain soil layers. The total Fe content increased between 11.5 and 390.8 mmol/kg ds in the affected soil layers, and the accumulated Fe was found to be 56-100% crystalline. These results suggest that precipitated amorphous Fe hydroxides have transformed to Fe hydroxides of higher crystallinity. These crystalline, compact Fe hydroxides have not noticeably clogged the investigated well and/or aquifer between 1996 and 2008. The subsurface iron removal wells even need less frequent rehabilitation, as drawdown increases more slowly than in

High spatial-temporal resolution and integrated surface and subsurface precipitation-runoff modelling for a small stormwater catchment

Science.gov (United States)

Hailegeorgis, Teklu T.; Alfredsen, Knut

2018-02-01

Reliable runoff estimation is important for design of water infrastructure and flood risk management in urban catchments. We developed a spatially distributed Precipitation-Runoff (P-R) model that explicitly represents the land cover information, performs integrated modelling of surface and subsurface components of the urban precipitation water cycle and flow routing. We conducted parameter calibration and validation for a small (21.255 ha) stormwater catchment in Trondheim City during Summer-Autumn events and season, and snow-influenced Winter-Spring seasons at high spatial and temporal resolutions of respectively 5 m × 5 m grid size and 2 min. The calibration resulted in good performance measures (Nash-Sutcliffe efficiency, NSE = 0.65-0.94) and acceptable validation NSE for the seasonal and snow-influenced periods. The infiltration excess surface runoff dominates the peak flows while the contribution of subsurface flow to the sewer pipes also augments the peak flows. Based on the total volumes of simulated flow in sewer pipes (Qsim) and precipitation (P) during the calibration periods, the Qsim/P ranges from 21.44% for an event to 56.50% for the Winter-Spring season, which are in close agreement with the observed volumes (Qobs/P). The lowest percentage of precipitation volume that is transformed to the total simulated runoff in the catchment (QT) is 79.77%. Computation of evapotranspiration (ET) indicated that the ET/P is less than 3% for the events and snow-influenced seasons while it is about 18% for the Summer-Autumn season. The subsurface flow contribution to the sewer pipes are markedly higher than the total surface runoff volume for some events and the Summer-Autumn season. The peakiest flow rates correspond to the Winter-Spring season. Therefore, urban runoff simulation for design and management purposes should include two-way interactions between the subsurface runoff and flow in sewer pipes, and snow-influenced seasons. The developed urban P-R model is

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

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

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

Evaluation and analysis of polished fused silica subsurface quality by the nanoindenter technique

International Nuclear Information System (INIS)

Ma Bin; Shen Zhengxiang; He Pengfei; Sha Fei; Wang Chunliang; Wang Bin; Ji Yiqin; Liu Huasong; Li Weihao; Wang Zhanshan

2011-01-01

We evaluate the subsurface quality of polished fused silica samples using the nanoindenter technique. Two kinds of samples, consisting of hundreds of nanometers and micrometers of subsurface damage layers, are fabricated by controlling the grinding and polishing processes, and the subsurface quality has been verified by the chemical etching method. Then several nanoindentation experiments are performed using the Berkovich tip to investigate the subsurface quality. Some differences are found by relative measurements in terms of the relationship between the total penetration and the peak load on the surfaces, the modulus calculated over the defined depths and from unload, and the indented morphology at a constant load near the surface collapse threshold. Finally, the capabilities of such a mechanical method for detecting subsurface flaws are discussed and analyzed.

Subsurface Profile Mapping using 3-D Compressive Wave Imaging

Directory of Open Access Journals (Sweden)

Hazreek Z A M

2017-01-01

Full Text Available Geotechnical site investigation related to subsurface profile mapping was commonly performed to provide valuable data for design and construction stage based on conventional drilling techniques. From past experience, drilling techniques particularly using borehole method suffer from limitations related to expensive, time consuming and limited data coverage. Hence, this study performs subsurface profile mapping using 3-D compressive wave imaging in order to minimize those conventional method constraints. Field measurement and data analysis of compressive wave (p-wave, vp was performed using seismic refraction survey (ABEM Terraloc MK 8, 7 kg of sledgehammer and 24 units of vertical geophone and OPTIM (SeisOpt@Picker & SeisOpt@2D software respectively. Then, 3-D compressive wave distribution of subsurface studied was obtained using analysis of SURFER software. Based on 3-D compressive wave image analyzed, it was found that subsurface profile studied consist of three main layers representing top soil (vp = 376 – 600 m/s, weathered material (vp = 900 – 2600 m/s and bedrock (vp > 3000 m/s. Thickness of each layer was varied from 0 – 2 m (first layer, 2 – 20 m (second layer and 20 m and over (third layer. Moreover, groundwater (vp = 1400 – 1600 m/s starts to be detected at 2.0 m depth from ground surface. This study has demonstrated that geotechnical site investigation data related to subsurface profiling was applicable to be obtained using 3-D compressive wave imaging. Furthermore, 3-D compressive wave imaging was performed based on non destructive principle in ground exploration thus consider economic, less time, large data coverage and sustainable to our environment.

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...

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.

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.

Subsurface Investigations Program at the radioactive waste management complex of the Idaho National Engineering Laboratory. Annual progress report, FY-1985

International Nuclear Information System (INIS)

Hubbell, J.M.; Hull, L.C.; Humphrey, T.G.; Russell, B.F.; Pittman, J.R.; Cannon, K.M.

1985-12-01

This report describes work conducted in FY-85 in support of the Subsurface Investigation Program at the Radioactive Waste Management Complex of the Idaho National Engineering Laboratory. The work is part of a continuing effort to define and predict radionuclide migration from buried waste. The Subsurface Investigation Program is a cooperative study conducted by EG and G Idaho and the US Geological Survey, INEL Office. EG and G is responsible for the shallow drilling, solution chemistry, and net downward flux portions of this program, while the US Geological Survey is responsible for the weighing lysimeters and test trench. Data collection was initiated by drilling, sampling, and instrumenting shallow wells, continuing the installation of test trenches, and modifying the two weighing lysimeters. Twenty-one shallow auger holes were around the Radioactive Waste Management Complex (RWMC) to evaluate radionuclide content in the surficial sediments, to determine the geologic and hydrologic characteristics of the surficial sediments, and to provide as monitoring sites for moisture in these sediments. Eighteen porous cup lysimeters were installed in 12 auger holes to collect soil water samples from the surficial sediments. Fourteen auger holes were instrumented with tensiometers, gypsum blocks and/or psychrometers at various depths throughout the RWMC. Readings from these instruments are taken on a monthly basis

Level II scour analysis for Bridge 30, (HUNTTH00220030), on Town Highway 22, crossing Brush Brook, Huntington, Vermont

Science.gov (United States)

Burns, Ronda L.

1997-01-01

This report provides the results of a detailed Level II analysis of scour potential at structure HUNTTH00220030 on Town Highway 22 crossing Brush Brook, Huntington, Vermont (figures 1–8). A Level II study is a basic engineering analysis of the site, including a quantitative analysis of stream stability and scour (U.S. Department of Transportation, 1993). Results of a Level I scour investigation also are included in Appendix E of this report. A Level I investigation provides a qualitative geomorphic characterization of the study site. Information on the bridge, gleaned from Vermont Agency of Transportation (VTAOT) files, was compiled prior to conducting Level I and Level II analyses and is found in Appendix D.

Parameterizing road construction in route-based road weather models: can ground-penetrating radar provide any answers?

International Nuclear Information System (INIS)

Hammond, D S; Chapman, L; Thornes, J E

2011-01-01

A ground-penetrating radar (GPR) survey of a 32 km mixed urban and rural study route is undertaken to assess the usefulness of GPR as a tool for parameterizing road construction in a route-based road weather forecast model. It is shown that GPR can easily identify even the smallest of bridges along the route, which previous thermal mapping surveys have identified as thermal singularities with implications for winter road maintenance. Using individual GPR traces measured at each forecast point along the route, an inflexion point detection algorithm attempts to identify the depth of the uppermost subsurface layers at each forecast point for use in a road weather model instead of existing ordinal road-type classifications. This approach has the potential to allow high resolution modelling of road construction and bridge decks on a scale previously not possible within a road weather model, but initial results reveal that significant future research will be required to unlock the full potential that this technology can bring to the road weather industry. (technical design note)

SUBSURFACE REPOSITORY INTEGRATED CONTROL SYSTEM DESIGN

International Nuclear Information System (INIS)

Randle, D.C.

2000-01-01

The primary purpose of this document is to develop a preliminary high-level functional and physical control system architecture for the potential repository at Yucca Mountain. This document outlines an overall control system concept that encompasses and integrates the many diverse process and communication systems being developed for the subsurface repository design. This document presents integrated design concepts for monitoring and controlling the diverse set of subsurface operations. The Subsurface Repository Integrated Control System design will be composed of a series of diverse process systems and communication networks. The subsurface repository design contains many systems related to instrumentation and control (I andC) for both repository development and waste emplacement operations. These systems include waste emplacement, waste retrieval, ventilation, radiological and air monitoring, rail transportation, construction development, utility systems (electrical, lighting, water, compressed air, etc.), fire protection, backfill emplacement, and performance confirmation. Each of these systems involves some level of I andC and will typically be integrated over a data communications network throughout the subsurface facility. The subsurface I andC systems will also interface with multiple surface-based systems such as site operations, rail transportation, security and safeguards, and electrical/piped utilities. In addition to the I andC systems, the subsurface repository design also contains systems related to voice and video communications. The components for each of these systems will be distributed and linked over voice and video communication networks throughout the subsurface facility. The scope and primary objectives of this design analysis are to: (1) Identify preliminary system-level functions and interfaces (Section 6.2). (2) Examine the overall system complexity and determine how and on what levels the engineered process systems will be monitored

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.

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...

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

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...

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

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

Subsurface clade of Geobacteraceae that predominates in a diversity of Fe(III)-reducing subsurface environments

Science.gov (United States)

Holmes, Dawn E.; O'Neil, Regina A.; Vrionis, Helen A.; N'Guessan, Lucie A.; Ortiz-Bernad, Irene; Larrahondo, Maria J.; Adams, Lorrie A.; Ward, Joy A.; Nicoll , Julie S.; Nevin, Kelly P.; Chavan, Milind A.; Johnson, Jessica P.; Long, Philip E.; Lovely, Derek R.

2007-01-01

There are distinct differences in the physiology of Geobacter species available in pure culture. Therefore, to understand the ecology of Geobacter species in subsurface environments, it is important to know which species predominate. Clone libraries were assembled with 16S rRNA genes and transcripts amplified from three subsurface environments in which Geobacter species are known to be important members of the microbial community: (1) a uranium-contaminated aquifer located in Rifle, CO, USA undergoing in situ bioremediation; (2) an acetate-impacted aquifer that serves as an analog for the long-term acetate amendments proposed for in situ uranium bioremediation and (3) a petroleum-contaminated aquifer in which Geobacter species play a role in the oxidation of aromatic hydrocarbons coupled with the reduction of Fe(III). The majority of Geobacteraceae 16S rRNA sequences found in these environments clustered in a phylogenetically coherent subsurface clade, which also contains a number of Geobacter species isolated from subsurface environments. Concatamers constructed with 43 Geobacter genes amplified from these sites also clustered within this subsurface clade. 16S rRNA transcript and gene sequences in the sediments and groundwater at the Rifle site were highly similar, suggesting that sampling groundwater via monitoring wells can recover the most active Geobacter species. These results suggest that further study of Geobacter species in the subsurface clade is necessary to accurately model the behavior of Geobacter species during subsurface bioremediation of metal and organic contaminants.

Surface/subsurface observation and removal mechanisms of ground reaction bonded silicon carbide

Science.gov (United States)

Yao, Wang; Zhang, Yu-Min; Han, Jie-cai; Zhang, Yun-long; Zhang, Jian-han; Zhou, Yu-feng; Han, Yuan-yuan

2006-01-01

Reaction Bonded Silicon Carbide (RBSiC) has long been recognized as a promising material for optical applications because of its unique combination of favorable properties and low-cost fabrication. Grinding of silicon carbide is difficult because of its high hardness and brittleness. Grinding often induces surface and subsurface damage, residual stress and other types of damage, which have great influence on the ceramic components for optical application. In this paper, surface integrity, subsurface damage and material removal mechanisms of RBSiC ground using diamond grinding wheel on creep-feed surface grinding machine are investigated. The surface and subsurface are studied with scanning electron microscopy (SEM) and optical microscopy. The effects of grinding conditions on surface and subsurface damage are discussed. This research links the surface roughness, surface and subsurface cracks to grinding parameters and provides valuable insights into the material removal mechanism and the dependence of grind induced damage on grinding conditions.

Routing and scheduling problems

DEFF Research Database (Denmark)

Reinhardt, Line Blander

couple of decades. To deliver competitive service and price, transportation today needs to be cost effective. A company requiring for things to be shipped will aim at having the freight shipped as cheaply as possible while often satisfying certain time constraints. For the transportation company......, the effectiveness of the network is of importance aiming at satisfying as many costumer demands as possible at a low cost. Routing represent a path between locations such as an origin and destination for the object routed. Sometimes routing has a time dimension as well as the physical paths. This may...... set cost making the cost of the individual vehicle routes inter-dependant. Depending on the problem type, the size of the problems and time available for solving, different solution methods can be applicable. In this thesis both heuristic methods and several exact methods are investigated depending...

The Serpentinite Subsurface Microbiome

Science.gov (United States)

Schrenk, M. O.; Nelson, B. Y.; Brazelton, W. J.

2011-12-01

Microbial habitats hosted in ultramafic rocks constitute substantial, globally-distributed portions of the subsurface biosphere, occurring both on the continents and beneath the seafloor. The aqueous alteration of ultramafics, in a process known as serpentinization, creates energy rich, high pH conditions, with low concentrations of inorganic carbon which place fundamental constraints upon microbial metabolism and physiology. Despite their importance, very few studies have attempted to directly access and quantify microbial activities and distributions in the serpentinite subsurface microbiome. We have initiated microbiological studies of subsurface seeps and rocks at three separate continental sites of serpentinization in Newfoundland, Italy, and California and compared these results to previous analyses of the Lost City field, near the Mid-Atlantic Ridge. In all cases, microbial cell densities in seep fluids are extremely low, ranging from approximately 100,000 to less than 1,000 cells per milliliter. Culture-independent analyses of 16S rRNA genes revealed low-diversity microbial communities related to Gram-positive Firmicutes and hydrogen-oxidizing bacteria. Interestingly, unlike Lost City, there has been little evidence for significant archaeal populations in the continental subsurface to date. Culturing studies at the sites yielded numerous alkaliphilic isolates on nutrient-rich agar and putative iron-reducing bacteria in anaerobic incubations, many of which are related to known alkaliphilic and subsurface isolates. Finally, metagenomic data reinforce the culturing results, indicating the presence of genes associated with organotrophy, hydrogen oxidation, and iron reduction in seep fluid samples. Our data provide insight into the lifestyles of serpentinite subsurface microbial populations and targets for future quantitative exploration using both biochemical and geochemical approaches.

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)

Consideration of impact of atmospheric intrusion in subsurface sampling for investigation of suspected underground nuclear explosions

International Nuclear Information System (INIS)

Lowrey, J.D.; Bowyer, T.W.; Haas, D.A.; Hayes, J.C.; Biegalski, S.R.

2016-01-01

Radioactive noble gases radioxenon and radioargon constitute the primary smoking gun of an underground nuclear explosion. The aim of subsurface sampling of soil gas as part of an on-site inspection (OSI) is to search for evidence of a suspected underground nuclear event. It has been hypothesized that atmospheric gas can disturb soil gas concentrations and therefore potentially add to problems in civilian source discrimination verifying treaty compliance under the comprehensive nuclear-test ban treaty. This work describes a study of intrusion of atmospheric air into the subsurface and its potential impact on an OSI using results of simulations from the underground transport of environmental xenon (UTEX) model. (author)

A preliminary investigation of vertical crustal movements in the United Kingdom in the context of subsurface nuclear waste isolation

International Nuclear Information System (INIS)

1981-03-01

Two types of change will influence the environment of a subsurface nuclear waste isolation facility: natural geological changes and changes caused by the construction of the waste repository and introduction of the waste. This report is concerned with vertical crustal movements, which are an expression of natural geological changes. Vertical crustal movements observed outside the United Kingdom are reviewed, and vertical movements in a test region of the UK investigated by comparison of geodetic levellings. The implications of vertical crustal movement to waste isolation facilities and some potentially valuable lines of research are discussed. (author)

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....

Pharmacokinetics of Alternative Administration Routes of Melatonin

DEFF Research Database (Denmark)

Zetner, D.; Andersen, L. P.H.; Rosenberg, J.

2016-01-01

Background: Melatonin is traditionally administered orally but has a poor and variable bioavailability. This study aims to present an overview of studies investigating the pharmacokinetics of alternative administration routes of melatonin. Methods: A systematic literature search was performed...... and included experimental or clinical studies, investigating pharmacokinetics of alternative administration routes of melatonin in vivo. Alternative administration routes were defined as all administration routes except oral and intravenous. Results: 10 studies were included in the review. Intranasal....... Subcutaneous injection of melatonin displayed a rapid absorption rate compared to oral administration. Conclusion: Intranasal administration of melatonin has a large potential, and more research in humans is warranted. Transdermal application of melatonin has a possible use in a local application, due to slow...

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...

Frequency band adjustment match filtering based on variable frequency GPR antennas pairing scheme for shallow subsurface investigations

Science.gov (United States)

Shaikh, Shahid Ali; Tian, Gang; Shi, Zhanjie; Zhao, Wenke; Junejo, S. A.

2018-02-01

Ground penetrating Radar (GPR) is an efficient tool for subsurface geophysical investigations, particularly at shallow depths. The non-destructiveness, cost efficiency, and data reliability are the important factors that make it an ideal tool for the shallow subsurface investigations. Present study encompasses; variations in central frequency of transmitting and receiving GPR antennas (Tx-Rx) have been analyzed and frequency band adjustment match filters are fabricated and tested accordingly. Normally, the frequency of both the antennas remains similar to each other whereas in this study we have experimentally changed the frequencies of Tx-Rx and deduce the response. Instead of normally adopted three pairs, a total of nine Tx-Rx pairs were made from 50 MHz, 100 MHz, and 200 MHz antennas. The experimental data was acquired at the designated near surface geophysics test site of the Zhejiang University, Hangzhou, China. After the impulse response analysis of acquired data through conventional as well as varied Tx-Rx pairs, different swap effects were observed. The frequency band and exploration depth are influenced by transmitting frequencies rather than the receiving frequencies. The impact of receiving frequencies was noticed on the resolution; the more noises were observed using the combination of high frequency transmitting with respect to low frequency receiving. On the basis of above said variable results we have fabricated two frequency band adjustment match filters, the constant frequency transmitting (CFT) and the variable frequency transmitting (VFT) frequency band adjustment match filters. By the principle, the lower and higher frequency components were matched and then incorporated with intermediate one. Therefore, this study reveals that a Tx-Rx combination of low frequency transmitting with high frequency receiving is a better choice. Moreover, both the filters provide better radargram than raw one, the result of VFT frequency band adjustment filter is

Nitrogen patterns in subsurface waters of the Yzeron stream: effect of combined sewer overflows and subsurface-surface water mixing.

Science.gov (United States)

Aucour, A M; Bariac, T; Breil, P; Namour, P; Schmitt, L; Gnouma, R; Zuddas, P

2013-01-01

Urbanization subjects streams to increased nitrogen loads. Therefore studying nitrogen forms at the interface between urban stream and groundwater is important for water resource management. In this study we report results on water δ(18)O and nitrogen forms in subsurface waters of a stream (Yzeron, France). The sites studied were located upstream and downstream of combined sewer overflows (CSO) in a rural area and a periurban area, respectively. Water δ(18)O allowed us to follow the mixing of subsurface water with surface water. Dissolved organic nitrogen and organic carbon of fine sediment increased by 20-30% between rural and periurban subsurface waters in the cold season, under high flow. The highest nitrate levels were observed in rural subsurface waters in the cold season. The lowest nitrate levels were found in periurban subsurface waters in the warm season, under low flow. They corresponded to slow exchange of subsurface waters with channel water. Thus reduced exchange between surface and subsurface waters and organic-matter-rich input seemed to favor nitrate reduction in the downstream, periurban, subsurface waters impacted by CSO.

Is Entrepreneurship a Route Out of Deprivation?

DEFF Research Database (Denmark)

Frankish, Julian S.; Roberts, Richard G.; Coad, Alexander Jean-Luc

2014-01-01

Frankish J. S., Roberts R. G., Coad A. and Storey D. J. Is entrepreneurship a route out of deprivation?, Regional Studies. This paper investigates whether entrepreneurship constitutes a route out of deprivation for those living in deprived areas. The measure of income/wealth used is based...... the wealth distribution. Hence, entrepreneurship can be a route out of deprivation....

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.

DETERMINATION OF IMPORTANCE EVALUATION FOR THE SUBSURFACE EXPLORATORY STUDIES FACILITY

International Nuclear Information System (INIS)

Clark, W.J.

1999-01-01

This Determination of Importance Evaluation (DIE) applies to the Subsurface Exploratory Studies Facility (ESF), encompassing the Topopah Spring (TS) Loop from Station 0+00 meters (m) at the North Portal to breakthrough at the South Portal (approximately 78+77 m), the Enhanced Characterization of the Repository Block (ECRB) East-West Cross Drift Starter Tunnel (to approximate ECRB Station 0+26 m), and ancillary test and operation support areas in the TS Loop. This evaluation applies to the construction, operation, and maintenance of these excavations. A more detailed description of these items is provided in Section 6.0. Testing activities are not evaluated in this DIE. Certain construction activities with respect to testing activities are evaluated; but the testing activities themselves are not evaluated. The DIE for ESF Subsurface Testing Activities (BAJ3000000-01717-2200-000111) (CRWMS M and O 1998a) evaluates Subsurface ESF Testing activities. The construction, operation, and maintenance of the TS Loop niches and alcove slot cuts is evaluated herein and is also discussed in CRWMS M and O 1998a. The construction, operation, and maintenance of the Busted Butte subsurface test area in support of the Unsaturated Zone (UZ) Transport Test is evaluated in CRWMS M and O 1998a. Potential test-to-test interference and the waste isolation impacts of testing activities are evaluated in the ESF Subsurface Testing Activities DIE and other applicable evaluation(s) for the Job Package (JP), Test Planning Package (TPP), and/or Field Work Package (FWP). The objectives of this DIE are to determine whether the Subsurface ESF TS Loop and associated excavations, including activities associated with their construction and operation, potentially impact site characterization testing or the waste isolation capabilities of the site. Controls needed to limit any potential impacts are identified. The validity and veracity of the individual tests, including data collection, are the

DETERMINATION OF IMPORTANCE EVALUATION FOR THE SUBSURFACE EXPORATORY STUDIES FACILITY

Energy Technology Data Exchange (ETDEWEB)

W.J. Clark

1999-06-28

This Determination of Importance Evaluation (DIE) applies to the Subsurface Exploratory Studies Facility (ESF), encompassing the Topopah Spring (TS) Loop from Station 0+00 meters (m) at the North Portal to breakthrough at the South Portal (approximately 78+77 m), the Enhanced Characterization of the Repository Block (ECRB) East-West Cross Drift Starter Tunnel (to approximate ECRB Station 0+26 m), and ancillary test and operation support areas in the TS Loop. This evaluation applies to the construction, operation, and maintenance of these excavations. A more detailed description of these items is provided in Section 6.0. Testing activities are not evaluated in this DIE. Certain construction activities with respect to testing activities are evaluated; but the testing activities themselves are not evaluated. The DIE for ESF Subsurface Testing Activities (BAJ3000000-01717-2200-00011 Rev 01) (CRWMS M&O 1998a) evaluates Subsurface ESF Testing activities. The construction, operation, and maintenance of the TS Loop niches and alcove slot cuts is evaluated herein and is also discussed in CRWMS M&O 1998a. The construction, operation, and maintenance of the Busted Butte subsurface test area in support of the Unsaturated Zone (UZ) Transport Test is evaluated in CRWMS M&O 1998a. Potential test-to-test interference and the waste isolation impacts of testing activities are evaluated in the ESF Subsurface Testing Activities DIE and other applicable evaluation(s) for the Job Package (JP), Test Planning Package (TPP), and/or Field Work Package (FWP). The objectives of this DIE are to determine whether the Subsurface ESF TS Loop and associated excavations, including activities associated with their construction and operation, potentially impact site characterization testing or the waste isolation capabilities of the site. Controls needed to limit any potential impacts are identified. The validity and veracity of the individual tests, including data collection, are the responsibility

A study of routing algorithms for SCI-Based multistage networks

International Nuclear Information System (INIS)

Wu Bin; Kristiansen, E.; Skaali, B.; Bogaerts, A.; )

1994-03-01

The report deals with a particular class of multistage Scalable Coherent Interface (SCI) network systems and two important routing algorithms, namely self-routing and table-look up routing. The effect of routing delay on system performance is investigated by simulations. Adaptive routing and deadlock-free routing are studied. 8 refs., 11 figs., 1 tab

Enamel subsurface damage due to tooth preparation with diamonds.

Science.gov (United States)

Xu, H H; Kelly, J R; Jahanmir, S; Thompson, V P; Rekow, E D

1997-10-01

In clinical tooth preparation with diamond burs, sharp diamond particles indent and scratch the enamel, causing material removal. Such operations may produce subsurface damage in enamel. However, little information is available on the mechanisms and the extent of subsurface damage in enamel produced during clinical tooth preparation. The aim of this study, therefore, was to investigate the mechanisms of subsurface damage produced in enamel during tooth preparation by means of diamond burs, and to examine the dependence of such damage on enamel rod orientation, diamond particle size, and removal rate. Subsurface damage was evaluated by a bonded-interface technique. Tooth preparation was carried out on two enamel rod orientations, with four clinical diamond burs (coarse, medium, fine, and superfine) used in a dental handpiece. The results of this study showed that subsurface damage in enamel took the form of median-type cracks and distributed microcracks, extending preferentially along the boundaries between the enamel rods. Microcracks within individual enamel rods were also observed. The median-type cracks were significantly longer in the direction parallel to the enamel rods than perpendicular to the rods. Preparation with the coarse diamond bur produced cracks as deep as 84 +/- 30 microns in enamel. Finishing with fine diamond burs was effective in crack removal. The crack lengths in enamel were not significantly different when the removal rate was varied. Based on these results, it is concluded that subsurface damage in enamel induced by tooth preparation takes the form of median-type cracks as well as inter- and intra-rod microcracks, and that the lengths of these cracks are sensitive to diamond particle size and enamel rod orientation, but insensitive to removal rate.

Linking Chaotic Advection with Subsurface Biogeochemical Processes

Science.gov (United States)

Mays, D. C.; Freedman, V. L.; White, S. K.; Fang, Y.; Neupauer, R.

2017-12-01

This work investigates the extent to which groundwater flow kinematics drive subsurface biogeochemical processes. In terms of groundwater flow kinematics, we consider chaotic advection, whose essential ingredient is stretching and folding of plumes. Chaotic advection is appealing within the context of groundwater remediation because it has been shown to optimize plume spreading in the laminar flows characteristic of aquifers. In terms of subsurface biogeochemical processes, we consider an existing model for microbially-mediated reduction of relatively mobile uranium(VI) to relatively immobile uranium(IV) following injection of acetate into a floodplain aquifer beneath a former uranium mill in Rifle, Colorado. This model has been implemented in the reactive transport code eSTOMP, the massively parallel version of STOMP (Subsurface Transport Over Multiple Phases). This presentation will report preliminary numerical simulations in which the hydraulic boundary conditions in the eSTOMP model are manipulated to simulate chaotic advection resulting from engineered injection and extraction of water through a manifold of wells surrounding the plume of injected acetate. This approach provides an avenue to simulate the impact of chaotic advection within the existing framework of the eSTOMP code.

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

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...

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.

Patterns and drivers of bacterial α- and β-diversity across vertical profiles from surface to subsurface sediments.

Science.gov (United States)

Luna, Gian Marco; Corinaldesi, Cinzia; Rastelli, Eugenio; Danovaro, Roberto

2013-10-01

We investigated the patterns and drivers of bacterial α- and β-diversity, along with viral and prokaryotic abundance and the carbon production rates, in marine surface and subsurface sediments (down to 1 m depth) in two habitats: vegetated sediments (seagrass meadow) and non-vegetated sediments. Prokaryotic abundance and production decreased with depth in the sediment, but cell-specific production rates and the virus-to-prokaryote ratio increased, highlighting unexpectedly high activity in the subsurface. The highest diversity was observed in vegetated sediments. Bacterial β-diversity between sediment horizons was high, and only a minor number of taxa was shared between surface and subsurface layers. Viruses significantly contributed to explain α- and β-diversity patterns. Despite potential limitations due to the only use of fingerprinting techniques, this study indicates that the coastal subsurface host highly active and diversified bacterial assemblages, that subsurface cells are more active than expected and that viruses promote β-diversity and stimulate bacterial metabolism in subsurface layers. The limited number of taxa shared between habitats, and between surface and subsurface sediment horizons, suggests that future investigations of the shallow subsurface will provide insights into the census of bacterial diversity, and the comprehension of the patterns and drivers of prokaryotic diversity in marine ecosystems. © 2013 John Wiley & Sons Ltd and Society for Applied Microbiology.

Great differences in the critical erosion threshold between surface and subsurface sediments: A field investigation of an intertidal mudflat, Jiangsu, China

Science.gov (United States)

Shi, Benwei; Wang, Ya Ping; Wang, Li Hua; Li, Peng; Gao, Jianhua; Xing, Fei; Chen, Jing Dong

2018-06-01

Understanding of bottom sediment erodibility is necessary for the sustainable management and protection of coastlines, and is of great importance for numerical models of sediment dynamics and transport. To investigate the dependence of sediment erodibility on degree of consolidation, we measured turbidity, waves, tidal currents, intratidal bed-level changes, and sediment properties on an exposed macrotidal mudflat during a series of tidal cycles. We estimated the water content of surface sediments (in the uppermost 2 cm of sediment) and sub-surface sediments (at 2 cm below the sediment surface). Bed shear stress values due to currents (τc), waves (τw), and combined current-wave action (τcw) were calculated using a hydrodynamic model. In this study, we estimate the critical shear stress for erosion using two approaches and both of them give similar results. We found that the critical shear stress for erosion (τce) was 0.17-0.18 N/m2 in the uppermost 0-2 cm of sediment and 0.29 N/m2 in sub-surface sediment layers (depth, 2 cm), as determined by time series of τcw values and intratidal bed-level changes, and values of τce, obtained using the water content of bottom sediments, were 0.16 N/m2 in the uppermost 2 cm and 0.28 N/m2 in the sub-surface (depth, 2 cm) sediment. These results indicate that the value of τce for sub-surface sediments (depth, 2 cm) is much greater than that for the uppermost sediments (depth, 0-2 cm), and that the τce value is mainly related to the water content, which is determined by the extent of consolidation. Our results have implications for improving the predictive accuracy of models of sediment transport and morphological evolution, by introducing variable τce values for corresponding sediment layers, and can also provide a mechanistic understanding of bottom sediment erodibility at different sediment depths on intertidal mudflats, as related to differences in the consolidation time.

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

Ma_MISS on ExoMars: Mineralogical Characterization of the Martian Subsurface

Science.gov (United States)

De Sanctis, Maria Cristina; Altieri, Francesca; Ammannito, Eleonora; Biondi, David; De Angelis, Simone; Meini, Marco; Mondello, Giuseppe; Novi, Samuele; Paolinetti, Riccardo; Soldani, Massimo; Mugnuolo, Raffaele; Pirrotta, Simone; Vago, Jorge L.; Ma_MISS Team

2017-07-01

The Ma_MISS (Mars Multispectral Imager for Subsurface Studies) experiment is the visible and near infrared (VNIR) miniaturized spectrometer hosted by the drill system of the ExoMars 2020 rover. Ma_MISS will perform IR spectral reflectance investigations in the 0.4-2.2 μm range to characterize the mineralogy of excavated borehole walls at different depths (between 0 and 2 m). The spectral sampling is about 20 nm, whereas the spatial resolution over the target is 120 μm. Making use of the drill's movement, the instrument slit can scan a ring and build up hyperspectral images of a borehole. The main goal of the Ma_MISS instrument is to study the martian subsurface environment. Access to the martian subsurface is crucial to our ability to constrain the nature, timing, and duration of alteration and sedimentation processes on Mars, as well as habitability conditions. Subsurface deposits likely host and preserve H2O ice and hydrated materials that will contribute to our understanding of the H2O geochemical environment (both in the liquid and in the solid state) at the ExoMars 2020 landing site. The Ma_MISS spectral range and sampling capabilities have been carefully selected to allow the study of minerals and ices in situ before the collection of samples. Ma_MISS will be implemented to accomplish the following scientific objectives: (1) determine the composition of subsurface materials, (2) map the distribution of subsurface H2O and volatiles, (3) characterize important optical and physical properties of materials (e.g., grain size), and (4) produce a stratigraphic column that will inform with regard to subsurface geological processes. The Ma_MISS findings will help to refine essential criteria that will aid in our selection of the most interesting subsurface formations from which to collect samples.

Routing Protocols for Underwater Wireless Sensor Networks: Taxonomy, Research Challenges, Routing Strategies and Future Directions.

Science.gov (United States)

Khan, Anwar; Ali, Ihsan; Ghani, Abdullah; Khan, Nawsher; Alsaqer, Mohammed; Rahman, Atiq Ur; Mahmood, Hasan

2018-05-18

Recent research in underwater wireless sensor networks (UWSNs) has gained the attention of researchers in academia and industry for a number of applications. They include disaster and earthquake prediction, water quality and environment monitoring, leakage and mine detection, military surveillance and underwater navigation. However, the aquatic medium is associated with a number of limitations and challenges: long multipath delay, high interference and noise, harsh environment, low bandwidth and limited battery life of the sensor nodes. These challenges demand research techniques and strategies to be overcome in an efficient and effective fashion. The design of routing protocols for UWSNs is one of the promising solutions to cope with these challenges. This paper presents a survey of the routing protocols for UWSNs. For the ease of description, the addressed routing protocols are classified into two groups: localization-based and localization-free protocols. These groups are further subdivided according to the problems they address or the major parameters they consider during routing. Unlike the existing surveys, this survey considers only the latest and state-of-the-art routing protocols. In addition, every protocol is described in terms of its routing strategy and the problem it addresses and solves. The merit(s) of each protocol is (are) highlighted along with the cost. A description of the protocols in this fashion has a number of advantages for researchers, as compared to the existing surveys. Firstly, the description of the routing strategy of each protocol makes its routing operation easily understandable. Secondly, the demerit(s) of a protocol provides (provide) insight into overcoming its flaw(s) in future investigation. This, in turn, leads to the foundation of new protocols that are more intelligent, robust and efficient with respect to the desired parameters. Thirdly, a protocol can be selected for the appropriate application based on its described

The Development of Route Learning in Down Syndrome, Williams Syndrome and Typical Development: Investigations with Virtual Environments

Science.gov (United States)

Purser, Harry R. M.; Farran, Emily K.; Courbois, Yannick; Lemahieu, Axelle; Sockeel, Pascal; Mellier, Daniel; Blades, Mark

2015-01-01

The ability to navigate new environments has a significant impact on the daily life and independence of people with learning difficulties. The aims of this study were to investigate the development of route learning in Down syndrome (N = 50), Williams syndrome (N = 19), and typically developing children between 5 and 11 years old (N = 108); to…

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.

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.

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.

Load Balancing Routing with Bounded Stretch

Directory of Open Access Journals (Sweden)

Chen Siyuan

2010-01-01

Full Text Available Routing in wireless networks has been heavily studied in the last decade. Many routing protocols are based on classic shortest path algorithms. However, shortest path-based routing protocols suffer from uneven load distribution in the network, such as crowed center effect where the center nodes have more load than the nodes in the periphery. Aiming to balance the load, we propose a novel routing method, called Circular Sailing Routing (CSR, which can distribute the traffic more evenly in the network. The proposed method first maps the network onto a sphere via a simple stereographic projection, and then the route decision is made by a newly defined "circular distance" on the sphere instead of the Euclidean distance in the plane. We theoretically prove that for a network, the distance traveled by the packets using CSR is no more than a small constant factor of the minimum (the distance of the shortest path. We also extend CSR to a localized version, Localized CSR, by modifying greedy routing without any additional communication overhead. In addition, we investigate how to design CSR routing for 3D networks. For all proposed methods, we conduct extensive simulations to study their performances and compare them with global shortest path routing or greedy routing in 2D and 3D wireless networks.

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.

Integrated geomechanical modelling for deep subsurface damage

NARCIS (Netherlands)

Wees, J.D. van; Orlic, B.; Zijl, W.; Jongerius, P.; Schreppers, G.J.; Hendriks, M.

2001-01-01

Government, E&P and mining industry increasingly demand fundamental insight and accurate predictions on subsurface and surface deformation and damage due to exploitation of subsurface natural resources, and subsurface storage of energy residues (e.g. CO2). At this moment deformation is difficult to

Subsurface damage mechanism of high speed grinding process in single crystal silicon revealed by atomistic simulations

International Nuclear Information System (INIS)

Li, Jia; Fang, Qihong; Zhang, Liangchi; Liu, Youwen

2015-01-01

Highlights: • Molecular dynamic model of nanoscale high speed grinding of silicon workpiece has been established. • The effect of grinding speed on subsurface damage and grinding surface integrity by analyzing the chip, dislocation movement, and phase transformation during high speed grinding process are thoroughly investigated. • Subsurface damage is studied by the evolution of surface area at first time for more obvious observation on transition from ductile to brittle. • The hydrostatic stress and von Mises stress by the established analytical model are studied subsurface damage mechanism during nanoscale grinding. - Abstract: Three-dimensional molecular dynamics (MD) simulations are performed to investigate the nanoscale grinding process of single crystal silicon using diamond tool. The effect of grinding speed on subsurface damage and grinding surface integrity by analyzing the chip, dislocation movement, and phase transformation are studied. We also establish an analytical model to calculate several important stress fields including hydrostatic stress and von Mises stress for studying subsurface damage mechanism, and obtain the dislocation density on the grinding subsurface. The results show that a higher grinding velocity in machining brittle material silicon causes a larger chip and a higher temperature, and reduces subsurface damage. However, when grinding velocity is above 180 m s −1 , subsurface damage thickness slightly increases because a higher grinding speed leads to the increase in grinding force and temperature, which accelerate dislocation nucleation and motion. Subsurface damage is studied by the evolution of surface area at first time for more obvious observation on transition from ductile to brittle, that provides valuable reference for machining nanometer devices. The von Mises stress and the hydrostatic stress play an important role in the grinding process, and explain the subsurface damage though dislocation mechanism under high

Program overview: Subsurface science program

International Nuclear Information System (INIS)

1994-03-01

The OHER Subsurface Science Program is DOE's core basic research program concerned with subsoils and groundwater. These practices have resulted in contamination by mixtures of organic chemicals, inorganic chemicals, and radionuclides. A primary long-term goal is to provide a foundation of knowledge that will lead to the reduction of environmental risks and to cost-effective cleanup strategies. Since the Program was initiated in 1985, a substantial amount of research in hydrogeology, subsurface microbiology, and the geochemistry of organically complexed radionuclides has been completed, leading to a better understanding of contaminant transport in groundwater and to new insights into microbial distribution and function in the subsurface environments. The Subsurface Science Program focuses on achieving long-term scientific advances that will assist DOE in the following key areas: providing the scientific basis for innovative in situ remediation technologies that are based on a concept of decontamination through benign manipulation of natural systems; understanding the complex mechanisms and process interactions that occur in the subsurface; determining the influence of chemical and geochemical-microbial processes on co-contaminant mobility to reduce environmental risks; improving predictions of contaminant transport that draw on fundamental knowledge of contaminant behavior in the presence of physical and chemical heterogeneities to improve cleanup effectiveness and to predict environmental risks

Routing strategies in traffic network and phase transition in network ...

Indian Academy of Sciences (India)

The dynamics of information traffic over scale-free networks has been investigated systematically. A series of routing strategies of data packets have been proposed, including the local routing strategy, the next-nearest-neighbour routing strategy, and the mixed routing strategy based on local static and dynamic information.

Using lagged dependence to identify (de)coupled surface and subsurface soil moisture values

Science.gov (United States)

Carranza, Coleen D. U.; van der Ploeg, Martine J.; Torfs, Paul J. J. F.

2018-04-01

Recent advances in radar remote sensing popularized the mapping of surface soil moisture at different spatial scales. Surface soil moisture measurements are used in combination with hydrological models to determine subsurface soil moisture values. However, variability of soil moisture across the soil column is important for estimating depth-integrated values, as decoupling between surface and subsurface can occur. In this study, we employ new methods to investigate the occurrence of (de)coupling between surface and subsurface soil moisture. Using time series datasets, lagged dependence was incorporated in assessing (de)coupling with the idea that surface soil moisture conditions will be reflected at the subsurface after a certain delay. The main approach involves the application of a distributed-lag nonlinear model (DLNM) to simultaneously represent both the functional relation and the lag structure in the time series. The results of an exploratory analysis using residuals from a fitted loess function serve as a posteriori information to determine (de)coupled values. Both methods allow for a range of (de)coupled soil moisture values to be quantified. Results provide new insights into the decoupled range as its occurrence among the sites investigated is not limited to dry conditions.

Route-specific analysis for radioactive materials transportation

International Nuclear Information System (INIS)

1986-01-01

This report addresses a methodology for route-specific analysis, of which route-selection is one aspect. Identification and mitigation of specific hazards along a chosen route is another important facet of route-specific analysis. Route-selection and route-specific mitigation are two tools to be used in minimizing the risk of radioactive materials transportation and promoting public confidence. Other tools exist to improve the safety of transportation under the Nuclear Waste Policy Act. Selection of a transportation mode and other, non-route-specific measures, such as improved driver training and improved cask designs, are additional tools to minimize transportation risk and promote public confidence. This report addresses the route-specific analysis tool and does not attempt to evaluate its relative usefulness as compared to other available tools. This report represents a preliminary attempt to develop a route-specific analysis methodlogy. The Western Interstate Energy Board High-Level Waste Committee has formed a Route-Specific Analysis Task Force which will build upon the methodology proposed in this Staff Report. As western states continue to investigate route-specific analysis issues, it is expected that the methodology will evolve into a more refined product representing the views of a larger group of interested parties in the West

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

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...

Integrated geophysical investigations for the delineation of source and subsurface structure associated with hydro-uranium anomaly: A case study from South Purulia Shear Zone (SPSZ), India

Science.gov (United States)

Sharma, S. P.; Biswas, A.

2012-12-01

South Purulia Shear Zone (SPSZ) is an important region for prospecting of uranium mineralization. Geological studies and hydro-uranium anomaly suggest the presence of Uranium deposit around Raghunathpur village which lies about 8 km north of SPSZ. However, detailed geophysical investigations have not been carried out in this region for investigation of uranium mineralization. Since surface signature of uranium mineralization is not depicted near the location, a deeper subsurface source is expected for hydro uranium anomaly. To delineate the subsurface structure and to investigate the origin of hydro-uranium anomaly present in the area, Vertical Electrical Sounding (VES) using Schlumberger array and Gradient Resistivity Profiling (GRP) were performed at different locations along a profile perpendicular to the South Purulia Shear Zone. Apparent resistivity computed from the measured sounding data at various locations shows a continuously increasing trend. As a result, conventional apparent resistivity data is not able to detect the possible source of hydro uranium anomaly. An innovative approach is applied which depicts the apparent conductivity in the subsurface revealed a possible connection from SPSZ to Raghunathpur. On the other hand resistivity profiling data suggests a low resistive zone which is also characterized by low Self-Potential (SP) anomaly zone. Since SPSZ is characterized by the source of uranium mineralization; hydro-uranium anomaly at Raghunathpur is connected with the SPSZ. The conducting zone has been delineated from SPSZ to Raghunathpur at deeper depths which could be uranium bearing. Since the location is also characterized by a low gravity and high magnetic anomaly zone, this conducting zone is likely to be mineralized zone. Keywords: Apparent resistivity; apparent conductivity; Self Potential; Uranium mineralization; shear zone; hydro-uranium anomaly.

Oxygenated fuel (M-85) behavior in the subsurface -- A Laboratory scale investigation

International Nuclear Information System (INIS)

Donaldson, C.R.; Barker, J.F.; Chatzis, I.

1993-01-01

M-85 is an oxygenated fuel consisting of 85% methanol and 15% gasoline. The complete miscibility of methanol with water may lead to a significantly different source behavior and contamination scenario for M-85 relative to a conventional gasoline. A laboratory investigation involving phase equilibria, cosolvency and column experiments was designed to assess the subsurface behavior of M-85 in comparison to API PS-6 gasoline. Liquid-liquid equilibrium determinations have indicated that unlike PS-6, which is essentially immiscible with water, M-85 would behave as a single miscible phase with groundwater under certain conditions. However, as mixing with groundwater proceeded, two distinct, immiscible phases would result. Column experiments involved the injection of a slug of M-85 or PS-6 onto a saturated, packed column of Borden sand. Aqueous BTEX and methanol breakthrough curves were prepared based on column effluent analyses. Subsequent soil sample analyses provided information regarding the residual gasoline phase distributions from each source. The results of the column experiments indicated that the groundwater contamination arising from an M-85 source would be more complex than from PS-6. The plume of dissolved organic compounds migrating from an M-85 source was characterized by a high methanol content front and associated enhanced BTEX concentrations relative to PS-6, due to methanol's cosolubility effects

Riluzole protects Huntington disease patients from brain glucose hypometabolism and grey matter volume loss and increases production of neurotrophins

Energy Technology Data Exchange (ETDEWEB)

Squitieri, Ferdinando; Orobello, Sara; Cannella, Milena; Martino, Tiziana [IRCCS Neuromed, Neurogenetics Unit and Centre for Rare Disease, Pozzilli (Italy); Romanelli, Pantaleo [IRCCS Neuromed, Department of Neurosurgery, Pozzilli (Italy); Giovacchini, Giampiero; Ciarmiello, Andrea [S. Andrea Hospital, Unit of Nuclear Medicine, La Spezia (Italy); Frati, Luigi [University ' ' Sapienza' ' , Department of Experimental Medicine, Rome (Italy); Mansi, Luigi [Second University of Naples, Department of Nuclear Medicine, Naples (Italy)

2009-07-15

Huntington disease (HD) mutation increases gain-of-toxic functions contributing to glutamate-mediated excitotoxicity. Riluzole interferes with glutamatergic neurotransmission, thereby reducing excitotoxicity, enhancing neurite formation in damaged motoneurons and increasing serum concentrations of BDNF, a brain cortex neurotrophin protecting striatal neurons from degeneration. We investigated metabolic and volumetric differences in distinct brain areas between 11 riluzole-treated and 12 placebo-treated patients by MRI and {sup 18}F-fluoro-2-deoxy-d-glucose (FDG) PET scanning, according to fully automated protocols. We also investigated the influence of riluzole on peripheral growth factor blood levels. Placebo-treated patients showed significantly greater proportional volume loss of grey matter and decrease in metabolic FDG uptake than patients treated with riluzole in all cortical areas (p<0.05). The decreased rate of metabolic FDG uptake correlated with worsening clinical scores in placebo-treated patients, compared to those who were treated with riluzole. The progressive decrease in metabolic FDG uptake observed in the frontal, parietal and occipital cortex correlated linearly with the severity of motor scores calculated by Unified Huntington Disease Rating Scale (UHDRS-I) in placebo-treated patients. Similarly, the rate of metabolic changes in the frontal and temporal areas of the brain cortex correlated linearly with worsening behavioural scores calculated by UHDRS-III in the placebo-treated patients. Finally, BDNF and transforming growth factor beta-1 serum levels were significantly higher in patients treated with riluzole. The linear correlation between decreased metabolic FDG uptake and worsening clinical scores in the placebo-treated patients suggests that FDG-PET may be a valuable procedure to assess brain markers of HD. (orig.)

Riluzole protects Huntington disease patients from brain glucose hypometabolism and grey matter volume loss and increases production of neurotrophins

International Nuclear Information System (INIS)

Squitieri, Ferdinando; Orobello, Sara; Cannella, Milena; Martino, Tiziana; Romanelli, Pantaleo; Giovacchini, Giampiero; Ciarmiello, Andrea; Frati, Luigi; Mansi, Luigi

2009-01-01

Huntington disease (HD) mutation increases gain-of-toxic functions contributing to glutamate-mediated excitotoxicity. Riluzole interferes with glutamatergic neurotransmission, thereby reducing excitotoxicity, enhancing neurite formation in damaged motoneurons and increasing serum concentrations of BDNF, a brain cortex neurotrophin protecting striatal neurons from degeneration. We investigated metabolic and volumetric differences in distinct brain areas between 11 riluzole-treated and 12 placebo-treated patients by MRI and 18 F-fluoro-2-deoxy-d-glucose (FDG) PET scanning, according to fully automated protocols. We also investigated the influence of riluzole on peripheral growth factor blood levels. Placebo-treated patients showed significantly greater proportional volume loss of grey matter and decrease in metabolic FDG uptake than patients treated with riluzole in all cortical areas (p<0.05). The decreased rate of metabolic FDG uptake correlated with worsening clinical scores in placebo-treated patients, compared to those who were treated with riluzole. The progressive decrease in metabolic FDG uptake observed in the frontal, parietal and occipital cortex correlated linearly with the severity of motor scores calculated by Unified Huntington Disease Rating Scale (UHDRS-I) in placebo-treated patients. Similarly, the rate of metabolic changes in the frontal and temporal areas of the brain cortex correlated linearly with worsening behavioural scores calculated by UHDRS-III in the placebo-treated patients. Finally, BDNF and transforming growth factor beta-1 serum levels were significantly higher in patients treated with riluzole. The linear correlation between decreased metabolic FDG uptake and worsening clinical scores in the placebo-treated patients suggests that FDG-PET may be a valuable procedure to assess brain markers of HD. (orig.)

3-D numerical investigation of subsurface flow in anisotropic porous media using multipoint flux approximation method

KAUST Repository

Negara, Ardiansyah; Salama, Amgad; Sun, Shuyu

2013-01-01

Anisotropy of hydraulic properties of subsurface geologic formations is an essential feature that has been established as a consequence of the different geologic processes that they undergo during the longer geologic time scale. With respect

Exploring en-route parking type and parking-search route choice

DEFF Research Database (Denmark)

Kaplan, Sigal; Bekhor, Sholomo

2011-01-01

This paper describes the first phase of an on-going research investigating the joint choice of parking type, parking facility and cruising-for-parking route. The importance of this issue derives from the significant share of cruising-for-parking traffic in urban areas, the relevance of parking po...

SUBSURFACE REPOSITORY INTEGRATED CONTROL SYSTEM DESIGN

International Nuclear Information System (INIS)

C.J. Fernado

1998-01-01

The purpose of this document is to develop preliminary high-level functional and physical control system architectures for the proposed subsurface repository at Yucca Mountain. This document outlines overall control system concepts that encompass and integrate the many diverse systems being considered for use within the subsurface repository. This document presents integrated design concepts for monitoring and controlling the diverse set of subsurface operations. The subsurface repository design will be composed of a series of diverse systems that will be integrated to accomplish a set of overall functions and objectives. The subsurface repository contains several Instrumentation and Control (I andC) related systems including: waste emplacement systems, ventilation systems, communication systems, radiation monitoring systems, rail transportation systems, ground control monitoring systems, utility monitoring systems (electrical, lighting, water, compressed air, etc.), fire detection and protection systems, retrieval systems, and performance confirmation systems. Each of these systems involve some level of I andC and will typically be integrated over a data communication network. The subsurface I andC systems will also integrate with multiple surface-based site-wide systems such as emergency response, health physics, security and safeguards, communications, utilities and others. The scope and primary objectives of this analysis are to: (1) Identify preliminary system level functions and interface needs (Presented in the functional diagrams in Section 7.2). (2) Examine the overall system complexity and determine how and on what levels these control systems will be controlled and integrated (Presented in Section 7.2). (3) Develop a preliminary subsurface facility-wide design for an overall control system architecture, and depict this design by a series of control system functional block diagrams (Presented in Section 7.2). (4) Develop a series of physical architectures

SUBSURFACE REPOSITORY INTEGRATED CONTROL SYSTEM DESIGN

Energy Technology Data Exchange (ETDEWEB)

C.J. Fernado

1998-09-17

The purpose of this document is to develop preliminary high-level functional and physical control system architectures for the proposed subsurface repository at Yucca Mountain. This document outlines overall control system concepts that encompass and integrate the many diverse systems being considered for use within the subsurface repository. This document presents integrated design concepts for monitoring and controlling the diverse set of subsurface operations. The subsurface repository design will be composed of a series of diverse systems that will be integrated to accomplish a set of overall functions and objectives. The subsurface repository contains several Instrumentation and Control (I&C) related systems including: waste emplacement systems, ventilation systems, communication systems, radiation monitoring systems, rail transportation systems, ground control monitoring systems, utility monitoring systems (electrical, lighting, water, compressed air, etc.), fire detection and protection systems, retrieval systems, and performance confirmation systems. Each of these systems involve some level of I&C and will typically be integrated over a data communication network. The subsurface I&C systems will also integrate with multiple surface-based site-wide systems such as emergency response, health physics, security and safeguards, communications, utilities and others. The scope and primary objectives of this analysis are to: (1) Identify preliminary system level functions and interface needs (Presented in the functional diagrams in Section 7.2). (2) Examine the overall system complexity and determine how and on what levels these control systems will be controlled and integrated (Presented in Section 7.2). (3) Develop a preliminary subsurface facility-wide design for an overall control system architecture, and depict this design by a series of control system functional block diagrams (Presented in Section 7.2). (4) Develop a series of physical architectures that

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

SUBSURFACE CONSTRUCTION AND DEVELOPMENT ANALYSIS

International Nuclear Information System (INIS)

N.E. Kramer

1998-01-01

The purpose of this analysis is to identify appropriate construction methods and develop a feasible approach for construction and development of the repository subsurface facilities. The objective of this analysis is to support development of the subsurface repository layout for License Application (LA) design. The scope of the analysis for construction and development of the subsurface Repository facilities covers: (1) Excavation methods, including application of knowledge gained from construction of the Exploratory Studies Facility (ESF). (2) Muck removal from excavation headings to the surface. This task will examine ways of preventing interference with other subsurface construction activities. (3) The logistics and equipment for the construction and development rail haulage systems. (4) Impact of ground support installation on excavation and other construction activities. (5) Examination of how drift mapping will be accomplished. (6) Men and materials handling. (7) Installation and removal of construction utilities and ventilation systems. (8) Equipping and finishing of the emplacement drift mains and access ramps to fulfill waste emplacement operational needs. (9) Emplacement drift and access mains and ramps commissioning prior to handover for emplacement operations. (10) Examination of ways to structure the contracts for construction of the repository. (11) Discussion of different construction schemes and how to minimize the schedule risks implicit in those schemes. (12) Surface facilities needed for subsurface construction activities

The Development of 3d Sub-Surface Mapping Scheme and its Application to Martian Lobate Debris Aprons

Science.gov (United States)

Baik, H.; Kim, J.

2017-07-01

The Shallow Subsurface Radar (SHARAD), a sounding radar equipped on the Mars Reconnaissance Orbiter (MRO), has produced highly valuable information about the Martian subsurface. In particular, the complicated substructures of Mars such as polar deposit, pedestal crater and the other geomorphic features involving possible subsurface ice body has been successfully investigated by SHARAD. In this study, we established a 3D subsurface mapping strategy employing the multiple SHARAD profiles. A number of interpretation components of SHARAD signals were integrated into a subsurface mapping scheme using radargram information and topographic data, then applied over a few mid latitude Lobate Debris Aprons (LDAs). From the identified subsurface layers of LDA, and the GIS data base incorporating the other interpretation outcomes, we are expecting to trace the origin of LDAs. Also, the subsurface mapping scheme developed in this study will be further applied to other interesting Martian geological features such as inter crater structures, aeolian deposits and fluvial sediments. To achieve higher precision sub-surface mapping, the clutter simulation employing the high resolution topographic data and the upgraded clustering algorithms assuming multiple sub-surface layers will be also developed.

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

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

Effect of exposure routes on the relationships of lethal toxicity to rats from oral, intravenous, intraperitoneal and intramuscular routes.

Science.gov (United States)

Ning, Zhong H; Long, Shuang; Zhou, Yuan Y; Peng, Zi Y; Sun, Yi N; Chen, Si W; Su, Li M; Zhao, Yuan H

2015-11-01

The lethal toxicity values (log 1/LD(50)) of 527 aliphatic and aromatic compounds in oral, intravenous, intramuscular and intraperitoneal routes were used to investigate the relationships of log 1/LD(50) from different exposure routes. Regression analysis shows that the log 1/LD(50) values are well correlated between intravenous and intraperitoneal or intramuscular injections. However, the correlations between oral and intravenous or intraperitoneal routes are relatively poor. Comparison of the average residuals indicates that intravenous injection is the most sensitive exposure route and oral administration is the least sensitive exposure route. This is attributed to the difference in kinetic process of toxicity testing. The toxic effect of a chemical can be similar or significantly different between exposure routes, depending on the absorption rates of chemicals into blood. Inclusion of hydrophobic parameter and fractions of ionic forms can improve the correlations between intravenous and intraperitoneal or oral routes, but not between intraperitoneal and oral routes. This is due to the differences of absorption rate in different exposure environments from different routes. Several factors, such as experimental uncertainty, metabolism and toxic kinetics, can affect the correlations between intravenous and intraperitoneal or oral routes. Copyright © 2015 Elsevier Inc. All rights reserved.

Applications of electrical resistance tomography to subsurface environmental restoration

Energy Technology Data Exchange (ETDEWEB)

Ramirez, A.L. [Lawrence Livermore National Lab., CA (United States); Daily, W.D.

1994-11-15

We are developing a new imaging technique, Electrical Resistance Tomography (ERT), to map subsurface liquids as flow occurs during natural or clean-up processes and to map geologic structure. Natural processes (such as surface water infiltrating the vadose zone) and man-induced processes (such as tank leaks and clean-up processes such as steam injection), can create changes in a soil`s electrical properties that are readily measured. We have conducted laboratory and a variety of field experiments to investigate the capabilities and limitations of ERT for imaging underground structures and processes. In the last four years we have used ERT to successfully monitor several field processes including: a subsurface steam injection process (for VOC removal), an air injection process (below the water table) for VOC removal, water infiltration through the vadose zone, radio-frequency heating, ohmic heating, and tank and pond leaks. The information derived from ERT can be used by remediation projects to: detect and locate leaks, determine the effectiveness of clean-up processes, select appropriate clean-up alternatives, and to verify the installation and performance of subsurface barriers.

Subsurface ecosystems. Oil triggered life. Opportunities for the petroleum industry

Energy Technology Data Exchange (ETDEWEB)

Van der Kraan, G.M.

2010-10-05

As it is getting more difficult to retrieve oil from the subsurface, there is a renewed interest from the petroleum industry regarding microbial processes in oil-water systems, like oil reservoirs and their associated refineries. Oil fields are specific ecosystems, they are oxygen depleted, contain a variety of hydrocarbons and often have elevated temperatures and pressures. Through human exploitation, active changes in oil field ecosystems are induced. An example is seawater injection to displace oil. Seawater injection causes a decrease in temperature and induces the growth of sulphate reducing bacteria due to the introduction of sulphate and thereby as a consequence the production of harmful H2S. The current idea is that microorganisms detected in, for example, production water from an oil well, hold additional information on the oil field itself and the processes that are occurring in this oil field during exploitation of the field, so-called 'Biomonitoring'. Through the application of 'smart well' technology, viz. clever exploitation of the oil field, more oil can be retrieved from the field. This however requires new information sources from the field itself. Biotechnology might offer an additional information source. Also it is expected that growth of microorganisms in oil field can plug so called 'thief zones' in oil fields, which forces injected water to take an alternative route and thereby displacing more of the oil. This process however has first to be understood on the pore level. This thesis investigates the concept of 'biomonitoring'. To this purpose the microbial community of water and core samples taken from various oil fields, their separation facilities, and other subsurface environments have been investigated with the use of various molecular techniques like denaturing gradient gel electrophoresis (DGGE) and clone library construction of 16S rRNA gene fragments. The presence of several species can be

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.

Single and Multipolarimetric P-Band SAR Tomography of Subsurface Ice Structure

DEFF Research Database (Denmark)

Banda, Francesco; Dall, Jørgen; Tebaldini, Stefano

2016-01-01

In this paper, first results concerning the characterization of the subsurface of ice sheets and glaciers through single and multipolarization synthetic aperture radar (SAR) tomography (TomoSAR) are illustrated. To this aim, the processing of data acquired in the framework of the European Space...... that scattering in the upper layers of glacial subsurface can be achieved up to an extent of about 20–60 m, conditional on the different types of glaciological zone observed. Moreover, clear morphological structures have been found beneath the ice surface at one of the investigated sites....

A molecular dynamics investigation into the mechanisms of subsurface damage and material removal of monocrystalline copper subjected to nanoscale high speed grinding

International Nuclear Information System (INIS)

Li, Jia; Fang, Qihong; Liu, Youwen; Zhang, Liangchi

2014-01-01

This paper investigates the mechanisms of subsurface damage and material removal of monocrystalline copper when it is under a nanoscale high speed grinding of a diamond tip. The analysis was carried out with the aid of three-dimensional molecular dynamics simulations. The key factors that would influence the deformation of the material were carefully explored by analyzing the chip, dislocation movement, and workpiece deformation, which include grinding speed, depth of cut, grid tip radius, crystal orientation and machining angle of copper. An analytical model was also established to predict the emission of partial dislocations during the nanoscale high speed grinding. The investigation showed that a higher grinding velocity, a larger tip radius or a larger depth of cut would result in a larger chipping volume and a greater temperature rise in the copper workpiece. A lower grinding velocity would produce more intrinsic stacking faults. It was also found that the transition of deformation mechanisms depends on the competition between the dislocations and deformation twinning. There is a critical machining angle, at which a higher velocity, a smaller tip radius, or a smaller depth of cut will reduce the subsurface damage and improve the smoothness of a ground surface. The established analytical model showed that the Shockley dislocation emission is most likely to occur with the crystal orientations of (0 0 1)[1 0 0] at 45° angle.

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...

SUBSURFACE EMPLACEMENT TRANSPORTATION SYSTEM

International Nuclear Information System (INIS)

Wilson, T.; Novotny, R.

1999-01-01

The objective of this analysis is to identify issues and criteria that apply to the design of the Subsurface Emplacement Transportation System (SET). The SET consists of the track used by the waste package handling equipment, the conductors and related equipment used to supply electrical power to that equipment, and the instrumentation and controls used to monitor and operate those track and power supply systems. Major considerations of this analysis include: (1) Operational life of the SET; (2) Geometric constraints on the track layout; (3) Operating loads on the track; (4) Environmentally induced loads on the track; (5) Power supply (electrification) requirements; and (6) Instrumentation and control requirements. This analysis will provide the basis for development of the system description document (SDD) for the SET. This analysis also defines the interfaces that need to be considered in the design of the SET. These interfaces include, but are not limited to, the following: (1) Waste handling building; (2) Monitored Geologic Repository (MGR) surface site layout; (3) Waste Emplacement System (WES); (4) Waste Retrieval System (WRS); (5) Ground Control System (GCS); (6) Ex-Container System (XCS); (7) Subsurface Electrical Distribution System (SED); (8) MGR Operations Monitoring and Control System (OMC); (9) Subsurface Facility System (SFS); (10) Subsurface Fire Protection System (SFR); (11) Performance Confirmation Emplacement Drift Monitoring System (PCM); and (12) Backfill Emplacement System (BES)

User habits and multimodal route planning

Directory of Open Access Journals (Sweden)

Géza Katona

2017-10-01

Full Text Available The results of route planning researches are monitored by logistic and automotive industries. The economic aspects of the cost saving are in the focus of the attention. An optimal route could cause time or fuel savings. An effective driving or an optimal route is a good basis to achieve an economical aim. Moreover the spread of new automotive solutions especially in case of electric cars the optimisation has particular significance regarding the limited battery storage. Additionally the autonomous car development could not be neglected. As a result the society could expect safer roads, better space usage and effective resource management. Nevertheless the requirements of users are extremely diverse, which is not negligible. Supporting these aims, in this paper the connection between the multimodal route planning and the user requirements are investigated. The examination is focused to a sensitivity analysis and a survey to evaluate the data and support the settings of a user habit effect to the final route.

An Investigation of Immunogenicity of Chitosan-Based Botulinum Neurotoxin E Binding Domain Recombinant Candidate Vaccine via Mucosal Route

Directory of Open Access Journals (Sweden)

Mohammad Javad Bagheripour

2017-01-01

Full Text Available Background and Objectives: Botulism syndrome is caused by serotypes A-G of neurotoxins of Clostridium genus. Neurotoxin binding domain is an appropriate vaccine candidate due to its immunogenic activity. In this study, the immunogenicity of chitosan-based botulinum neurotoxin E binding domain recombinant candidate vaccine was investigated via mucosal route of administration. Methods: In this experimental study, chitosan nanoparticles containing rBoNT/E protein were synthesized by ionic gelation method and were administered orally and intranasally to mice. After each administration, IgG antibody titer was measured by ELISA method. Finally, all groups were challenged with active botulinum neurotoxin type E. Data were analyzed using Duncan and repeated ANOVA tests. The significance level was considered as p0.05, even intranasal route reduced the immunogenicity.

Subsurface Shielding Source Term Specification Calculation

International Nuclear Information System (INIS)

S.Su

2001-01-01

The purpose of this calculation is to establish appropriate and defensible waste-package radiation source terms for use in repository subsurface shielding design. This calculation supports the shielding design for the waste emplacement and retrieval system, and subsurface facility system. The objective is to identify the limiting waste package and specify its associated source terms including source strengths and energy spectra. Consistent with the Technical Work Plan for Subsurface Design Section FY 01 Work Activities (CRWMS M and O 2001, p. 15), the scope of work includes the following: (1) Review source terms generated by the Waste Package Department (WPD) for various waste forms and waste package types, and compile them for shielding-specific applications. (2) Determine acceptable waste package specific source terms for use in subsurface shielding design, using a reasonable and defensible methodology that is not unduly conservative. This calculation is associated with the engineering and design activity for the waste emplacement and retrieval system, and subsurface facility system. The technical work plan for this calculation is provided in CRWMS M and O 2001. Development and performance of this calculation conforms to the procedure, AP-3.12Q, Calculations

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.

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

[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.

Structure and function of subsurface microbial communities affecting radionuclide transport and bioimmobilization

Energy Technology Data Exchange (ETDEWEB)

Kostka, Joel E. [Florida State Univ., Tallahassee, FL (United States); Prakash, Om [Florida State Univ., Tallahassee, FL (United States); Green, Stefan J. [Florida State Univ., Tallahassee, FL (United States); Akob, Denise [Florida State Univ., Tallahassee, FL (United States); Jasrotia, Puja [Florida State Univ., Tallahassee, FL (United States); Kerkhof, Lee [Rutgers Univ., New Brunswick, NJ (United States); Chin, Kuk-Jeong [Georgia State Univ., Atlanta, GA (United States); Sheth, Mili [Georgia State Univ., Atlanta, GA (United States); Keller, Martin [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Venkateswaran, Amudhan [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States); Elkins, James G. [Univ. of Illinois, Urbana-Champaign, IL (United States); Stucki, Joseph W. [Univ. of Illinois, Urbana-Champaign, IL (United States)

2012-05-01

Our objectives were to: 1) isolate and characterize novel anaerobic prokaryotes from subsurface environments exposed to high levels of mixed contaminants (U(VI), nitrate, sulfate), 2) elucidate the diversity and distribution of metabolically active metal- and nitrate-reducing prokaryotes in subsurface sediments, and 3) determine the biotic and abiotic mechanisms linking electron transport processes (nitrate, Fe(III), and sulfate reduction) to radionuclide reduction and immobilization. Mechanisms of electron transport and U(VI) transformation were examined under near in situ conditions in sediment microcosms and in field investigations. Field sampling was conducted at the Oak Ridge Field Research Center (ORFRC), in Oak Ridge, Tennessee. The ORFRC subsurface is exposed to mixed contamination predominated by uranium and nitrate. In short, we effectively addressed all 3 stated objectives of the project. In particular, we isolated and characterized a large number of novel anaerobes with a high bioremediation potential that can be used as model organisms, and we are now able to quantify the function of subsurface sedimentary microbial communities in situ using state-of-the-art gene expression methods (molecular proxies).

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

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

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

Component-based framework for subsurface simulations

International Nuclear Information System (INIS)

Palmer, B J; Fang, Yilin; Hammond, Glenn; Gurumoorthi, Vidhya

2007-01-01

Simulations in the subsurface environment represent a broad range of phenomena covering an equally broad range of scales. Developing modelling capabilities that can integrate models representing different phenomena acting at different scales present formidable challenges both from the algorithmic and computer science perspective. This paper will describe the development of an integrated framework that will be used to combine different models into a single simulation. Initial work has focused on creating two frameworks, one for performing smooth particle hydrodynamics (SPH) simulations of fluid systems, the other for performing grid-based continuum simulations of reactive subsurface flow. The SPH framework is based on a parallel code developed for doing pore scale simulations, the continuum grid-based framework is based on the STOMP (Subsurface Transport Over Multiple Phases) code developed at PNNL Future work will focus on combining the frameworks together to perform multiscale, multiphysics simulations of reactive subsurface flow

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...

Urban heat islands in the subsurface of German cities

Science.gov (United States)

Menberg, K.; Blum, P.; Zhu, K.; Bayer, P.

2012-04-01

In the subsurface of many cities there are widespread and persistent thermal anomalies (subsurface urban heat islands) that result in a warming of urban aquifers. The reasons for this heating are manifold. Possible heat sources are basements of buildings, leakage of sewage systems, buried district heating networks, re-injection of cooling water and solar irradiation on paved surfaces. In the current study, the reported groundwater temperatures in several German cities, such as Berlin, Munich, Cologne and Karlsruhe, are compared. Available data sets are supplemented by temperature measurements and depth profiles in observation wells. Trend analyses are conducted with time series of groundwater temperatures, and three-dimensional groundwater temperature maps are provided. In all investigated cities, pronounced positive temperature anomalies are present. The distribution of groundwater temperatures appears to be spatially and temporally highly variable. Apparently, the increased heat input into the urban subsurface is controlled by very local and site-specific parameters. In the long-run, the superposition of various heat sources results in an extensive temperature increase. In many cases, the maximum temperature elevation is found close to the city centre. Regional groundwater temperature differences between the city centre and the rural background are up to 5 °C, with local hot spots of even more pronounced anomalies. Particular heat sources, like cooling water injections or case-specific underground constructions, can cause local temperatures > 20°C in the subsurface. Examination of the long-term variations in isotherm maps shows that temperatures have increased by about 1°C in the city, as well as in the rural background areas over the last decades. This increase could be reproduced with trend analysis of temperature data gathered from several groundwater wells. Comparison between groundwater and air temperatures in Karlsruhe, for example, also indicates a

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

Sub-surface laser nanostructuring in stratified metal/dielectric media: a versatile platform towards flexible, durable and large-scale plasmonic writing

International Nuclear Information System (INIS)

Siozios, A; Bellas, D V; Lidorikis, E; Patsalas, P; Kalfagiannis, N; Cranton, W M; Koutsogeorgis, D C; Bazioti, C; Dimitrakopulos, G P; Vourlias, G

2015-01-01

Laser nanostructuring of pure ultrathin metal layers or ceramic/metal composite thin films has emerged as a promising route for the fabrication of plasmonic patterns with applications in information storage, cryptography, and security tagging. However, the environmental sensitivity of pure Ag layers and the complexity of ceramic/metal composite film growth hinder the implementation of this technology to large-scale production, as well as its combination with flexible substrates. In the present work we investigate an alternative pathway, namely, starting from non-plasmonic multilayer metal/dielectric layers, whose growth is compatible with large scale production such as in-line sputtering and roll-to-roll deposition, which are then transformed into plasmonic templates by single-shot UV-laser annealing (LA). This entirely cold, large-scale process leads to a subsurface nanoconstruction involving plasmonic Ag nanoparticles (NPs) embedded in a hard and inert dielectric matrix on top of both rigid and flexible substrates. The subsurface encapsulation of Ag NPs provides durability and long-term stability, while the cold character of LA suits the use of sensitive flexible substrates. The morphology of the final composite film depends primarily on the nanocrystalline character of the dielectric host and its thermal conductivity. We demonstrate the emergence of a localized surface plasmon resonance, and its tunability depending on the applied fluence and environmental pressure. The results are well explained by theoretical photothermal modeling. Overall, our findings qualify the proposed process as an excellent candidate for versatile, large-scale optical encoding applications. (paper)

Determination of Importance Evaluation for Exploratory Studies Facility (ESF) Subsurface Testing Activities

International Nuclear Information System (INIS)

Goodin, S.

2002-01-01

individual tests, including data collection, are the responsibility of the assigned Principal Investigator(s) (PIS) and are not evaluated in this DIE. This DIE focuses on integrating and compiling the evaluations of previous DIEs which were prepared for various ESF subsurface testing activities, including the use of temporary items currently located or being developed for these testing activities (see Table 1. l), and to provide a bounding evaluation for potential future ESF subsurface testing activities that are sufficiently similar to the generic testing activities addressed herein. Subsurface testing activities items/facilities evaluated herein include: ongoing and planned testing in the TS Loop, alcoves, and niches, planned testing in the ECRB Starter Tunnel, borehole drilling and workover, and tracers, fluids, and materials (TFM) usage. Detailed identification of individual testing items/facilities and generic descriptions for subsurface-testing-related activities are provided in Section 6

Quantum chemistry-assisted synthesis route development

International Nuclear Information System (INIS)

Hori, Kenji; Sumimoto, Michinori; Murafuji, Toshihiro

2015-01-01

We have been investigating “quantum chemistry-assisted synthesis route development” using in silico screenings and applied the method to several targets. Another example was conducted to develop synthesis routes for a urea derivative, namely 1-(4-(trifluoromethyl)-2-oxo-2H-chromen-7-yl)urea. While five synthesis routes were examined, only three routes passed the second in silico screening. Among them, the reaction of 7-amino-4-(trifluoromethyl)-2H-chromen-2-one and O-methyl carbamate with BF 3 as an additive was ranked as the first choice for synthetic work. We were able to experimentally obtain the target compound even though its yield was as low as 21 %. The theoretical result was thus consistent with that observed. The summary of transition state data base (TSDB) is also provided. TSDB is the key to reducing time of in silico screenings

Influence of Si wafer thinning processes on (sub)surface defects

Energy Technology Data Exchange (ETDEWEB)

Inoue, Fumihiro, E-mail: fumihiro.inoue@imec.be [Imec, Kapeldreef 75, 3001 Leuven (Belgium); Jourdain, Anne; Peng, Lan; Phommahaxay, Alain; De Vos, Joeri; Rebibis, Kenneth June; Miller, Andy; Sleeckx, Erik; Beyne, Eric [Imec, Kapeldreef 75, 3001 Leuven (Belgium); Uedono, Akira [Division of Applied Physics, Faculty of Pure and Applied Science, University of Tsukuba, Tsukuba, Ibaraki 305-8573 (Japan)

2017-05-15

Highlights: • Mono-vacancy free Si-thinning can be accomplished by combining several thinning techniques. • The grinding damage needs to be removed prior to dry etching, otherwise vacancies remain in the Si at a depth around 0.5 to 2 μm after Si wafer thickness below 5 μm. • The surface of grinding + CMP + dry etching is equivalent mono vacancy level as that of grinding + CMP. - Abstract: Wafer-to-wafer three-dimensional (3D) integration with minimal Si thickness can produce interacting multiple devices with significantly scaled vertical interconnections. Realizing such a thin 3D structure, however, depends critically on the surface and subsurface of the remaining backside Si after the thinning processes. The Si (sub)surface after mechanical grinding has already been characterized fruitfully for a range of few dozen of μm. Here, we expand the characterization of Si (sub)surface to 5 μm thickness after thinning process on dielectric bonded wafers. The subsurface defects and damage layer were investigated after grinding, chemical mechanical polishing (CMP), wet etching and plasma dry etching. The (sub)surface defects were characterized using transmission microscopy, atomic force microscopy, and positron annihilation spectroscopy. Although grinding provides the fastest removal rate of Si, the surface roughness was not compatible with subsequent processing. Furthermore, mechanical damage such as dislocations and amorphous Si cannot be reduced regardless of Si thickness and thin wafer handling systems. The CMP after grinding showed excellent performance to remove this grinding damage, even though the removal amount is 1 μm. For the case of Si thinning towards 5 μm using grinding and CMP, the (sub)surface is atomic scale of roughness without vacancy. For the case of grinding + dry etch, vacancy defects were detected in subsurface around 0.5–2 μm. The finished surface after wet etch remains in the nm scale in the strain region. By inserting a CMP step in

Route-external and route-internal landmarks in route descriptions : Effects of route length and map design

NARCIS (Netherlands)

Westerbeek, Hans; Maes, Alfons

2013-01-01

Landmarks are basic ingredients in route descriptions. They often mark choice points: locations where travellers choose from different options how to continue the route. This study focuses on one of the loose ends in the taxonomy of landmarks. In a memory-based production experiment in which

SUBSURFACE VISUAL ALARM SYSTEM ANALYSIS

International Nuclear Information System (INIS)

D.W. Markman

2001-01-01

The ''Subsurface Fire Hazard Analysis'' (CRWMS M andO 1998, page 61), and the document, ''Title III Evaluation Report for the Surface and Subsurface Communication System'', (CRWMS M andO 1999a, pages 21 and 23), both indicate the installed communication system is adequate to support Exploratory Studies Facility (ESF) activities with the exception of the mine phone system for emergency notification purposes. They recommend the installation of a visual alarm system to supplement the page/party phone system The purpose of this analysis is to identify data communication highway design approaches, and provide justification for the selected or recommended alternatives for the data communication of the subsurface visual alarm system. This analysis is being prepared to document a basis for the design selection of the data communication method. This analysis will briefly describe existing data or voice communication or monitoring systems within the ESF, and look at how these may be revised or adapted to support the needed data highway of the subsurface visual alarm. system. The existing PLC communication system installed in subsurface is providing data communication for alcove No.5 ventilation fans, south portal ventilation fans, bulkhead doors and generator monitoring system. It is given that the data communication of the subsurface visual alarm system will be a digital based system. It is also given that it is most feasible to take advantage of existing systems and equipment and not consider an entirely new data communication system design and installation. The scope and primary objectives of this analysis are to: (1) Briefly review and describe existing available data communication highways or systems within the ESF. (2) Examine technical characteristics of an existing system to disqualify a design alternative is paramount in minimizing the number of and depth of a system review. (3) Apply general engineering design practices or criteria such as relative cost, and degree

Subsurface microbial habitats on Mars

Science.gov (United States)

Boston, P. J.; Mckay, C. P.

1991-01-01

We developed scenarios for shallow and deep subsurface cryptic niches for microbial life on Mars. Such habitats could have considerably prolonged the persistence of life on Mars as surface conditions became increasingly inhospitable. The scenarios rely on geothermal hot spots existing below the near or deep subsurface of Mars. Recent advances in the comparatively new field of deep subsurface microbiology have revealed previously unsuspected rich aerobic and anaerobic microbal communities far below the surface of the Earth. Such habitats, protected from the grim surface conditions on Mars, could receive warmth from below and maintain water in its liquid state. In addition, geothermally or volcanically reduced gases percolating from below through a microbiologically active zone could provide the reducing power needed for a closed or semi-closed microbial ecosystem to thrive.

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

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

Integral Analysis of Seismic Refraction and Ambient Vibration Survey for Subsurface Profile Evaluation

Science.gov (United States)

Hazreek, Z. A. M.; Kamarudin, A. F.; Rosli, S.; Fauziah, A.; Akmal, M. A. K.; Aziman, M.; Azhar, A. T. S.; Ashraf, M. I. M.; Shaylinda, M. Z. N.; Rais, Y.; Ishak, M. F.; Alel, M. N. A.

2018-04-01

Geotechnical site investigation as known as subsurface profile evaluation is the process of subsurface layer characteristics determination which finally used for design and construction phase. Traditionally, site investigation was performed using drilling technique thus suffers from several limitation due to cost, time, data coverage and sustainability. In order to overcome those problems, this study adopted surface techniques using seismic refraction and ambient vibration method for subsurface profile depth evaluation. Seismic refraction data acquisition and processing was performed using ABEM Terraloc and OPTIM software respectively. Meanwhile ambient vibration data acquisition and processing was performed using CityShark II, Lennartz and GEOPSY software respectively. It was found that studied area consist of two layers representing overburden and bedrock geomaterials based on p-wave velocity value (vp = 300 – 2500 m/s and vp > 2500 m/s) and natural frequency value (Fo = 3.37 – 3.90 Hz) analyzed. Further analysis found that both methods show some good similarity in term of depth and thickness with percentage accuracy at 60 – 97%. Consequently, this study has demonstrated that the application of seismic refractin and ambient vibration method was applicable in subsurface profile depth and thickness estimation. Moreover, surface technique which consider as non-destructive method adopted in this study was able to compliment conventional drilling method in term of cost, time, data coverage and environmental sustainaibility.

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.

Routing architecture and security for airborne networks

Science.gov (United States)

Deng, Hongmei; Xie, Peng; Li, Jason; Xu, Roger; Levy, Renato

2009-05-01

Airborne networks are envisioned to provide interconnectivity for terrestial and space networks by interconnecting highly mobile airborne platforms. A number of military applications are expected to be used by the operator, and all these applications require proper routing security support to establish correct route between communicating platforms in a timely manner. As airborne networks somewhat different from traditional wired and wireless networks (e.g., Internet, LAN, WLAN, MANET, etc), security aspects valid in these networks are not fully applicable to airborne networks. Designing an efficient security scheme to protect airborne networks is confronted with new requirements. In this paper, we first identify a candidate routing architecture, which works as an underlying structure for our proposed security scheme. And then we investigate the vulnerabilities and attack models against routing protocols in airborne networks. Based on these studies, we propose an integrated security solution to address routing security issues in airborne networks.

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 ...

Subsurface Investigations Program at the Radioactive Waste Management Complex of the Idaho National Engineering Laboratory: Annual progress report, FY-1987

International Nuclear Information System (INIS)

Laney, P.T.; Minkin, S.C.; Baca, R.G.; McElroy, D.L.; Hubbell, J.M.; Hull, L.C.; Russell, B.F.; Stormberg, G.J.; Pittman, J.T.

1988-04-01

The Subsurface Investigations Program is obtaining program objectives of a field calibration of a model to predict long-term radionuclide migration and measurement of the actual migration to date. Three deep boreholes were drilled at the Radioactive Waste Management Complex (RWMC) to collect sample material for evaluation of radionuclide content in the interbeds, to determine geologic and hydrologic characteristics of the sediments, and to provide monitoring sites for moisture movement in these sediments. Suction lysimeters and heat dissipation sensors were installed in two deep boreholes to collect moisture data. Data from the moisture sensing instruments installed at the RWMC continued to be collected during FY-1987. Because of the large volume of collected data, the RWMC Data Management System was developed and implemented to facilitate the storage, retrieval, and manipulation of the database. Work on the Computer Model Development task focused on a detailed review of previous vadose zone modeling studies at INEL, acquisition and installation of a suite of computer models for unsaturated flow and contaminant transport, and preliminary applications of computer models using site-specific data. Computer models installed on the INEL CRAY computer for modeling transport through the subsurface pathway include SEMTRA, FEMTRA, TRACR3D, MAGNUM, and CHAINT. In addition to the major computer models, eight other codes, referred to as support codes and models, have been acquired and implemented. 27 refs., 70 figs., 22 tabs

Remediation of contaminated subsurface materials by a metal-reducing bacterium

International Nuclear Information System (INIS)

Gorby, Y.A.; Amonette, J.E.; Fruchter, J.S.

1994-11-01

A biotic approach for remediating subsurface sediments and groundwater contaminated with carbon tetrachloride (CT) and chromium was evaluated. Cells of the Fe(iii)-reducing bacterium strain BrY were added to sealed, anoxic flasks containing Hanford groundwater, natural subsurface sediments, and either carbon tetrachloride, CT, or oxidized chromium, Cr(VI). With lactate as the electron donor, BrY transformed CT to chloroform (CF), which accumulated to about 1 0 % of the initial concentration of CT. The remainder of the CT was transformed to unidentified, nonvolatile compounds. Transformation of CT by BrY was an indirect process Cells reduced solid phase Fe(ill) to chemically reactive FE(II) that chemically transformed the chlorinated contaminant. Cr(VI), in contrast, was reduced by a direct enzymatic reaction in the presence or absence of Fe(III)-bearing sediments. These results demonstrate that Fe(ill)-reducing bacteria provide potential for transforming CT and for reducing CR(VI) to less toxic Cr(III). Technologies for stimulating indigenous populations of metal-reducing bacteria or for introducing specific metal-reducing bacteria to the subsurface are being investigated

The Route Analysis Based On Flight Plan

Science.gov (United States)

Feriyanto, Nur; Saleh, Chairul; Fauzi, Achmad; Rachman Dzakiyullah, Nur; Riza Iwaputra, Kahfi

2016-02-01

Economic development effects use of air transportation since the business process in every aspect was increased. Many people these days was prefer using airplane because it can save time and money. This situation also effects flight routes, many airlines offer new routes to deal with competition. Managing flight routes is one of the problems that must be faced in order to find the efficient and effective routes. This paper investigates the best routes based on flight performance by determining the amount of block fuel for the Jakarta-Denpasar flight route. Moreover, in this work compares a two kinds of aircraft and tracks by calculating flight distance, flight time and block fuel. The result shows Jakarta-Denpasar in the Track II has effective and efficient block fuel that can be performed by Airbus 320-200 aircraft. This study can contribute to practice in making an effective decision, especially helping executive management of company due to selecting appropriate aircraft and the track in the flight plan based on the block fuel consumption for business operation.

Routing Service Quality—Local Driver Behavior Versus Routing Services

DEFF Research Database (Denmark)

Ceikute, Vaida; Jensen, Christian S.

2013-01-01

of the quality of one kind of location-based service, namely routing services. Specifically, the paper presents a framework that enables the comparison of the routes provided by routing services with the actual driving behaviors of local drivers. Comparisons include route length, travel time, and also route...... popularity, which are enabled by common driving behaviors found in available trajectory data. The ability to evaluate the quality of routing services enables service providers to improve the quality of their services and enables users to identify the services that best serve their needs. The paper covers......Mobile location-based services is a very successful class of services that are being used frequently by users with GPS-enabled mobile devices such as smartphones. This paper presents a study of how to exploit GPS trajectory data, which is available in increasing volumes, for the assessment...

Mobility Tolerant Firework Routing for Improving Reachability in MANETs

Directory of Open Access Journals (Sweden)

Gen Motoyoshi

2014-03-01

Full Text Available In this paper, we investigate our mobility-assisted and adaptive broadcast routing mechanism, called Mobility Tolerant Firework Routing (MTFR, which utilizes the concept of potentials for routing and improves node reachability, especially in situations with high mobility, by including a broadcast mechanism. We perform detailed evaluations by simulations in a mobile environment and demonstrate the advantages of MTFR over conventional potential-based routing. In particular, we show that MTFR produces better reachability in many aspects at the expense of a small additional transmission delay and intermediate traffic overhead, making MTFR a promising routing protocol and feasible for future mobile Internet infrastructures.

Subsurface Biogeochemistry of Actinides

Energy Technology Data Exchange (ETDEWEB)

Kersting, Annie B. [Lawrence Livermore National Lab. (LLNL), Livermore, CA (United States). Univ. Relations and Science Education; Zavarin, Mavrik [Lawrence Livermore National Lab. (LLNL), Livermore, CA (United States). Glenn T. Seaborg Inst.

2016-06-29

A major scientific challenge in environmental sciences is to identify the dominant processes controlling actinide transport in the environment. It is estimated that currently, over 2200 metric tons of plutonium (Pu) have been deposited in the subsurface worldwide, a number that increases yearly with additional spent nuclear fuel (Ewing et al., 2010). Plutonium has been shown to migrate on the scale of kilometers, giving way to a critical concern that the fundamental biogeochemical processes that control its behavior in the subsurface are not well understood (Kersting et al., 1999; Novikov et al., 2006; Santschi et al., 2002). Neptunium (Np) is less prevalent in the environment; however, it is predicted to be a significant long-term dose contributor in high-level nuclear waste. Our focus on Np chemistry in this Science Plan is intended to help formulate a better understanding of Pu redox transformations in the environment and clarify the differences between the two long-lived actinides. The research approach of our Science Plan combines (1) Fundamental Mechanistic Studies that identify and quantify biogeochemical processes that control actinide behavior in solution and on solids, (2) Field Integration Studies that investigate the transport characteristics of Pu and test our conceptual understanding of actinide transport, and (3) Actinide Research Capabilities that allow us to achieve the objectives of this Scientific Focus Area (SFA and provide new opportunities for advancing actinide environmental chemistry. These three Research Thrusts form the basis of our SFA Science Program (Figure 1).

Yield response and economics of shallow subsurface drip irrigation systems

Science.gov (United States)

Field tests were conducted using shallow subsurface drip irrigation (S3DI) on cotton (Gossypium hirsutum, L.), corn (Zea mays, L.), and peanut (Arachis hypogeae, L.) in rotation to investigate yield potential and economic sustainability of this irrigation system technique over a six year period. Dri...

Aircraft route forecasting under adverse weather conditions

Directory of Open Access Journals (Sweden)

Thomas Hauf

2017-04-01

Full Text Available In this paper storm nowcasts in the terminal manoeuvring area (TMA of Hong Kong International Airport are used to forecast deviation routes through a field of storms for arriving and departing aircraft. Storms were observed and nowcast by the nowcast system SWIRLS from the Hong Kong Observatory. Storms were considered as no-go zones for aircraft and deviation routes were determined with the DIVSIM software package. Two days (21 and 22 May 2011 with 22 actual flown routes were investigated. Flights were simulated with a nowcast issued at the time an aircraft entered the TMA or departed from the airport. These flights were compared with a posteriori simulations, in which all storm fields were known and circumnavigated. Both types of simulated routes were then compared with the actual flown routes. The qualitative comparison of the various routes revealed generally good agreement. Larger differences were found in more complex situations with many active storms in the TMA. Route differences resulted primarily from air traffic control measures imposed such as holdings, slow-downs and shortcuts, causing the largest differences between the estimated and actual landing time. Route differences could be enhanced as aircraft might be forced to circumnavigate a storm ahead in a different sense. The use of route forecasts to assist controllers coordinating flights in a complex moving storm field is discussed. The study emphasises the important application of storm nowcasts in aviation meteorology.

Deep sequencing of subseafloor eukaryotic rRNA reveals active Fungi across marine subsurface provinces.

Directory of Open Access Journals (Sweden)

William Orsi

Full Text Available The deep marine subsurface is a vast habitat for microbial life where cells may live on geologic timescales. Because DNA in sediments may be preserved on long timescales, ribosomal RNA (rRNA is suggested to be a proxy for the active fraction of a microbial community in the subsurface. During an investigation of eukaryotic 18S rRNA by amplicon pyrosequencing, unique profiles of Fungi were found across a range of marine subsurface provinces including ridge flanks, continental margins, and abyssal plains. Subseafloor fungal populations exhibit statistically significant correlations with total organic carbon (TOC, nitrate, sulfide, and dissolved inorganic carbon (DIC. These correlations are supported by terminal restriction length polymorphism (TRFLP analyses of fungal rRNA. Geochemical correlations with fungal pyrosequencing and TRFLP data from this geographically broad sample set suggests environmental selection of active Fungi in the marine subsurface. Within the same dataset, ancient rRNA signatures were recovered from plants and diatoms in marine sediments ranging from 0.03 to 2.7 million years old, suggesting that rRNA from some eukaryotic taxa may be much more stable than previously considered in the marine subsurface.

Determination of Importance Evaluation for Exploratory Studies Facility (ESF) Subsurface Testing Activities

International Nuclear Information System (INIS)

C.J. Byrne

2001-01-01

individual tests, including data collection, are the responsibility of the assigned Principal Investigator(s) (PIS) and are not evaluated in this DIE. This DIE focuses on integrating and compiling the evaluations of previous DIES which were prepared for various ESF subsurface testing activities, including the use of temporary items currently located or being developed for these testing activities, and to provide a bounding evaluation for potential future ESF subsurface testing activities that are sufficiently similar to the generic testing activities addressed herein. Subsurface testing activities items/facilities evaluated herein include: ongoing and planned testing in the TS Loop, alcoves, and niches, planned testing in the ECRB Starter Tunnel, borehole drilling and workover, and tracers, fluids, and materials (TFM) usage. Detailed identification of individual testing items/facilities and generic descriptions for subsurface-testing-related activities are provided in Section 6. The conclusions and requirements of this DIE conservatively bound the conclusions and requirements of previously approved DIES for the ESF subsurface testing activities addressed herein, based on conservative engineering judgment and on concurrence with this DIE (via a formal review process) by the originating and reviewing organizations of the previously approved evaluations. Hence, this DIE supersedes the following DIES listed in Table 1.1

Determination of Importance Evaluation for Exploratory Studies Facility (ESF) Subsurface Testing Activities

Energy Technology Data Exchange (ETDEWEB)

C.J. Byrne

2001-02-20

tests, including data collection, are the responsibility of the assigned Principal Investigator(s) (PIS) and are not evaluated in this DIE. This DIE focuses on integrating and compiling the evaluations of previous DIES which were prepared for various ESF subsurface testing activities, including the use of temporary items currently located or being developed for these testing activities, and to provide a bounding evaluation for potential future ESF subsurface testing activities that are sufficiently similar to the generic testing activities addressed herein. Subsurface testing activities items/facilities evaluated herein include: ongoing and planned testing in the TS Loop, alcoves, and niches, planned testing in the ECRB Starter Tunnel, borehole drilling and workover, and tracers, fluids, and materials (TFM) usage. Detailed identification of individual testing items/facilities and generic descriptions for subsurface-testing-related activities are provided in Section 6. The conclusions and requirements of this DIE conservatively bound the conclusions and requirements of previously approved DIES for the ESF subsurface testing activities addressed herein, based on conservative engineering judgment and on concurrence with this DIE (via a formal review process) by the originating and reviewing organizations of the previously approved evaluations. Hence, this DIE supersedes the following DIES listed in Table 1.1.

Planning Routes Across Economic Terrains: Maximizing Utility, Following Heuristics

Science.gov (United States)

Zhang, Hang; Maddula, Soumya V.; Maloney, Laurence T.

2010-01-01

We designed an economic task to investigate human planning of routes in landscapes where travel in different kinds of terrain incurs different costs. Participants moved their finger across a touch screen from a starting point to a destination. The screen was divided into distinct kinds of terrain and travel within each kind of terrain imposed a cost proportional to distance traveled. We varied costs and spatial configurations of terrains and participants received fixed bonuses minus the total cost of the routes they chose. We first compared performance to a model maximizing gain. All but one of 12 participants failed to adopt least-cost routes and their failure to do so reduced their winnings by about 30% (median value). We tested in detail whether participants’ choices of routes satisfied three necessary conditions (heuristics) for a route to maximize gain. We report failures of one heuristic for 7 out of 12 participants. Last of all, we modeled human performance with the assumption that participants assign subjective utilities to costs and maximize utility. For 7 out 12 participants, the fitted utility function was an accelerating power function of actual cost and for the remaining 5, a decelerating power function. We discuss connections between utility aggregation in route planning and decision under risk. Our task could be adapted to investigate human strategy and optimality of route planning in full-scale landscapes. PMID:21833269

PLANNING ROUTES ACROSS ECONOMIC TERRAINS: MAXIMIZING UTILITY, FOLLOWING HEURISTICS

Directory of Open Access Journals (Sweden)

Hang eZhang

2010-12-01

Full Text Available We designed an economic task to investigate human planning of routes in landscapes where travel in different kinds of terrain incurs different costs. Participants moved their finger across a touch screen from a starting point to a destination. The screen was divided into distinct kinds of terrain and travel within each kind of terrain imposed a cost proportional to distance traveled. We varied costs and spatial configurations of terrains and participants received fixed bonuses minus the total cost of the routes they chose. We first compared performance to a model maximizing gain. All but one of 12 participants failed to adopt least-cost routes and their failure to do so reduced their winnings by about 30% (median value. We tested in detail whether participants’ choices of routes satisfied three necessary conditions (heuristics for a route to maximize gain. We report failures of one heuristic for 7 out of 12 participants. Last of all, we modeled human performance with the assumption that participants assign subjective utilities to costs and maximize utility. For 7 out 12 participants, the fitted utility function was an accelerating power function of actual cost and for the remaining 5, a decelerating power function. We discuss connections between utility aggregation in route planning and decision under risk. Our task could be adapted to investigate human strategy and optimality of route planning in full-scale landscapes.

An electric and electromagnetic geophysical approach for subsurface investigation of anthropogenic mounds in an urban environment

Science.gov (United States)

Pazzi, Veronica; Tapete, Deodato; Cappuccini, Luca; Fanti, Riccardo

2016-11-01

Scientific interest in mounds as geomorphological features that currently represent topographic anomalies in flat urban landscapes mainly lies on the understanding of their origin, either purely natural or anthropogenic. In this second circumstance, another question is whether traces of lost buildings are preserved within the mound subsurface and can be mapped as remnants testifying past settlement. When these landforms have been modified in centuries for civilian use, structural stability is a further element of concern. To address these issues we applied a geophysical approach based on a very low frequency electromagnetic (VLF-EM) technique and two-dimensional electrical resistivity tomography (2D-ERT) and integrated it with well-established surface survey methods within a diagnostic workflow of structural assessment. We demonstrate the practical benefits of this method in the English Cemetery of Florence, Italy, whose mixed nature and history of morphological changes are suggested by archival records. The combination of the two selected geophysical techniques allowed us to overcome the physical obstacles caused by tomb density and to prevent interference from the urban vehicular traffic on the geophysical signals. Eighty-two VLF-EM profiles and five 2D-ERTs were collected to maximise the spatial coverage of the subsurface prospection, while surface indicators of instability (e.g., tomb tilt, location, and direction of ground fractures and wall cracks) were mapped by standard metric survey. High resistive anomalies (> 300 and 400 Ωm) observed in VLF-EM tomographies are attributed to remnants of the ancient perimeter wall that are still buried along the southern side of the mound. While no apparent correlation is found between the causes of tomb and ground movements, the crack pattern map supplements the overall structural assessment. The main outcome is that the northern portion of the retaining wall is classed with the highest hazard rate. The impact of this

Confocal examination of subsurface cracking in ceramic materials.

Science.gov (United States)

Etman, Maged K

2009-10-01

The original ceramic surface finish and its microstructure may have an effect on crack propagation. The purpose of this study was to investigate the relation between crack propagation and ceramic microstructure following cyclic fatigue loading, and to qualitatively evaluate and quantitatively measure the surface and subsurface crack depths of three types of ceramic restorations with different microstructures using a Confocal Laser Scanning Microscope (CLSM) and Scanning Electron Microscope (SEM). Twenty (8 x 4 x 2 mm(3)) blocks of AllCeram (AC), experimental ceramic (EC, IPS e.max Press), and Sensation SL (SSL) were prepared, ten glazed and ten polished of each material. Sixty antagonist enamel specimens were made from the labial surfaces of permanent incisors. The ceramic abraders were attached to a wear machine, so that each enamel specimen presented at 45 degrees to the vertical movement of the abraders, and immersed in artificial saliva. Wear was induced for 80K cycles at 60 cycles/min with a load of 40 N and 2-mm horizontal deflection. The specimens were examined for cracks at baseline, 5K, 10K, 20K, 40K, and 80K cycles. Twenty- to 30-microm deep subsurface cracking appeared in SSL, with 8 to 10 microm in AC, and 7 microm close to the margin of the wear facets in glazed EC after 5K cycles. The EC showed no cracks with increasing wear cycles. Seventy-microm deep subsurface cracks were detected in SSL and 45 microm in AC after 80K cycles. Statistically, there was significant difference among the three materials (p 0.05) in crack depth within the same ceramic material with different surface finishes. The ceramic materials with different microstructures showed different patterns of subsurface cracking.

Lower-Temperature Subsurface Layout and Ventilation Concepts

International Nuclear Information System (INIS)

Christine L. Linden; Edward G. Thomas

2001-01-01

This analysis combines work scope identified as subsurface facility (SSF) low temperature (LT) Facilities System and SSF LT Ventilation System in the Technical Work Plan for Subsurface Design Section FY 01 Work Activities (CRWMS M and O 2001b, pp. 6 and 7, and pp. 13 and 14). In accordance with this technical work plan (TWP), this analysis is performed using AP-3.10Q, Analyses and Models. It also incorporates the procedure AP-SI.1Q, Software Management. The purpose of this analysis is to develop an overall subsurface layout system and the overall ventilation system concepts that address a lower-temperature operating mode for the Monitored Geologic Repository (MGR). The objective of this analysis is to provide a technical design product that supports the lower-temperature operating mode concept for the revision of the system description documents and to provide a basis for the system description document design descriptions. The overall subsurface layout analysis develops and describes the overall subsurface layout, including performance confirmation facilities (also referred to as Test and Evaluation Facilities) for the Site Recommendation design. This analysis also incorporates current program directives for thermal management

Comparison of Mucosal, Subcutaneous and Intraperitoneal Routes of Rat Leptospira Infection

Science.gov (United States)

Zilber, Anne-Laure; Belli, Patrick; Grezel, Delphine; Artois, Marc; Kodjo, Angeli; Djelouadji, Zoheira

2016-01-01

Leptospirosis is a zoonosis found worldwide that is caused by a spirochete. The main reservoirs of Leptospira, which presents an asymptomatic infection, are wild rodents, including the brown rat (Rattus norvegicus). Experimental studies of the mechanisms of its renal colonization in rats have previously used an intraperitoneal inoculation route. However, knowledge of rat-rat transmission requires the use of a natural route of inoculation, such as a mucosal or subcutaneous route. We investigated for the first time the effects of subcutaneous and mucosal inoculation routes compared to the reference intraperitoneal route during Leptospira infection in adult rats. Infection characteristics were studied using Leptospira renal isolation, serology, and molecular and histological analyses. Leptospira infection was asymptomatic using each inoculation route, and caused similar antibody production regardless of renal colonization. The observed renal colonization rates were 8 out of 8 rats, 5 out of 8 rats and 1 out of 8 rats for the intraperitoneal, mucosal and subcutaneous inoculation routes, respectively. Thus, among the natural infection routes studied, mucosal inoculation was more efficient for renal colonization associated with urinary excretion than the subcutaneous route and induced a slower-progressing infection than the intraperitoneal route. These results can facilitate understanding of the infection modalities in rats, unlike the epidemiological studies conducted in wild rats. Future studies of other natural inoculation routes in rat models will increase our knowledge of rat-rat disease transmission and allow the investigation of infection kinetics. PMID:27031867

Potential for Nitrogen Fixation and Nitrification in the Granite-Hosted Subsurface at Henderson Mine, CO

Science.gov (United States)

Swanner, Elizabeth D.; Templeton, Alexis S.

2011-01-01

The existence of life in the deep terrestrial subsurface is established, yet few studies have investigated the origin of nitrogen that supports deep life. Previously, 16S rRNA gene surveys cataloged a diverse microbial community in subsurface fluids draining from boreholes 3000 feet deep at Henderson Mine, CO, USA (Sahl et al., 2008). The prior characterization of the fluid chemistry and microbial community forms the basis for the further investigation here of the source of NH4+. The reported fluid chemistry included N2, NH4+ (5–112 μM), NO2− (27–48 μM), and NO3− (17–72 μM). In this study, the correlation between low NH4+ concentrations in dominantly meteoric fluids and higher NH4+ in rock-reacted fluids is used to hypothesize that NH4+ is sourced from NH4+-bearing biotite. However, biotite samples from the host rocks and ore-body minerals were analyzed by Fourier transform infrared (FTIR) microscopy and none-contained NH4+. However, the nitrogenase-encoding gene nifH was successfully amplified from DNA of the fluid sample with high NH4+, suggesting that subsurface microbes have the capability to fix N2. If so, unregulated nitrogen fixation may account for the relatively high NH4+ concentrations in the fluids. Additionally, the amoA and nxrB genes for archaeal ammonium monooxygenase and nitrite oxidoreductase, respectively, were amplified from the high NH4+ fluid DNA, while bacterial amoA genes were not. Putative nitrifying organisms are closely related to ammonium-oxidizing Crenarchaeota and nitrite-oxidizing Nitrospira detected in other subsurface sites based upon 16S rRNA sequence analysis. Thermodynamic calculations underscore the importance of NH4+ as an energy source in a subsurface nitrification pathway. These results suggest that the subsurface microbial community at Henderson is adapted to the low nutrient and energy environment by their capability of fixing nitrogen, and that fixed nitrogen may support subsurface biomass via

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.

Biogenic Carbon on Mars: A Subsurface Chauvinistic Viewpoint

Science.gov (United States)

Onstott, T. C.; Lau, C. Y. M.; Magnabosco, C.; Harris, R.; Chen, Y.; Slater, G.; Sherwood Lollar, B.; Kieft, T. L.; van Heerden, E.; Borgonie, G.; Dong, H.

2015-12-01

A review of 150 publications on the subsurface microbiology of the continental subsurface provides ~1,400 measurements of cellular abundances down to 4,800 meter depth. These data suggest that the continental subsurface biomass is comprised of ~1016-17 grams of carbon, which is higher than the most recent estimates of ~1015 grams of carbon (1 Gt) for the marine deep biosphere. If life developed early in Martian history and Mars sustained an active hydrological cycle during its first 500 million years, then is it possible that Mars could have developed a subsurface biomass of comparable size to that of Earth? Such a biomass would comprise a much larger fraction of the total known Martian carbon budget than does the subsurface biomass on Earth. More importantly could a remnant of this subsurface biosphere survive to the present day? To determine how sustainable subsurface life could be in isolation from the surface we have been studying subsurface fracture fluids from the Precambrian Shields in South Africa and Canada. In these environments the energetically efficient and deeply rooted acetyl-CoA pathway for carbon fixation plays a central role for chemolithoautotrophic primary producers that form the base of the biomass pyramid. These primary producers appear to be sustained indefinitely by H2 generated through serpentinization and radiolytic reactions. Carbon isotope data suggest that in some subsurface locations a much larger population of secondary consumers are sustained by the primary production of biogenic CH4 from a much smaller population of methanogens. These inverted biomass and energy pyramids sustained by the cycling of CH4 could have been and could still be active on Mars. The C and H isotopic signatures of Martian CH4 remain key tools in identifying potential signatures of an extant Martian biosphere. Based upon our results to date cavity ring-down spectroscopic technologies provide an option for making these measurements on future rover missions.

Subsurface contaminants focus area

International Nuclear Information System (INIS)

1996-08-01

The US Department of Enregy (DOE) Subsurface Contaminants Focus Area is developing technologies to address environmental problems associated with hazardous and radioactive contaminants in soil and groundwater that exist throughout the DOE complex, including radionuclides, heavy metals; and dense non-aqueous phase liquids (DNAPLs). More than 5,700 known DOE groundwater plumes have contaminated over 600 billion gallons of water and 200 million cubic meters of soil. Migration of these plumes threatens local and regional water sources, and in some cases has already adversely impacted off-site rsources. In addition, the Subsurface Contaminants Focus Area is responsible for supplying technologies for the remediation of numerous landfills at DOE facilities. These landfills are estimated to contain over 3 million cubic meters of radioactive and hazardous buried Technology developed within this specialty area will provide efective methods to contain contaminant plumes and new or alternative technologies for development of in situ technologies to minimize waste disposal costs and potential worker exposure by treating plumes in place. While addressing contaminant plumes emanating from DOE landfills, the Subsurface Contaminants Focus Area is also working to develop new or alternative technologies for the in situ stabilization, and nonintrusive characterization of these disposal sites

Subsurface contaminants focus area

Energy Technology Data Exchange (ETDEWEB)

NONE

1996-08-01

The US Department of Enregy (DOE) Subsurface Contaminants Focus Area is developing technologies to address environmental problems associated with hazardous and radioactive contaminants in soil and groundwater that exist throughout the DOE complex, including radionuclides, heavy metals; and dense non-aqueous phase liquids (DNAPLs). More than 5,700 known DOE groundwater plumes have contaminated over 600 billion gallons of water and 200 million cubic meters of soil. Migration of these plumes threatens local and regional water sources, and in some cases has already adversely impacted off-site rsources. In addition, the Subsurface Contaminants Focus Area is responsible for supplying technologies for the remediation of numerous landfills at DOE facilities. These landfills are estimated to contain over 3 million cubic meters of radioactive and hazardous buried Technology developed within this specialty area will provide efective methods to contain contaminant plumes and new or alternative technologies for development of in situ technologies to minimize waste disposal costs and potential worker exposure by treating plumes in place. While addressing contaminant plumes emanating from DOE landfills, the Subsurface Contaminants Focus Area is also working to develop new or alternative technologies for the in situ stabilization, and nonintrusive characterization of these disposal sites.

Subsurface Controls on Stream Intermittency in a Semi-Arid Landscape

Science.gov (United States)

Dohman, J.; Godsey, S.; Thackray, G. D.; Hale, R. L.; Wright, K.; Martinez, D.

2017-12-01

Intermittent streams currently constitute 30% to greater than 50% of the global river network. In addition, the number of intermittent streams is expected to increase due to changes in land use and climate. These streams provide important ecosystem services, such as water for irrigation, increased biodiversity, and high rates of nutrient cycling. Many hydrological studies have focused on mapping current intermittent flow regimes or evaluating long-term flow records, but very few have investigated the underlying causes of stream intermittency. The disconnection and reconnection of surface flow reflects the capacity of the subsurface to accommodate flow, so characterizing subsurface flow is key to understanding stream drying. We assess how subsurface flow paths control local surface flows during low-flow periods, including intermittency. Water table dynamics were monitored in an intermittent reach of Gibson Jack Creek in southeastern Idaho. Four transects were delineated with a groundwater well located in the hillslope, riparian zone, and in the stream, for a total of 12 groundwater wells. The presence or absence of surface flow was determined by frequent visual observations as well as in situ loggers every 30m along the 200m study reach. The rate of surface water drying was measured in conjunction with temperature, precipitation, subsurface hydraulic conductivity, hillslope-riparian-stream connectivity and subsurface travel time. Initial results during an unusually wet year suggest different responses in reaches that were previously observed to occasionally cease flowing. Flows in the intermittent reaches had less coherent and lower amplitude diel variations during base flow periods than reaches that had never been observed to dry out. Our findings will help contribute to our understanding of mechanisms driving expansion and contraction cycles in intermittent streams, increase our ability to predict how land use and climate change will affect flow regimes, and

Subsurface Science Program Bibliography, 1985--1992

International Nuclear Information System (INIS)

1992-08-01

The Subsurface Science Program sponsors long-term basic research on (1) the fundamental physical, chemical, and biological mechanisms that control the reactivity, mobilization, stability, and transport of chemical mixtures in subsoils and ground water; (2) hydrogeology, including the hydraulic, microbiological, and geochemical properties of the vadose and saturated zones that control contaminant mobility and stability, including predictive modeling of coupled hydraulic-geochemical-microbial processes; and (3) the microbiology of deep sediments and ground water. TWs research, focused as it is on the natural subsurface environments that are most significantly affected by the more than 40 years of waste generation and disposal at DOE sites, is making important contributions to cleanup of DOE sites. Past DOE waste-disposal practices have resulted in subsurface contamination at DOE sites by unique combinations of radioactive materials and organic and inorganic chemicals (including heavy metals), which make site cleanup particularly difficult. The long- term (10- to 30-year) goal of the Subsurface Science Program is to provide a foundation of fundamental knowledge that can be used to reduce environmental risks and to provide a sound scientific basis for cost-effective cleanup strategies. The Subsurface Science Program is organized into nine interdisciplinary subprograms, or areas of basic research emphasis. The subprograms currently cover the areas of Co-Contaminant Chemistry, Colloids/Biocolloids, Multiphase Fluid Flow, Biodegradation/ Microbial Physiology, Deep Microbiology, Coupled Processes, Field-Scale (Natural Heterogeneity and Scale), and Environmental Science Research Center

Imaging subsurface damage of grinded fused silica optics by confocal fluorescence microscopy

International Nuclear Information System (INIS)

Neauport, J.; Cormont, P.; Destribats, J.; Legros, P.; Ambard, C.

2009-01-01

We report an experimental investigation of fluorescence confocal microscopy as a tool to measure subsurface damage on grinded fused silica optics. Confocal fluorescence microscopy was performed with an excitation at the wavelength of 405 nm on fixed abrasive diamond grinded fused silica samples. We detail the measured fluorescence spectrums and compare them to those of oil based coolants and grinding slurries. We evidence that oil based coolant used in diamond grinding induces a fluorescence that marks the subsurface damages and eases its observation. Such residual traces might also be involved in the laser damage process. (authors)

Quantifying induced effects of subsurface renewable energy storage

Science.gov (United States)

Bauer, Sebastian; Beyer, Christof; Pfeiffer, Tilmann; Boockmeyer, Anke; Popp, Steffi; Delfs, Jens-Olaf; Wang, Bo; Li, Dedong; Dethlefsen, Frank; Dahmke, Andreas

2015-04-01

New methods and technologies for energy storage are required for the transition to renewable energy sources. Subsurface energy storage systems such as salt caverns or porous formations offer the possibility of hosting large amounts of energy or substance. When employing these systems, an adequate system and process understanding is required in order to assess the feasibility of the individual storage option at the respective site and to predict the complex and interacting effects induced. This understanding is the basis for assessing the potential as well as the risks connected with a sustainable usage of these storage options, especially when considering possible mutual influences. For achieving this aim, in this work synthetic scenarios for the use of the geological underground as an energy storage system are developed and parameterized. The scenarios are designed to represent typical conditions in North Germany. The types of subsurface use investigated here include gas storage and heat storage in porous formations. The scenarios are numerically simulated and interpreted with regard to risk analysis and effect forecasting. For this, the numerical simulators Eclipse and OpenGeoSys are used. The latter is enhanced to include the required coupled hydraulic, thermal, geomechanical and geochemical processes. Using the simulated and interpreted scenarios, the induced effects are quantified individually and monitoring concepts for observing these effects are derived. This presentation will detail the general investigation concept used and analyze the parameter availability for this type of model applications. Then the process implementation and numerical methods required and applied for simulating the induced effects of subsurface storage are detailed and explained. Application examples show the developed methods and quantify induced effects and storage sizes for the typical settings parameterized. This work is part of the ANGUS+ project, funded by the German Ministry

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.

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.

Subsurface probing

International Nuclear Information System (INIS)

Lytle, R.J.

1978-01-01

Imaging techniques that can be used to translate seismic and electromagnetic wave signals into visual representation are briefly discussed. The application of these techniques is illustrated on the example of determining the subsurface structure of a proposed power plant. Imaging makes the wave signals intelligible to the non-geologists. R and D work needed in this area are tabulated

The thermal impact of subsurface building structures on urban groundwater resources - A paradigmatic example.

Science.gov (United States)

Epting, Jannis; Scheidler, Stefan; Affolter, Annette; Borer, Paul; Mueller, Matthias H; Egli, Lukas; García-Gil, Alejandro; Huggenberger, Peter

2017-10-15

Shallow subsurface thermal regimes in urban areas are increasingly impacted by anthropogenic activities, which include infrastructure development like underground traffic lines as well as industrial and residential subsurface buildings. In combination with the progressive use of shallow geothermal energy systems, this results in the so-called subsurface urban heat island effect. This article emphasizes the importance of considering the thermal impact of subsurface structures, which commonly is underestimated due to missing information and of reliable subsurface temperature data. Based on synthetic heat-transport models different settings of the urban environment were investigated, including: (1) hydraulic gradients and conductivities, which result in different groundwater flow velocities; (2) aquifer properties like groundwater thickness to aquitard and depth to water table; and (3) constructional features, such as building depths and thermal properties of building structures. Our results demonstrate that with rising groundwater flow velocities, the heat-load from building structures increase, whereas down-gradient groundwater temperatures decrease. Thermal impacts on subsurface resources therefore have to be related to the permeability of aquifers and hydraulic boundary conditions. In regard to the urban settings of Basel, Switzerland, flow velocities of around 1 md -1 delineate a marker where either down-gradient temperature deviations or heat-loads into the subsurface are more relevant. Furthermore, no direct thermal influence on groundwater resources should be expected for aquifers with groundwater thicknesses larger 10m and when the distance of the building structure to the groundwater table is higher than around 10m. We demonstrate that measuring temperature changes down-gradient of subsurface structures is insufficient overall to assess thermal impacts, particularly in urban areas. Moreover, in areas which are densely urbanized, and where groundwater flow

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.

The impact of occipital lobe cortical thickness on cognitive task performance: An investigation in Huntington's Disease.

Science.gov (United States)

Johnson, Eileanoir B; Rees, Elin M; Labuschagne, Izelle; Durr, Alexandra; Leavitt, Blair R; Roos, Raymund A C; Reilmann, Ralf; Johnson, Hans; Hobbs, Nicola Z; Langbehn, Douglas R; Stout, Julie C; Tabrizi, Sarah J; Scahill, Rachael I

2015-12-01

The occipital lobe is an important visual processing region of the brain. Following consistent findings of early neural changes in the occipital lobe in Huntington's Disease (HD), we examined cortical thickness across four occipital regions in premanifest (preHD) and early HD groups compared with controls. Associations between cortical thickness in gene positive individuals and performance on six cognitive tasks, each with a visual component, were examined. In addition, the association between cortical thickness in gene positive participants and one non-visual motor task was also examined for comparison. Cortical thickness was determined using FreeSurfer on T1-weighted 3T MR datasets from controls (N=97), preHD (N=109) and HD (N=69) from the TRACK-HD study. Regression models were fitted to assess between-group differences in cortical thickness, and relationships between performance on the cognitive tasks, the motor task and occipital thickness were examined in a subset of gene-positive participants (N=141). Thickness of the occipital cortex in preHD and early HD participants was reduced compared with controls. Regionally-specific associations between reduced cortical thickness and poorer performance were found for five of the six cognitive tasks, with the strongest associations in lateral occipital and lingual regions. No associations were found with the cuneus. The non-visual motor task was not associated with thickness of any region. The heterogeneous pattern of associations found in the present study suggests that occipital thickness negatively impacts cognition, but only in regions that are linked to relatively advanced visual processing (e.g., lateral occipital, lingual regions), rather than in basic visual processing regions such as the cuneus. Our results show, for the first time, the functional implications of occipital atrophy highlighted in recent studies in HD. Copyright © 2015 Elsevier Ltd. All rights reserved.

Test-Retest Reliability of Diffusion Tensor Imaging in Huntington's Disease.

Science.gov (United States)

Cole, James H; Farmer, Ruth E; Rees, Elin M; Johnson, Hans J; Frost, Chris; Scahill, Rachael I; Hobbs, Nicola Z

2014-03-21

Diffusion tensor imaging (DTI) has shown microstructural abnormalities in patients with Huntington's Disease (HD) and work is underway to characterise how these abnormalities change with disease progression. Using methods that will be applied in longitudinal research, we sought to establish the reliability of DTI in early HD patients and controls. Test-retest reliability, quantified using the intraclass correlation coefficient (ICC), was assessed using region-of-interest (ROI)-based white matter atlas and voxelwise approaches on repeat scan data from 22 participants (10 early HD, 12 controls). T1 data was used to generate further ROIs for analysis in a reduced sample of 18 participants. The results suggest that fractional anisotropy (FA) and other diffusivity metrics are generally highly reliable, with ICCs indicating considerably lower within-subject compared to between-subject variability in both HD patients and controls. Where ICC was low, particularly for the diffusivity measures in the caudate and putamen, this was partly influenced by outliers. The analysis suggests that the specific DTI methods used here are appropriate for cross-sectional research in HD, and give confidence that they can also be applied longitudinally, although this requires further investigation. An important caveat for DTI studies is that test-retest reliability may not be evenly distributed throughout the brain whereby highly anisotropic white matter regions tended to show lower relative within-subject variability than other white or grey matter regions.

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.

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.)

Subsurface transport program: Research summary

International Nuclear Information System (INIS)

1987-01-01

DOE's research program in subsurface transport is designed to provide a base of fundamental scientific information so that the geochemical, hydrological, and biological mechanisms that contribute to the transport and long term fate of energy related contaminants in subsurface ecosystems can be understood. Understanding the physical and chemical mechanisms that control the transport of single and co-contaminants is the underlying concern of the program. Particular attention is given to interdisciplinary research and to geosphere-biosphere interactions. The scientific results of the program will contribute to resolving Departmental questions related to the disposal of energy-producing and defense wastes. The background papers prepared in support of this document contain additional information on the relevance of the research in the long term to energy-producing technologies. Detailed scientific plans and other research documents are available for high priority research areas, for example, in subsurface transport of organic chemicals and mixtures and in the microbiology of deep aquifers. 5 figs., 1 tab

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.

State alternative route designations

International Nuclear Information System (INIS)

1989-07-01

Pursuant to the Hazardous Materials Transportation Act (HMTA), the Department of Transportation (DOT) has promulgated a comprehensive set of regulations regarding the highway transportation of high-level radioactive materials. These regulations, under HM-164 and HM-164A, establish interstate highways as the preferred routes for the transportation of radioactive materials within and through the states. The regulations also provide a methodology by which a state may select alternative routes. First,the state must establish a ''state routing agency,'' defined as an entity authorized to use the state legal process to impose routing requirements on carriers of radioactive material (49 CFR 171.8). Once identified, the state routing agency must select routes in accordance with Large Quantity Shipments of Radioactive Materials or an equivalent routing analysis. Adjoining states and localities should be consulted on the impact of proposed alternative routes as a prerequisite of final route selection. Lastly, the states must provide written notice of DOT of any alternative route designation before the routes are deemed effective

MSTS - Multiphase Subsurface Transport Simulator theory manual

International Nuclear Information System (INIS)

White, M.D.; Nichols, W.E.

1993-05-01

The US Department of Energy, through the Yucca Mountain Site Characterization Project Office, has designated the Yucca Mountain site in Nevada for detailed study as the candidate US geologic repository for spent nuclear fuel and high-level radioactive waste. Site characterization will determine the suitability of the Yucca Mountain site for the potential waste repository. If the site is determined suitable, subsequent studies and characterization will be conducted to obtain authorization from the Nuclear Regulatory Commission to construct the potential waste repository. A principal component of the characterization and licensing processes involves numerically predicting the thermal and hydrologic response of the subsurface environment of the Yucca Mountain site to the potential repository over a 10,000-year period. The thermal and hydrologic response of the subsurface environment to the repository is anticipated to include complex processes of countercurrent vapor and liquid migration, multiple-phase heat transfer, multiple-phase transport, and geochemical reactions. Numerical simulators based on mathematical descriptions of these subsurface phenomena are required to make numerical predictions of the thermal and hydrologic response of the Yucca Mountain subsurface environment The engineering simulator called the Multiphase Subsurface Transport Simulator (MSTS) was developed at the request of the Yucca Mountain Site Characterization Project Office to produce numerical predictions of subsurface flow and transport phenomena at the potential Yucca Mountain site. This document delineates the design architecture and describes the specific computational algorithms that compose MSTS. Details for using MSTS and sample problems are given in the open-quotes User's Guide and Referenceclose quotes companion document

Using Muons to Image the Subsurface.

Energy Technology Data Exchange (ETDEWEB)

Bonal, Nedra [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Cashion, Avery Ted [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Cieslewski, Grzegorz [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Dorsey, Daniel J. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Foris, Adam [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Miller, Timothy J. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Roberts, Barry L [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Su, Jiann-Cherng [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Dreesen, Wendi [NSTec, Livermore, CA (United States); Green, J. Andrew [NSTec, Livermore, CA (United States); Schwellenbach, David [NSTec, Livermore, CA (United States)

2016-11-01

Muons are subatomic particles that can penetrate the earth 's crust several kilometers and may be useful for subsurface characterization . The absorption rate of muons depends on the density of the materials through which they pass. Muons are more sensitive to density variation than other phenomena, including gravity, making them beneficial for subsurface investigation . Measurements of muon flux rate at differing directions provide density variations of the materials between the muon source (cosmic rays and neutrino interactions) and the detector, much like a CAT scan. Currently, muon tomography can resolve features to the sub-meter scale. This work consists of three parts to address the use of muons for subsurface characterization : 1) assess the use of muon scattering for estimating density differences of common rock types, 2 ) using muon flux to detect a void in rock, 3) measure muon direction by designing a new detector. Results from this project lay the groundwork for future directions in this field. Low-density objects can be detected by muons even when enclosed in high-density material like lead, and even small changes in density (e.g. changes due to fracturing of material) can be detected. Rock density has a linear relationship with muon scattering density per rock volume when this ratio is greater than 0.10 . Limitations on using muon scattering to assess density changes among common rock types have been identified. However, other analysis methods may show improved results for these relatively low density materials. Simulations show that muons can be used to image void space (e.g. tunnels) within rock but experimental results have been ambiguous. Improvements are suggested to improve imaging voids such as tunnels through rocks. Finally, a muon detector has been designed and tested to measure muon direction, which will improve signal-to-noise ratio and help address fundamental questions about the source of upgoing muons .

"To Control Tibet, First Pacify Kham": Trade Routes and "Official Routes" (Guandao in Easternmost Kham

Directory of Open Access Journals (Sweden)

Patrick Booz

2016-06-01

Full Text Available This article focuses on the trade routes in the western Sichuan borderlands that facilitated contact and trade between Chinese counties and Eastern Tibet. In particular, the article offers a description of “official routes” (guandao—which the Chinese emperor twice proclaimed to be the vital mode of access between China and Tibet—from Chengdu, Sichuan’s provincial capital, to Khampa areas, with Lhasa as the final destination. The exchange of goods in this region followed various routes during different periods. From the tenth to sixteenth centuries, transactions occurred primarily along the borders of Amdo (Tib. A mdo, Northeastern Tibet, but for political, economic, and practical reasons, such exchanges became more limited geographically and eventually focused along the Sichuan–Kham/Ngawa border. Many routes shifted to the towns of Kangding (Tib. Dartsedo and Songpan (Tib. Zungchu, the main sites of distribution, where rich opportunities for trade and a strictly limiting transport geography made them important entrepôts that evolved into centers of prosperity. The geographic range of this article reaches to these two towns and leaves the investigation of the routes that led to western centers such as Derge, Batang, Chamdo, and Jyekundo for future research.

High pressure-elevated temperature x-ray micro-computed tomography for subsurface applications.

Science.gov (United States)

Iglauer, Stefan; Lebedev, Maxim

2018-06-01

Physical, chemical and mechanical pore-scale (i.e. micrometer-scale) mechanisms in rock are of key importance in many, if not all, subsurface processes. These processes are highly relevant in various applications, e.g. hydrocarbon recovery, CO 2 geo-sequestration, geophysical exploration, water production, geothermal energy production, or the prediction of the location of valuable hydrothermal deposits. Typical examples are multi-phase flow (e.g. oil and water) displacements driven by buoyancy, viscous or capillary forces, mineral-fluid interactions (e.g. mineral dissolution and/or precipitation over geological times), geo-mechanical rock behaviour (e.g. rock compaction during diagenesis) or fines migration during water production, which can dramatically reduce reservoir permeability (and thus reservoir performance). All above examples are 3D processes, and 2D experiments (as traditionally done for micro-scale investigations) will thus only provide qualitative information; for instance the percolation threshold is much lower in 3D than in 2D. However, with the advent of x-ray micro-computed tomography (μCT) - which is now routinely used - this limitation has been overcome, and such pore-scale processes can be observed in 3D at micrometer-scale. A serious complication is, however, the fact that in the subsurface high pressures and elevated temperatures (HPET) prevail, due to the hydrostatic and geothermal gradients imposed upon it. Such HPET-reservoir conditions significantly change the above mentioned physical and chemical processes, e.g. gas density is much higher at high pressure, which strongly affects buoyancy and wettability and thus gas distributions in the subsurface; or chemical reactions are significantly accelerated at increased temperature, strongly affecting fluid-rock interactions and thus diagenesis and deposition of valuable minerals. It is thus necessary to apply HPET conditions to the aforementioned μCT experiments, to be able to mimic subsurface

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...

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...

A radiological and chemical investigation of the 7500 Area Contamination Site at Oak Ridge National Laboratory, Oak Ridge, Tennessee

International Nuclear Information System (INIS)

Williams, J.K.; Foley, R.D.; Tiner, P.F.; Hatmaker, T.L.; Uziel, M.S.; Swaja, R.E.

1993-05-01

A radiological and chemical investigation of the 7500 Area Contamination Site at Oak Ridge National Laboratory (ORNL) was conducted intermittently from February 1992 through May 1992. The investigation was performed by the Measurement Applications and Development Group of the Health and Safety Research Division of ORNL at the request of the US Department of Energy's Oak Ridge Operations Office and the ORNL Environmental Restoration Program. Results of this investigation indicate that the source of radioactive contamination at the point of the contamination incident is from one of the underground abandoned lines. The contamination in soil is likely the result of residual contamination from years of waste transport and maintenance operations (e.g., replacement of degraded joints, upgrading or replacement of entire pipelines, and associated landscaping activities). However, because (1) there is currently an active LLW line positioned in the same subsurface trench with the abandoned lines and (2) the physical condition of the abandoned lines may be brittle, this inquiry could not determine which abandoned line was responsible for the subsurface contamination. Soil sampling at the location of the contamination incident and along the pipeline route was performed in a manner so as not to damage the active LLW line and abandoned lines. Recommendations for corrective actions are included

Subsurface Fire Hazards Technical Report

International Nuclear Information System (INIS)

Logan, R.C.

1999-01-01

The results from this report are preliminary and cannot be used as input into documents supporting procurement, fabrication, or construction. This technical report identifies fire hazards and proposes their mitigation for the subsurface repository fire protection system. The proposed mitigation establishes the minimum level of fire protection to meet NRC regulations, DOE fire protection orders, that ensure fire containment, adequate life safety provisions, and minimize property loss. Equipment requiring automatic fire suppression systems is identified. The subsurface fire hazards that are identified can be adequately mitigated

Residues of endosulfan in surface and subsurface agricultural soil and its bioremediation.

Science.gov (United States)

Odukkathil, Greeshma; Vasudevan, Namasivayam

2016-01-01

The persistence of many hydrophobic pesticides has been reported by various workers in various soil environments and its bioremediation is a major concern due to less bioavailability. In the present study, the pesticide residues in the surface and subsurface soil in an area of intense agricultural activity in Pakkam Village of Thiruvallur District, Tamilnadu, India, and its bioremediation using a novel bacterial consortium was investigated. Surface (0-15 cm) and subsurface soils (15-30 cm and 30-40 cm) were sampled, and pesticides in different layers of the soil were analyzed. Alpha endosulfan and beta endosulfan concentrations ranged from 1.42 to 3.4 mg/g and 1.28-3.1 mg/g in the surface soil, 0.6-1.4 mg/g and 0.3-0.6 mg/g in the subsurface soil (15-30 cm), and 0.9-1.5 mg/g and 0.34-1.3 mg/g in the subsurface soil (30-40 cm) respectively. Residues of other persistent pesticides were also detected in minor concentrations. These soil layers were subjected to bioremediation using a novel bacterial consortium under a simulated soil profile condition in a soil reactor. The complete removal of alpha and beta endosulfan was observed over 25 days. Residues of endosulfate were also detected during bioremediation, which was subsequently degraded on the 30th day. This study revealed the existence of endosulfan in the surface and subsurface soils and also proved that the removal of such a ubiquitous pesticide in the surface and subsurface environment can be achieved in the field by bioaugumenting a biosurfactant-producing bacterial consortium that degrades pesticides. Copyright © 2015 Elsevier Ltd. All rights reserved.

Nested sampling algorithm for subsurface flow model selection, uncertainty quantification, and nonlinear calibration

KAUST Repository

Elsheikh, A. H.; Wheeler, M. F.; Hoteit, Ibrahim

2013-01-01

Calibration of subsurface flow models is an essential step for managing ground water aquifers, designing of contaminant remediation plans, and maximizing recovery from hydrocarbon reservoirs. We investigate an efficient sampling algorithm known

Subsurface barrier verification technologies, informal report

International Nuclear Information System (INIS)

Heiser, J.H.

1994-06-01

One of the more promising remediation options available to the DOE waste management community is subsurface barriers. Some of the uses of subsurface barriers include surrounding and/or containing buried waste, as secondary confinement of underground storage tanks, to direct or contain subsurface contaminant plumes and to restrict remediation methods, such as vacuum extraction, to a limited area. To be most effective the barriers should be continuous and depending on use, have few or no breaches. A breach may be formed through numerous pathways including: discontinuous grout application, from joints between panels and from cracking due to grout curing or wet-dry cycling. The ability to verify barrier integrity is valuable to the DOE, EPA, and commercial sector and will be required to gain full public acceptance of subsurface barriers as either primary or secondary confinement at waste sites. It is recognized that no suitable method exists for the verification of an emplaced barrier's integrity. The large size and deep placement of subsurface barriers makes detection of leaks challenging. This becomes magnified if the permissible leakage from the site is low. Detection of small cracks (fractions of an inch) at depths of 100 feet or more has not been possible using existing surface geophysical techniques. Compounding the problem of locating flaws in a barrier is the fact that no placement technology can guarantee the completeness or integrity of the emplaced barrier. This report summarizes several commonly used or promising technologies that have been or may be applied to in-situ barrier continuity verification

Feasibility of a subsurface storage

International Nuclear Information System (INIS)

1998-11-01

This report analyses the notion of subsurface storage under the scientifical, technical and legal aspects. This reflection belongs to the studies about long duration storage carried out in the framework of the axis 3 of the December 30, 1991 law. The report comprises 3 parts. The first part is a synthesis of the complete subsurface storage study: definitions, aim of the report, very long duration storage paradigm, description files of concepts, thematic synthesis (legal aspects, safety, monitoring, sites, seismicity, heat transfers, corrosion, concretes, R and works, handling, tailings and dismantlement, economy..), multi-criteria/multi-concept cross-analysis. The second part deals with the technical aspects of the subsurface storage: safety approach (long duration impact, radiation protection, mastery of effluents), monitoring strategy, macroscopic inventory of B-type waste packages, inventory of spent fuels, glasses, hulls and nozzles, geological contexts in the French territory (sites selection and characterization), on-site activities, hydrogeological and geochemical aspects, geo-technical works and infrastructures organization, subsurface seismic effects, cooling modes (ventilation, heat transfer with the geologic environment), heat transfer research programs (convection, poly-phase cooling in porous media), handling constraints, concretes (use, behaviour, durability), corrosion of metallic materials, technical-economical analysis, international context (experience feedback from Sweden (CLAB) and the USA (Yucca Mountain), other European and French facilities). The last part of the report is a graphical appendix with 3-D views and schemes of the different concepts. (J.S.)

Subsurface quality assurance practices

International Nuclear Information System (INIS)

1987-08-01

This report addresses only the concept of applying Nuclear Quality Assurance (NQA) practices to repository shaft and subsurface design and construction; how NQA will be applied; and the level of detail required in the documentation for construction of a shaft and subsurface repository in contrast to the level of detail required in the documentation for construction of a traditional mine. This study determined that NQA practices are viable, attainable, as well as required. The study identified the appropriate NQA criteria and the repository's major structures, systems, items, and activities to which the criteria are applicable. A QA plan, for design and construction, and a list of documentation, for construction, are presented. 7 refs., 1 fig., 18 tabs

Hurricane Evacuation Routes

Data.gov (United States)

Department of Homeland Security — Hurricane Evacuation Routes in the United States A hurricane evacuation route is a designated route used to direct traffic inland in case of a hurricane threat. This...

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.

The influence of advertisements on the conspicuity of routing information.

Science.gov (United States)

Boersema, T; Zwaga, H J

1985-12-01

An experiment is described in which the influence of advertisements on the conspicuity of routing information was investigated. Stimulus material consisted of colour slides of 12 railway station scenes. In two of these scenes, number and size of advertisements were systematically varied. Subjects were instructed to locate routing signs in the scenes. Performance on the location task was used as a measure of the routing sign conspicuity. The results show that inserting an advertisement lessens the conspicuity of the routing information. This effect becomes stronger if more or larger advertisements are added.

Drawing the subsurface : an integrative design approach

NARCIS (Netherlands)

Hooimeijer, F.L.; Lafleur, F.; Trinh, T.T.; Gogu, Constantin Radu; Campbell, Diarmad; de Beer, Johannes

2017-01-01

The sub-surface, with its man-made and natural components, plays an important, if not crucial, role in the urban climate and global energy transition. On the one hand, the sub-surface is associated with a variety of challenges such as subsidence, pollution, damage to infrastructure and shortages of

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

Influence of groundwater composition on subsurface iron and arsenic removal

KAUST Repository

Moed, David H.; Van Halem, Doris; Verberk, J. Q J C; Amy, Gary L.; Van Dijk, Johannis C.

2012-01-01

Subsurface arsenic and iron removal (SAR/SIR) is a novel technology to remove arsenic, iron and other groundwater components by using the subsoil. This research project investigated the influence of the groundwater composition on subsurface treatment. In anoxic sand column experiments, with synthetic groundwater and virgin sand, it was found that several dissolved substances in groundwater compete for adsorption sites with arsenic and iron. The presence of 0.01 mmol L -1phosphate, 0.2 mmol L -1 silicate, and 1 mmol L -1 nitrate greatly reduced the efficiency of SAR, illustrating the vulnerability of this technology in diverse geochemical settings. SIR was not as sensitive to other inorganic groundwater compounds, though iron retardation was limited by 1.2 mmol L -1 calcium and 0.06 mmol L -1 manganese. © IWA Publishing 2012.

Influence of groundwater composition on subsurface iron and arsenic removal

KAUST Repository

Moed, David H.

2012-06-01

Subsurface arsenic and iron removal (SAR/SIR) is a novel technology to remove arsenic, iron and other groundwater components by using the subsoil. This research project investigated the influence of the groundwater composition on subsurface treatment. In anoxic sand column experiments, with synthetic groundwater and virgin sand, it was found that several dissolved substances in groundwater compete for adsorption sites with arsenic and iron. The presence of 0.01 mmol L -1phosphate, 0.2 mmol L -1 silicate, and 1 mmol L -1 nitrate greatly reduced the efficiency of SAR, illustrating the vulnerability of this technology in diverse geochemical settings. SIR was not as sensitive to other inorganic groundwater compounds, though iron retardation was limited by 1.2 mmol L -1 calcium and 0.06 mmol L -1 manganese. © IWA Publishing 2012.

Improving the biodegradative capacity of subsurface bacteria

International Nuclear Information System (INIS)

Romine, M.F.; Brockman, F.J.

1993-04-01

The continual release of large volumes of synthetic materials into the environment by agricultural and industrial sources over the last few decades has resulted in pollution of the subsurface environment. Cleanup has been difficult because of the relative inaccessibility of the contaminants caused by their wide dispersal in the deep subsurface, often at low concentrations and in large volumes. As a possible solution for these problems, interest in the introduction of biodegradative bacteria for in situ remediation of these sites has increased greatly in recent years (Timmis et al. 1988). Selection of biodegradative microbes to apply in such cleanup is limited to those strains that can survive among the native bacterial and predator community members at the particular pH, temperature, and moisture status of the site (Alexander, 1984). The use of microorganisms isolated from subsurface environments would be advantageous because the organisms are already adapted to the subsurface conditions. The options are further narrowed to strains that are able to degrade the contaminant rapidly, even in the presence of highly recalcitrant anthropogenic waste mixtures, and in conditions that do not require addition of further toxic compounds for the expression of the biodegradative capacity (Sayler et al. 1990). These obstacles can be overcome by placing the genes of well-characterized biodegradative enzymes under the control of promoters that can be regulated by inexpensive and nontoxic external factors and then moving the new genetic constructs into diverse groups of subsurface microbes. ne objective of this research is to test this hypothesis by comparing expression of two different toluene biodegradative enzymatic pathways from two different regulatable promoters in a variety of subsurface isolates

Contaminant geochemistry. Interactions and transport in the subsurface environment

Energy Technology Data Exchange (ETDEWEB)

Berkowitz, Brian; Dror, Ishai; Yaron, Bruno [Weizmann Institute of Science, Rehovot (Israel). Dept. of Environmental Sciences and Energy Research

2008-07-01

This book combines earth science, subsurface hydrology and environmental geochemistry, providing a comprehensive background for specialists interested in the protection and sustainable management of the subsurface environment. The reader is introduced to the chemistry of contaminants, which usually disturb the natural equilibrium in the subsurface as a result of human activity. The major focus of the book is on contaminant reactions in soil solutions, groundwater and porous media solid phases, accounting for their persistence and transformation in the subsurface, as they are transported from the land surface into groundwater. Discussions on selected case studies are provided. (orig.)

Short-term memory, executive control, and children's route learning

OpenAIRE

Purser, H. R.; Farran, E. K.; Courbois, Y.; Lemahieu, A.; Mellier, D.; Sockeel, P.; Blades, M.

2012-01-01

The aim of this study was to investigate route-learning ability in 67 children aged 5 to 11years and to relate route-learning performance to the components of Baddeley's model of working memory. Children carried out tasks that included measures of verbal and visuospatial short-term memory and executive control and also measures of verbal and visuospatial long-term memory; the route-learning task was conducted using a maze in a virtual environment. In contrast to previous research, correlation...

Nanostructured palladium tailored via carbonyl chemical route towards oxygen reduction reaction

International Nuclear Information System (INIS)

Luo, Y.; Mora-Hernández, J.M.; Estudillo-Wong, L.A.; Arce-Estrada, E.M.; Alonso-Vante, N.

2015-01-01

Graphical Abstract: Mass-depending morphologies of nanostructured Palladium obtained via the carbonyl chemical route. Display Omitted -- Highlights: •Mass-depending morphology was observed in nanostructured palladium supported on carbon prepared by the carbonyl chemical route. •The Morphological effect of carbon supported Pd was investigated towards ORR. -- Abstract: Carbon supported palladium nanostructures were synthesized via the carbonyl chemical route. Compared with nanostructured platinum, prepared via carbonyl chemical route, Pd nanomaterials showed mass-loading morphology, whereas particle size and morphology of Pt nanostructures was constant. The oxygen reduction reaction (ORR) on nanostructured Pd, with different morphology in both acid and alkaline medium was investigated. A relationship, based on X-ray diffraction structural analysis pattern, transmission electron microscope, with the Pd morphological effect on ORR activity was identified

Influence of cutting parameters on the depth of subsurface deformed layer in nano-cutting process of single crystal copper.

Science.gov (United States)

Wang, Quanlong; Bai, Qingshun; Chen, Jiaxuan; Su, Hao; Wang, Zhiguo; Xie, Wenkun

2015-12-01

Large-scale molecular dynamics simulation is performed to study the nano-cutting process of single crystal copper realized by single-point diamond cutting tool in this paper. The centro-symmetry parameter is adopted to characterize the subsurface deformed layers and the distribution and evolution of the subsurface defect structures. Three-dimensional visualization and measurement technology are used to measure the depth of the subsurface deformed layers. The influence of cutting speed, cutting depth, cutting direction, and crystallographic orientation on the depth of subsurface deformed layers is systematically investigated. The results show that a lot of defect structures are formed in the subsurface of workpiece during nano-cutting process, for instance, stair-rod dislocations, stacking fault tetrahedron, atomic clusters, vacancy defects, point defects. In the process of nano-cutting, the depth of subsurface deformed layers increases with the cutting distance at the beginning, then decreases at stable cutting process, and basically remains unchanged when the cutting distance reaches up to 24 nm. The depth of subsurface deformed layers decreases with the increase in cutting speed between 50 and 300 m/s. The depth of subsurface deformed layer increases with cutting depth, proportionally, and basically remains unchanged when the cutting depth reaches over 6 nm.

Extracting subsurface fingerprints using optical coherence tomography

CSIR Research Space (South Africa)

Akhoury, SS

2015-02-01

Full Text Available Subsurface Fingerprints using Optical Coherence Tomography Sharat Saurabh Akhoury, Luke Nicholas Darlow Modelling and Digital Science, Council for Scientific and Industrial Research, Pretoria, South Africa Abstract Physiologists have found... approach to extract the subsurface fingerprint representation using a high-resolution imaging technology known as Optical Coherence Tomography (OCT). ...

Geophysical Investigation using Two Dimensional Electrical Resistivity Tomography method to delineate Subsurface Geological Structures at Dudhkoshi-II (230 MW) Hydroelectric Project, Solukhumbu District, Eastern Nepal

Science.gov (United States)

Ghimire, H.; Bhusal, U. C.; Khatiwada, B.; Pandey, D.

2017-12-01

Geophysical investigation using two dimensional electrical resistivity tomography (2D-ERT) method plays a significant role in determining the subsurface resistivity distribution by making measurement on the ground surface. This method was carried out at Dudhkoshi-II (230 MW) Hydroelectric Project, lies on Lesser Himalayan region of the Eastern Nepal to delineate the nature of the subsurface geology to assess its suitability for the construction of dam, desanding basin and powerhouse. The main objective of the proposed study consists of mapping vertical as well as horizontal variations of electrical resistivity to enable detection of the boundaries between unconsolidated materials and rocks of the different resistivity, possible geologic structures, such as possible presence of faults, fractures, and voids in intake and powerhouse area. For this purpose, the (WDJD-4 Multi-function Digital DC Resistivity/IP) equipment was used with Wenner array (60 electrodes). To fulfill these objectives of the study, the site area was mapped by Nine ERT profiles with different profile length and space between electrodes was 5 m. The depth of the investigation was 50 m. The acquired data were inverted to tomogram sections using tomographic inversion with RES2DINV commercial software. The Tomography sections show that the subsurface is classified into distinct geo-electric layers of dry unconsolidated overburden, saturated overburden, fractured rock and fresh bedrock of phyllites with quartzite and gneiss with different resistivity values. There were no voids and faults in the study area. Thickness of overburden at different region found to be different. Most of the survey area has bedrock of phyllites with quartzite; gneiss is also present in some location at intake area. Bedrock is found at the varies depth of 5-8 m at dam axis, 20-32 m at desanding basin and 3-10 m at powerhouse area. These results are confirmed and verified by using several boreholes data were drilled on the

Subsurface microbial communities and degradative capacities during trichloroethylene bioremediation

International Nuclear Information System (INIS)

Pfiffner, S.M.; Ringelberg, D.B.; Hedrick, D.B.; Phelps, T.J.; Palumbo, A.V.

1995-01-01

Subsurface amendments of air, methane, and nutrients were investigated for the in situ stimulation of trichloroethylene- degrading microorganisms at the US DOE Savannah River Integrated Demonstration. Amendments were injected into a lower horizontal well coupled with vacuum extraction from the vadose zone horizontal well. The amendments were sequenced to give increasingly more aggressive treatments. Microbial populations and degradative capacities were monitored in groundwaters samples bimonthly

Pseudo-Cycle-Based Multicast Routing in Wormhole-Routed Networks

Institute of Scientific and Technical Information of China (English)

SONG JianPing (宋建平); HOU ZiFeng (侯紫峰); XU Ming (许铭)

2003-01-01

This paper addresses the problem of fault-tolerant multicast routing in wormholerouted multicomputers. A new pseudo-cycle-based routing method is presented for constructing deadlock-free multicast routing algorithms. With at most two virtual channels this technique can be applied to any connected networks with arbitrary topologies. Simulation results show that this technique results in negligible performance degradation even in the presence of a large number of faulty nodes.

Routing in opportunistic networks

CERN Document Server

Dhurandher, Sanjay; Anpalagan, Alagan; Vasilakos, Athanasios

2013-01-01

This book provides a comprehensive guide to selected topics, both ongoing and emerging, in routing in OppNets. The book is edited by worldwide technical leaders, prolific researchers and outstanding academics, Dr. Isaac Woungang and co-editors, Dr. Sanjay Kumar Dhurandher, Prof. Alagan Anpalagan and Prof. Athanasios Vasilakos. Consisting of contributions from well known and high profile researchers and scientists in their respective specialties, the main topics that are covered in this book include mobility and routing, social-aware routing, context-based routing, energy-aware routing, incentive-aware routing, stochastic routing, modeling of intermittent connectivity, in both infrastructure and infrastructure-less OppNets. Key Features: Discusses existing and emerging techniques for routing in infrastructure and infrastructure-less OppNets. Provides a unified covering of otherwise disperse selected topics on routing in infrastructure and infrastructure-less OppNets.  Includes a set of PowerPoint slides and g...

3-D numerical investigation of subsurface flow in anisotropic porous media using multipoint flux approximation method

KAUST Repository

Negara, Ardiansyah

2013-01-01

Anisotropy of hydraulic properties of subsurface geologic formations is an essential feature that has been established as a consequence of the different geologic processes that they undergo during the longer geologic time scale. With respect to petroleum reservoirs, in many cases, anisotropy plays significant role in dictating the direction of flow that becomes no longer dependent only on the pressure gradient direction but also on the principal directions of anisotropy. Furthermore, in complex systems involving the flow of multiphase fluids in which the gravity and the capillarity play an important role, anisotropy can also have important influences. Therefore, there has been great deal of motivation to consider anisotropy when solving the governing conservation laws numerically. Unfortunately, the two-point flux approximation of finite difference approach is not capable of handling full tensor permeability fields. Lately, however, it has been possible to adapt the multipoint flux approximation that can handle anisotropy to the framework of finite difference schemes. In multipoint flux approximation method, the stencil of approximation is more involved, i.e., it requires the involvement of 9-point stencil for the 2-D model and 27-point stencil for the 3-D model. This is apparently challenging and cumbersome when making the global system of equations. In this work, we apply the equation-type approach, which is the experimenting pressure field approach that enables the solution of the global problem breaks into the solution of multitude of local problems that significantly reduce the complexity without affecting the accuracy of numerical solution. This approach also leads in reducing the computational cost during the simulation. We have applied this technique to a variety of anisotropy scenarios of 3-D subsurface flow problems and the numerical results demonstrate that the experimenting pressure field technique fits very well with the multipoint flux approximation

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.

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....

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

Defining the Post-Machined Sub-surface in Austenitic Stainless Steels

Science.gov (United States)

Srinivasan, N.; Sunil Kumar, B.; Kain, V.; Birbilis, N.; Joshi, S. S.; Sivaprasad, P. V.; Chai, G.; Durgaprasad, A.; Bhattacharya, S.; Samajdar, I.

2018-06-01

Austenitic stainless steels grades, with differences in chemistry, stacking fault energy, and thermal conductivity, were subjected to vertical milling. Anodic potentiodynamic polarization was able to differentiate (with machining speed/strain rate) between different post-machined sub-surfaces in SS 316L and Alloy A (a Cu containing austenitic stainless steel: Sanicroe 28™), but not in SS 304L. However, such differences (in the post-machined sub-surfaces) were revealed in surface roughness, sub-surface residual stresses and misorientations, and in the relative presence of sub-surface Cr2O3 films. It was shown, quantitatively, that higher machining speed reduced surface roughness and also reduced the effective depths of the affected sub-surface layers. A qualitative explanation on the sub-surface microstructural developments was provided based on the temperature-dependent thermal conductivity values. The results herein represent a mechanistic understanding to rationalize the corrosion performance of widely adopted engineering alloys.

Defining the Post-Machined Sub-surface in Austenitic Stainless Steels

Science.gov (United States)

Srinivasan, N.; Sunil Kumar, B.; Kain, V.; Birbilis, N.; Joshi, S. S.; Sivaprasad, P. V.; Chai, G.; Durgaprasad, A.; Bhattacharya, S.; Samajdar, I.

2018-04-01

Austenitic stainless steels grades, with differences in chemistry, stacking fault energy, and thermal conductivity, were subjected to vertical milling. Anodic potentiodynamic polarization was able to differentiate (with machining speed/strain rate) between different post-machined sub-surfaces in SS 316L and Alloy A (a Cu containing austenitic stainless steel: Sanicroe 28™), but not in SS 304L. However, such differences (in the post-machined sub-surfaces) were revealed in surface roughness, sub-surface residual stresses and misorientations, and in the relative presence of sub-surface Cr2O3 films. It was shown, quantitatively, that higher machining speed reduced surface roughness and also reduced the effective depths of the affected sub-surface layers. A qualitative explanation on the sub-surface microstructural developments was provided based on the temperature-dependent thermal conductivity values. The results herein represent a mechanistic understanding to rationalize the corrosion performance of widely adopted engineering alloys.

Coupled Monitoring and Inverse Modeling to Investigate Surface - Subsurface Hydrological and Thermal Dynamics in the Arctic Tundra

Science.gov (United States)

Tran, A. P.; Dafflon, B.; Hubbard, S. S.; Bisht, G.; Peterson, J.; Ulrich, C.; Romanovsky, V. E.; Kneafsey, T. J.; Wu, Y.

2015-12-01

Quantitative characterization of the soil surface-subsurface hydrological and thermal processes is essential as they are primary factors that control the biogeochemical processes, ecological landscapes and greenhouse gas fluxes. In the Artic region, the surface-subsurface hydrological and thermal regimes co-interact and are both largely influenced by soil texture and soil organic content. In this study, we present a coupled inversion scheme that jointly inverts hydrological, thermal and geophysical data to estimate the vertical profiles of clay, sand and organic contents. Within this inversion scheme, the Community Land Model (CLM4.5) serves as a forward model to simulate the land-surface energy balance and subsurface hydrological-thermal processes. Soil electrical conductivity (from electrical resistivity tomography), temperature and water content are linked together via petrophysical and geophysical models. Particularly, the inversion scheme accounts for the influences of the soil organic and mineral content on both of the hydrological-thermal dynamics and the petrophysical relationship. We applied the inversion scheme to the Next Generation Ecosystem Experiments (NGEE) intensive site in Barrow, AK, which is characterized by polygonal-shaped arctic tundra. The monitoring system autonomously provides a suite of above-ground measurements (e.g., precipitation, air temperature, wind speed, short-long wave radiation, canopy greenness and eddy covariance) as well as below-ground measurements (soil moisture, soil temperature, thaw layer thickness, snow thickness and soil electrical conductivity), which complement other periodic, manually collected measurements. The preliminary results indicate that the model can well reproduce the spatiotemporal dynamics of the soil temperature, and therefore, accurately predict the active layer thickness. The hydrological and thermal dynamics are closely linked to the polygon types and polygon features. The results also enable the

Brain energy metabolism and dopaminergic function in Huntington's disease measured in vivo using positron emission tomography

International Nuclear Information System (INIS)

Leenders, K.L.; Frackowiak, R.S.; Quinn, N.; Marsden, C.D.

1986-01-01

A 48-year-old man with typical Huntington's disease was investigated with computed tomography (CT) and positron emission tomography. Regional cerebral blood flow, oxygen extraction, oxygen and glucose utilization, L-Dopa uptake, and dopamine (D2) receptor binding were measured using several positron-labelled tracers. CT showed slight atrophy of the head of caudate but no cortical atrophy, although distinct frontal lobe dysfunction was present on psychometric testing. Oxygen and glucose metabolism and cerebral blood flow were decreased in the striata and to a lesser extent in frontal cortex. Cerebral blood flow was in the low normal range throughout the remainder of the brain. A normal metabolic ratio was found in all regions, since the changes in glucose utilization paralleled those in oxygen consumption. The capacity of the striatum to store dopamine as assessed by L-[ 18 F]-fluorodopa uptake was normal, but dopamine (D2) receptor binding was decreased when compared to normal subjects

Nitrate-nitrogen losses through subsurface drainage under various agricultural land covers.

Science.gov (United States)

Qi, Zhiming; Helmers, Matthew J; Christianson, Reid D; Pederson, Carl H

2011-01-01

Nitrate-nitrogen (NO₃-N) loading to surface water bodies from subsurface drainage is an environmental concern in the midwestern United States. The objective of this study was to investigate the effect of various land covers on NO₃-N loss through subsurface drainage. Land-cover treatments included (i) conventional corn ( L.) (C) and soybean [ (L.) Merr.] (S); (ii) winter rye ( L.) cover crop before corn (rC) and before soybean (rS); (iii) kura clover ( M. Bieb.) as a living mulch for corn (kC); and (iv) perennial forage of orchardgrass ( L.) mixed with clovers (PF). In spring, total N uptake by aboveground biomass of rye in rC, rye in rS, kura clover in kC, and grasses in PF were 14.2, 31.8, 87.0, and 46.3 kg N ha, respectively. Effect of land covers on subsurface drainage was not significant. The NO₃-N loss was significantly lower for kC and PF than C and S treatments (p rye cover crop did not reduce NO₃-N loss, but NO₃-N concentration was significantly reduced in rC during March to June and in rS during July to November (p rye cover crop on NO-N loss reduction needs further investigation under conditions of different N rates, wider weather patterns, and fall tillage. by the American Society of Agronomy, Crop Science Society of America, and Soil Science Society of America, Inc.

Association between mean and interannual equatorial Indian Ocean subsurface temperature bias in a coupled model

Science.gov (United States)

Srinivas, G.; Chowdary, Jasti S.; Gnanaseelan, C.; Prasad, K. V. S. R.; Karmakar, Ananya; Parekh, Anant

2018-03-01

In the present study the association between mean and interannual subsurface temperature bias over the equatorial Indian Ocean (EIO) is investigated during boreal summer (June through September; JJAS) in the National Centers for Environmental Prediction (NCEP) Climate Forecast System (CFSv2) hindcast. Anomalously high subsurface warm bias (greater than 3 °C) over the eastern EIO (EEIO) region is noted in CFSv2 during summer, which is higher compared to other parts of the tropical Indian Ocean. Prominent eastward current bias in the upper 100 m over the EIO region induced by anomalous westerly winds is primarily responsible for subsurface temperature bias. The eastward currents transport warm water to the EEIO and is pushed down to subsurface due to downwelling. Thus biases in both horizontal and vertical currents over the EIO region support subsurface warm bias. The evolution of systematic subsurface warm bias in the model shows strong interannual variability. These maximum subsurface warming episodes over the EEIO are mainly associated with La Niña like forcing. Strong convergence of low level winds over the EEIO and Maritime continent enhanced the westerly wind bias over the EIO during maximum warming years. This low level convergence of wind is induced by the bias in the gradient in the mean sea level pressure with positive bias over western EIO and negative bias over EEIO and parts of western Pacific. Consequently, changes in the atmospheric circulation associated with La Niña like conditions affected the ocean dynamics by modulating the current bias thereby enhancing the subsurface warm bias over the EEIO. It is identified that EEIO subsurface warming is stronger when La Niña co-occurred with negative Indian Ocean Dipole events as compared to La Niña only years in the model. Ocean general circulation model (OGCM) experiments forced with CFSv2 winds clearly support our hypothesis that ocean dynamics influenced by westerly winds bias is primarily

Location-Aided Reliable Routing for Opportunistic Networks

Institute of Scientific and Technical Information of China (English)

Yong Yang; Zhi Ren; Jibi Li

2012-01-01

In our study, we investigate the problem of location-based routing in opportunistic networks. Due to the uncertainty of end-to-end paths between sources and destinations, the classical location-based ad hoc routing protocols cannot adapt to the environment of opportunistic networks, which leads to the decrease of the delivery rate of data. To address it, we propose in this paper a novel location-aided routing protocol which is suitable for opportunistic networks. In our protocol, a node can send location information on demand by using prediction mechanism, and when sending a data packet, the node chooses multiple neighbors which are closer to the destination as next hops. If the candidate neighbors do not exist, the node carries the data till it meets a proper neighbor or the destination. Theoretical analysis and simulation results show that our proposed protocol outperforms DREAM (Distance Routing Effect Protocol Mobility) and one of its improved versions, BFDREAM (Boundary Forwarding DREAM), in terms of the delivery rate and overhead.

The influence of advertisements on the conspicuity of routing information

OpenAIRE

Boersema, T.; Zwaga, H.J.G.

1985-01-01

An experiment is described in which the influence of advertisements on the conspicuity of routing information was investigated. Stimulus material consisted of colour slides of 12 railway station scenes. In two of these scenes, number and size of advertisements were systematically varied. Subjects were instructed to locate routing signs in the scenes. Performance on the location task was used as a measure of the routing sign conspicuity. The results show that inserting an advertisement lessens...

Planning routes around the world: International evidence for southern route preferences

NARCIS (Netherlands)

Brunyé, Tad T.; Andonova, Elena; Meneghetti, Chiara; Noordzij, Matthijs Leendert; Pazzaglia, Francesca; Wienemann, Rasmus; Mahoney, Caroline R.; Taylor, Holly A.

2012-01-01

Three studies test whether the southern route preference, which describes the tendency for route planners to disproportionately select south- rather than north-going routes, can be attributed to regional elevation patterns; specifically, we ask whether this effect replicates in three topographically

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...

Regulatory issues and assumptions associated with polymers for subsurface barriers surrounding buried waste

International Nuclear Information System (INIS)

Heiser, J.; Siskind, B.

1993-01-01

One of the options for control of contaminant migration from buried waste sites is the construction of a subsurface barrier that consists of a wall of low permeability material. Subsurface barriers will improve remediation performance by removing pathways for contaminant transport due to groundwater movement, meteorological water infiltration, vapor- and gas-phase transport, transpiration, etc. Subsurface barriers may be used to open-quotes directclose quotes contaminant movement to collection sumps/lysimeters in cases of unexpected remediation failures or transport mechanisms, to contain leakage from underground storage tanks, and to restrict in-situ soil cleanup operation and chemicals. Brookhaven National Laboratory is currently investigating advanced polymer materials for subsurface barriers. This report addresses the regulatory aspects of using of non-traditional polymer materials as well as soil-bentonite or cement-bentonite mixtures for such barriers. The regulatory issues fall into two categories. The first category consists of issues associated with the acceptability of subsurface barriers to the Environmental Protection Agency (EPA) as a method for achieving waste site performance improvement. The second category encompasses those regulatory issues concerning health, safety and the environment which must be addressed regarding barrier installation and performance, especially if non-traditional materials are to be used. Since many of EPA's concerns regarding subsurface barriers focus on the chemicals used during installation of these barriers the authors discuss the results of a search of the Federal Register and the Code of Federal Regulations for references in Titles 29 and 40 pertaining to key chemicals likely to be utilized in installing non-traditional barrier materials. The use of polymeric materials in the construction industry has been accomplished with full compliance with the applicable health, safety, and environmental regulations

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.

Homogeneous Reactor Experiment (HRE) Pond cryogenic barrier technology demonstration: Pre-barrier subsurface hydrology and contaminant transport investigation

International Nuclear Information System (INIS)

Moline, G.R.

1998-03-01

The Homogeneous Reactor Experiment (HRE) Pond is the site of a former impoundment for radioactive wastes that has since been drained, filled with soil, and covered with an asphalt cap. The site is bordered to the east and south by a tributary that empties into Melton Branch Creek and that contains significant concentrations of radioactive contaminants, primarily 90 Sr. Because of the proximity of the tributary to the HRE disposal site and the probable flow of groundwater from the site to the tributary, it is hypothesized that the HRE Pond is a source of contamination to he creek. As a means for temporary containment of contaminants within the impoundment, a cryogenic barrier technology demonstration was initiated in FY96 with a background hydrologic investigation that continued through FY97. Cryogenic equipment installation was completed in FY97, and freezing was initiated in September of 1997. This report documents the results of a hydrologic and geologic investigation of the HRE Pond/cryogenic barrier site. The purpose of this investigation is to evaluate the hydrologic conditions within and around the impoundment in order to meet the following objectives: (1) to provide a pre-barrier subsurface hydrologic baseline for post-barrier performance assessment; (2) to confirm that the impoundment is hydraulically connected to the surrounding sediments; and (3) to determine the likely contaminant exit pathways from the impoundment. The methods of investigation included water level and temperature monitoring in a network of wells and standpipes in and surrounding the impoundment, a helium tracer test conducted under ambient flow conditions, and geologic logging during the drilling of boreholes for installation of cryogenic probes and temperature monitoring wells

Contaminated environments in the subsurface and bioremediation: organic contaminants

OpenAIRE

Holliger, Christof; Gaspard, Sarra; Glod, Guy; Heijman, Cornelis; Schumacher, Wolfram; Schwarzenbach, René P.; Vazquez, Francisco

2017-01-01

Due to leakages, spills, improper disposal and accidents during transport, organic compounds have become subsurface contaminants that threaten important drinking water resources. One strategy to remediate such polluted subsurface environments is to make use of the degradative capacity of bacteria. It is often sufficient to supply the subsurface with nutrients such as nitrogen and phosphorus, and aerobic treatments are still dominating. However, anaerobic processes have advantages such as low ...

Sight Word Reading in Prereaders: Use of Logographic vs. Alphabetic Access Routes.

Science.gov (United States)

Scott, Judith Anne; Ehri, Linnea C.

1990-01-01

Investigates whether prereaders who knew all their letters are better at forming logographic access routes than letter-sound access routes into memory from words read by sight. Concludes that prereaders become capable of forming letter-sound access routes when they learn letters well enough to take advantage of the phonetic cues the letters…

Integrated Surface/subsurface flow modeling in PFLOTRAN

Energy Technology Data Exchange (ETDEWEB)

Painter, Scott L [Oak Ridge National Lab. (ORNL), Oak Ridge, TN (United States)

2016-10-01

Understanding soil water, groundwater, and shallow surface water dynamics as an integrated hydrological system is critical for understanding the Earth’s critical zone, the thin outer layer at our planet’s surface where vegetation, soil, rock, and gases interact to regulate the environment. Computational tools that take this view of soil moisture and shallow surface flows as a single integrated system are typically referred to as integrated surface/subsurface hydrology models. We extend the open-source, highly parallel, subsurface flow and reactive transport simulator PFLOTRAN to accommodate surface flows. In contrast to most previous implementations, we do not represent a distinct surface system. Instead, the vertical gradient in hydraulic head at the land surface is neglected, which allows the surface flow system to be eliminated and incorporated directly into the subsurface system. This tight coupling approach leads to a robust capability and also greatly simplifies implementation in existing subsurface simulators such as PFLOTRAN. Successful comparisons to independent numerical solutions build confidence in the approximation and implementation. Example simulations of the Walker Branch and East Fork Poplar Creek watersheds near Oak Ridge, Tennessee demonstrate the robustness of the approach in geometrically complex applications. The lack of a robust integrated surface/subsurface hydrology capability had been a barrier to PFLOTRAN’s use in critical zone studies. This work addresses that capability gap, thus enabling PFLOTRAN as a community platform for building integrated models of the critical zone.

Clinical utility of FDG-PET in amyotrophic lateral sclerosis and Huntington's disease.

Science.gov (United States)

Agosta, Federica; Altomare, Daniele; Festari, Cristina; Orini, Stefania; Gandolfo, Federica; Boccardi, Marina; Arbizu, Javier; Bouwman, Femke; Drzezga, Alexander; Nestor, Peter; Nobili, Flavio; Walker, Zuzana; Pagani, Marco

2018-05-01

To evaluate the incremental value of FDG-PET over clinical tests in: (i) diagnosis of amyotrophic lateral sclerosis (ALS); (ii) picking early signs of neurodegeneration in patients with a genetic risk of Huntington's disease (HD); and detecting metabolic changes related to cognitive impairment in (iii) ALS and (iv) HD patients. Four comprehensive literature searches were conducted using the PICO model to extract evidence from relevant studies. An expert panel then voted using the Delphi method on these four diagnostic scenarios. The availability of evidence was good for FDG-PET utility to support the diagnosis of ALS, poor for identifying presymptomatic subjects carrying HD mutation who will convert to HD, and lacking for identifying cognitive-related metabolic changes in both ALS and HD. After the Delphi consensual procedure, the panel did not support the clinical use of FDG-PET for any of the four scenarios. Relative to other neurodegenerative diseases, the clinical use of FDG-PET in ALS and HD is still in its infancy. Once validated by disease-control studies, FDG-PET might represent a potentially useful biomarker for ALS diagnosis. FDG-PET is presently not justified as a routine investigation to predict conversion to HD, nor to detect evidence of brain dysfunction justifying cognitive decline in ALS and HD.

Co-segregation of Huntington disease and hereditary spastic paraplegia in 4 generations.

Science.gov (United States)

Panas, Marios; Karadima, Georgia; Kalfakis, Nikolaos; Vassilopoulos, Dimitris

2011-07-01

Huntington disease (HD) is an autosomal dominant neurodegenerative disease characterized by choreic hyperkinesias, cognitive decline, and psychiatric manifestations, caused by an increased number of CAG repeats in the IT15 gene on chromosome 4p16.3. Silver syndrome is a rare autosomal dominant form of complicated hereditary spastic paraplegia, characterized by lower limb spasticity in addition to amyotrophy of the small muscles of the hands. In addition to the previously identified locus SPG17 on chromosome 11q12-q14, a new locus (SPG38) on chromosome 4p16-p15 has been recently identified, a region that includes the HD gene. We present a Greek family with 5 members diagnosed with HD in 4 generations. All affected members also presented with clinical features of Silver syndrome showing severe spastic paraplegia and prominent atrophy of all small hand muscles bilaterally. None of the other family members showed features of either HD or spastic paraplegia. The reported coexistence of Silver syndrome with HD in 4 generations is not fortuitous, suggesting that these 2 distinct genetic disorders are in linkage disequilibrium. Although rare, it is reasonable to expect additional similar cases. Clinical neurologists should perhaps investigate this possibility in cases combining features of HD and involvement of the upper and lower motor neurons.

Electrode Cultivation and Interfacial Electron Transport in Subsurface Microorganisms

Science.gov (United States)

Karbelkar, A. A.; Jangir, Y.; Reese, B. K.; Wanger, G.; Anderson, C.; El-Naggar, M.; Amend, J.

2016-12-01

Continental subsurface environments can present significant energetic challenges to the resident microorganisms. While these environments are geologically diverse, potentially allowing energy harvesting by microorganisms that catalyze redox reactions, many of the abundant electron donors and acceptors are insoluble and therefore not directly bioavailable. Microbes can use extracellular electron transfer (EET) as a metabolic strategy to interact with redox active surfaces. This process can be mimicked on electrode surfaces and hence can lead to enrichment and quantification of subsurface microorganisms A primary bioelectrochemical enrichment with different oxidizing and reducing potentials set up in a single bioreactor was applied in situ to subsurface microorganisms residing in iron oxide rich deposits in the Sanford Underground Research Facility. Secondary enrichment revealed a plethora of classified and unclassified subsurface microbiota on both oxidizing and reducing potentials. From this enrichment, we have isolated a Gram-positive Bacillus along with Gram-negative Cupriavidus and Anaerospora strains (as electrode reducers) and Comamonas (as an electrode oxidizer). The Bacillus and Comamonas isolates were subjected to a detailed electrochemical characterization in half-reactors at anodic and cathodic potentials, respectively. An increase in cathodic current upon inoculation and cyclic voltammetry measurements confirm the hypothesis that Comamonas is capable of electron uptake from electrodes. In addition, measurements of Bacillus on anodes hint towards novel mechanisms that allow EET from Gram-positive bacteria. This study suggests that electrochemical approaches are well positioned to dissect such extracellular interactions that may be prevalent in the subsurface, while using physical electrodes to emulate the microhabitats, redox and geochemical gradients, and the spatially dependent interspecies interactions encountered in the subsurface. Electrochemical

Striatal pre-enkephalin overexpression improves Huntington's disease symptoms in the R6/2 mouse model of Huntington's disease.

Directory of Open Access Journals (Sweden)

Stéphanie Bissonnette

Full Text Available The reduction of pre-enkephalin (pENK mRNA expression might be an early sign of striatal neuronal dysfunction in Huntington's disease (HD, due to mutated huntingtin protein. Indeed, striatopallidal (pENK-containing neurodegeneration occurs at earlier stage of the disease, compare to the loss of striatonigral neurons. However, no data are available about the functional role of striatal pENK in HD. According to the neuroprotective properties of opioids that have been recognized recently, the objective of this study was to investigate whether striatal overexpression of pENK at early stage of HD can improve motor dysfunction, and/or reduce striatal neuronal loss in the R6/2 transgenic mouse model of HD. To achieve this goal recombinant adeno-associated-virus (rAAV2-containing green fluorescence protein (GFP-pENK was injected bilaterally in the striatum of R6/2 mice at 5 weeks old to overexpress opioid peptide pENK. Striatal injection of rAAV2-GFP was used as a control. Different behavioral tests were carried out before and/or after striatal injections of rAAV2. The animals were euthanized at 10 weeks old. Our results demonstrate that striatal overexpression of pENK had beneficial effects on behavioral symptoms of HD in R6/2 by: delaying the onset of decline in muscular force; reduction of clasping; improvement of fast motor activity, short-term memory and recognition; as well as normalization of anxiety-like behavior. The improvement of behavioral dysfunction in R6/2 mice having received rAAV2-GFP-pENK associated with upregulation of striatal pENK mRNA; the increased level of enkephalin peptide in the striatum, globus pallidus and substantia nigra; as well as the slight increase in the number of striatal neurons compared with other groups of R6/2. Accordingly, we suggest that at early stage of HD upregulation of striatal enkephalin might play a key role at attenuating illness symptoms.

Mitochondrial DNA levels in Huntington disease leukocytes and dermal fibroblasts.

Science.gov (United States)

Jędrak, Paulina; Krygier, Magdalena; Tońska, Katarzyna; Drozd, Małgorzata; Kaliszewska, Magdalena; Bartnik, Ewa; Sołtan, Witold; Sitek, Emilia J; Stanisławska-Sachadyn, Anna; Limon, Janusz; Sławek, Jarosław; Węgrzyn, Grzegorz; Barańska, Sylwia

2017-08-01

Huntington disease (HD) is an inherited neurodegenerative disorder caused by mutations in the huntingtin gene. Involvement of mitochondrial dysfunctions in, and especially influence of the level of mitochondrial DNA (mtDNA) on, development of this disease is unclear. Here, samples of blood from 84 HD patients and 79 controls, and dermal fibroblasts from 10 HD patients and 9 controls were analysed for mtDNA levels. Although the type of mitochondrial haplogroup had no influence on the mtDNA level, and there was no correlation between mtDNA level in leukocytes in HD patients and various parameters of HD severity, some considerable differences between HD patients and controls were identified. The average mtDNA/nDNA relative copy number was significantly higher in leukocytes, but lower in fibroblasts, of symptomatic HD patients relative to the control group. Moreover, HD women displayed higher mtDNA levels in leukocytes than HD men. Because this is the largest population analysed to date, these results might contribute to explanation of discrepancies between previously published studies concerning levels of mtDNA in cells of HD patients. We suggest that the size of the investigated population and type of cells from which DNA is isolated could significantly affect results of mtDNA copy number estimation in HD. Hence, these parameters should be taken into consideration in studies on mtDNA in HD, and perhaps also in other diseases where mitochondrial dysfunction occurs.

Pitfalls in the detection of cholesterol in Huntington's disease models.

Science.gov (United States)

Marullo, Manuela; Valenza, Marta; Leoni, Valerio; Caccia, Claudio; Scarlatti, Chiara; De Mario, Agnese; Zuccato, Chiara; Di Donato, Stefano; Carafoli, Ernesto; Cattaneo, Elena

2012-10-11

Background Abnormalities in brain cholesterol homeostasis have been reported in Huntington's disease (HD), an adult-onset neurodegenerative disorder caused by an expansion in the number of CAG repeats in the huntingtin (HTT) gene. However, the results have been contradictory with respect to whether cholesterol levels increase or decrease in HD models. Biochemical and mass spectrometry methods show reduced levels of cholesterol precursors and cholesterol in HD cells and in the brains of several HD animal models. Abnormal brain cholesterol homeostasis was also inferred from studies in HD patients. In contrast, colorimetric and enzymatic methods indicate cholesterol accumulation in HD cells and tissues. Here we used several methods to investigate cholesterol levels in cultured cells in the presence or absence of mutant HTT protein. Results Colorimetric and enzymatic methods with low sensitivity gave variable results, whereas results from a sensitive analytical method, gas chromatography-mass spectrometry, were more reliable. Sample preparation, high cell density and cell clonality also influenced the detection of intracellular cholesterol. Conclusions Detection of cholesterol in HD samples by colorimetric and enzymatic assays should be supplemented by detection using more sensitive analytical methods. Care must be taken to prepare the sample appropriately. By evaluating lathosterol levels using isotopic dilution mass spectrometry, we confirmed reduced cholesterol biosynthesis in knock-in cells expressing the polyQ mutation in a constitutive or inducible manner. *Correspondence should be addressed to Elena Cattaneo: elena.cattaneo@unimi.it.

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.

Contents and composition of organic matter in subsurface soils affected by land use and soil mineralogy

Science.gov (United States)

Ellerbrock, Ruth H.; Kaiser, Michael

2010-05-01

Land use and mineralogy affect the ability of surface as well as subsurface soils to sequester organic carbon and their contribution to mitigate the greenhouse effect. This study aimed to investigate the long-term impact of land use (i.e., arable and forest) and soil mineralogy on contents and composition of soil organic matter (SOM) from subsurface soils. Seven soils different in mineralogy (Albic and Haplic Luvisol, Colluvic and Haplic Regosol, Haplic and Vertic Cambisol, Haplic Stagnosol) were selected within Germany. Soil samples were taken from forest and adjacent arable sites. First, particulate and water soluble organic matter were separated from the subsurface soil samples. From the remaining solid residues the OM(PY) fractions were separated, analyzed for its OC content (OCPY) and characterized by FTIR spectroscopy. For the arable subsurface soils multiple regression analyses indicate significant positive relationships between the soil organic carbon contents and the contents of i) exchangeable Ca and oxalate soluble Fe, and Alox contents. Further for the neutral arable subsurface soils the contents OCPY weighted by its C=O contents were found to be related to the contents of Ca indicating interactions between OM(PY) and Ca cations. For the forest subsurface soils (pH <5) the OCPY contents were positively related with the contents of Na-pyrophosphate soluble Fe and Al. For the acidic forest subsurface soils such findings indicate interactions between OM(PY) and Fe3+ and Al3+ cations. The effects of land use and soil mineralogy on contents and composition of SOM and OM(PY) will be discussed.

A lower bound for the node, edge, and arc routing problem

DEFF Research Database (Denmark)

Bach, Lukas; Hasle, Geir; Wøhlk, Sanne

2013-01-01

The Node, Edge, and Arc Routing Problem (NEARP) was defined by Prins and Bouchenoua in 2004, although similar problems have been studied before. This problem, also called the Mixed Capacitated General Routing Problem (MCGRP), generalizes the classical Capacitated Vehicle Routing Problem (CVRP......), the Capacitated Arc Routing Problem (CARP), and the General Routing Problem. It captures important aspects of real-life routing problems that were not adequately modeled in previous Vehicle Routing Problem (VRP) variants. The authors also proposed a memetic algorithm procedure and defined a set of test instances...... called the CBMix benchmark. The NEARP definition and investigation contribute to the development of rich VRPs. In this paper we present the first lower bound procedure for the NEARP. It is a further development of lower bounds for the CARP. We also define two novel sets of test instances to complement...

Subsurface intakes for seawater reverse osmosis facilities: Capacity limitation, water quality improvement, and economics

KAUST Repository

Missimer, Thomas M.

2013-08-01

The use of subsurface intake systems for seawater reverse osmosis (SWRO) desalination plants significantly improves raw water quality, reduces chemical usage and environmental impacts, decreases the carbon footprint, and reduces cost of treated water to consumers. These intakes include wells (vertical, angle, and radial type) and galleries, which can be located either on the beach or in the seabed. Subsurface intakes act both as intakes and as part of the pretreatment system by providing filtration and active biological treatment of the raw seawater. Recent investigations of the improvement in water quality made by subsurface intakes show lowering of the silt density index by 75 to 90%, removal of nearly all algae, removal of over 90% of bacteria, reduction in the concentrations of TOC and DOC, and virtual elimination of biopolymers and polysaccharides that cause organic biofouling of membranes. Economic analyses show that overall SWRO operating costs can be reduced by 5 to 30% by using subsurface intake systems. Although capital costs can be slightly to significantly higher compared to open-ocean intake system costs, a preliminary life-cycle cost analysis shows significant cost saving over operating periods of 10 to 30. years. © 2013 Elsevier B.V.

30 CFR 250.119 - Will MMS approve subsurface gas storage?

Science.gov (United States)

2010-07-01

... 30 Mineral Resources 2 2010-07-01 2010-07-01 false Will MMS approve subsurface gas storage? 250....119 Will MMS approve subsurface gas storage? The Regional Supervisor may authorize subsurface storage of gas on the OCS, on and off-lease, for later commercial benefit. To receive MMS approval you must...

Altered hypothalamic protein expression in a rat model of Huntington's disease.

Directory of Open Access Journals (Sweden)

Wei-na Cong

Full Text Available Huntington's disease (HD is a neurodegenerative disorder, which is characterized by progressive motor impairment and cognitive alterations. Changes in energy metabolism, neuroendocrine function, body weight, euglycemia, appetite function, and circadian rhythm can also occur. It is likely that the locus of these alterations is the hypothalamus. We used the HD transgenic (tg rat model bearing 51 CAG repeats, which exhibits similar HD symptomology as HD patients to investigate hypothalamic function. We conducted detailed hypothalamic proteome analyses and also measured circulating levels of various metabolic hormones and lipids in pre-symptomatic and symptomatic animals. Our results demonstrate that there are significant alterations in HD rat hypothalamic protein expression such as glial fibrillary acidic protein (GFAP, heat shock protein-70, the oxidative damage protein glutathione peroxidase (Gpx4, glycogen synthase1 (Gys1 and the lipid synthesis enzyme acylglycerol-3-phosphate O-acyltransferase 1 (Agpat1. In addition, there are significant alterations in various circulating metabolic hormones and lipids in pre-symptomatic animals including, insulin, leptin, triglycerides and HDL, before any motor or cognitive alterations are apparent. These early metabolic and lipid alterations are likely prodromal signs of hypothalamic dysfunction. Gaining a greater understanding of the hypothalamic and metabolic alterations that occur in HD, could lead to the development of novel therapeutics for early interventional treatment of HD.

Short-Term Memory, Executive Control, and Children's Route Learning

Science.gov (United States)

Purser, Harry R. M.; Farran, Emily K.; Courbois, Yannick; Lemahieu, Axelle; Mellier, Daniel; Sockeel, Pascal; Blades, Mark

2012-01-01

The aim of this study was to investigate route-learning ability in 67 children aged 5 to 11 years and to relate route-learning performance to the components of Baddeley's model of working memory. Children carried out tasks that included measures of verbal and visuospatial short-term memory and executive control and also measures of verbal and…

Transformation of zinc-concentrate in surface and subsurface environments: Implications for assessing zinc mobility/toxicity and choosing an optimal remediation strategy.

Science.gov (United States)

Kwon, Man Jae; Boyanov, Maxim I; Yang, Jung-Seok; Lee, Seunghak; Hwang, Yun Ho; Lee, Ju Yeon; Mishra, Bhoopesh; Kemner, Kenneth M

2017-07-01

Zinc contamination in near- and sub-surface environments is a serious threat to many ecosystems and to public health. Sufficient understanding of Zn speciation and transport mechanisms is therefore critical to evaluating its risk to the environment and to developing remediation strategies. The geochemical and mineralogical characteristics of contaminated soils in the vicinity of a Zn ore transportation route were thoroughly investigated using a variety of analytical techniques (sequential extraction, XRF, XRD, SEM, and XAFS). Imported Zn-concentrate (ZnS) was deposited in a receiving facility and dispersed over time to the surrounding roadside areas and rice-paddy soils. Subsequent physical and chemical weathering resulted in dispersal into the subsurface. The species identified in the contaminated areas included Zn-sulfide, Zn-carbonate, other O-coordinated Zn-minerals, and Zn species bound to Fe/Mn oxides or clays, as confirmed by XAFS spectroscopy and sequential extraction. The observed transformation from S-coordinated Zn to O-coordinated Zn associated with minerals suggests that this contaminant can change into more soluble and labile forms as a result of weathering. For the purpose of developing a soil washing remediation process, the contaminated samples were extracted with dilute acids. The extraction efficiency increased with the increase of O-coordinated Zn relative to S-coordinated Zn in the sediment. This study demonstrates that improved understanding of Zn speciation in contaminated soils is essential for well-informed decision making regarding metal mobility and toxicity, as well as for choosing an appropriate remediation strategy using soil washing. Copyright © 2017 Elsevier Ltd. All rights reserved.

Routing strategy in a distribution network when the driver learning effect is considered

DEFF Research Database (Denmark)

Battini, Daria; Faccio, Maurizio; Persona, Alessandro

2015-01-01

This paper faces one critical short term decision: the construction of routes for daily goods' deliveries in a distribution network. It investigates the possibility of applying a fixed routing strategy instead of a daily routing optimisation strategy, analysing the benefits derived from the drive...

Method of imaging the electrical conductivity distribution of a subsurface

Science.gov (United States)

Johnson, Timothy C.

2017-09-26

A method of imaging electrical conductivity distribution of a subsurface containing metallic structures with known locations and dimensions is disclosed. Current is injected into the subsurface to measure electrical potentials using multiple sets of electrodes, thus generating electrical resistivity tomography measurements. A numeric code is applied to simulate the measured potentials in the presence of the metallic structures. An inversion code is applied that utilizes the electrical resistivity tomography measurements and the simulated measured potentials to image the subsurface electrical conductivity distribution and remove effects of the subsurface metallic structures with known locations and dimensions.

Enhancement of the complete autotrophic nitrogen removal over nitrite process in a modified single-stage subsurface vertical flow constructed wetland: Effect of saturated zone depth.

Science.gov (United States)

Huang, Menglu; Wang, Zhen; Qi, Ran

2017-06-01

This study was conducted to explore enhancement of the complete autotrophic nitrogen removal over nitrite (CANON) process in a modified single-stage subsurface vertical flow constructed wetland (VSSF) with saturated zone, and nitrogen transformation pathways in the VSSF treating digested swine wastewater were investigated at four different saturated zone depths (SZDs). SZD significantly affected nitrogen transformation pathways in the VSSF throughout the experiment. As the SZD was 45cm, the CANON process was enhanced most effectively in the system owing to the notable enhancement of anammox. Correspondingly, the VSSF had the best TN removal performance [(76.74±7.30)%] and lower N 2 O emission flux [(3.50±0.22)mg·(m 2 ·h) - 1 ]. It could be concluded that autotrophic nitrogen removal via CANON process could become a primary route for nitrogen removal in the VSSF with optimized microenvironment that developed as a result of the appropriate SZD. Copyright © 2017 Elsevier Ltd. All rights reserved.

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.

Transportation routing analysis geographic information system -- TRAGIS, a multimodal transportation routing tool

International Nuclear Information System (INIS)

Johnson, P.E.

1995-01-01

Over 15 years ago, Oak Ridge National Laboratory (ORNL) developed two transportation routing models: HIGHWAY, which predicts truck transportation routes, and INTERLINE, which predicts rail transportation routes. Subsequent modifications have been made to enhance each of these models. Some of these changes include population density information for routes, HM-164 routing regulations for highway route controlled quantities of radioactive materials (RAM) truck shipments, and inclusion of waterway routing into INTERLINE. The AIRPORT model, developed 2 years after the HIGHWAY and INTERLINE models, serves as an emergency response tool. This model identifies the nearest airports from a designated location. Currently, the AIRPORT model is inactive. The Transportation Management Division of the US Department of Energy held a Baseline Requirements Assessment Session on the HIGHWAY, INTERLINE, and AIRPORT models in April 1994 to bring together many users of these models and other experts in the transportation routing field to discuss these models and to decide on the capabilities that needed to be added. Of the many needs discussed, the primary one was to have the network databases within a geographic information system (GIS). As a result of the Baseline Requirements Session, the development of a new GIS model has been initiated. This paper will discuss the development of the new Transportation Routing Analysis GIS (TRAGIS) model at ORNL

VINE ROUTES IN BULGARIA

Directory of Open Access Journals (Sweden)

Lyuben Hristov

2015-03-01

Full Text Available The article deals with a scheme for the modern vine route in Bulgaria. Five basic vine routes and one international, between Bulgaria, Macedonia and Greece are defined. All routes consider characteristic varieties of grapes and kinds of vine products. Vine tourist products combined with visits of important natural and anthropological object are in the bases of the defined routes. The described routes are an important contribution to development of alternative tourist products in the country.

Robust against route failure using power proficient reliable routing in MANET

Directory of Open Access Journals (Sweden)

M. Malathi

2018-03-01

Full Text Available The aim of this paper was to propose a novel routing protocol for Mobile Adhoc Network communication which reduces the route failure during transmission. The proposed routing protocol uses 3 salient parameters to discover the path which ensure the reliable communication. The quality of the channel, link quality and energy level of the node are the major reasons for unintentional node failure in mobile Adhoc network. So the proposed routing protocol considers these three parameters to select the best forwarder node in the path. The reliable data communication is achieved by transmitting data via path selected by the proposed routing scheme has been proven using network simulator (NS2. Keywords: Channel quality, Link quality, Mobile Adhoc Network (MANET, Residual energy

Effect of electrochemical corrosion on the subsurface microstructure evolution of a CoCrMo alloy in albumin containing environment

International Nuclear Information System (INIS)

Wang, Zhongwei; Yan, Yu; Su, Yanjing; Qiao, Lijie

2017-01-01

Highlights: • Accelerated electrochemical corrosion results in severer plastic deformation with finer grains. • Lower applied potential can increase protein adsorption on sample surfaces. • The tribo-film decreases the shear stresses and relief subsurface deformation. • Tribocorrosion induced passive film can suppress the annihilation of stacking faults. - Abstract: The subsurface microstructures of metallic implants play a key role in bio-tribocorrosion. Due to wear or change of local environment, the implant surface can have inhomogeneous electrochemical corrosion properties. In this work, the effect of electrochemical corrosion conditions on the subsurface microstructure evolution of CoCrMo alloys for artificial joints was investigated. Transmission electron microscope (TEM) was employed to observe the subsurface microstructures of worn areas at different applied potentials in a simulated physiological solution. The results showed that applied potentials could affect the severity of the subsurface deformation not only by changing the surface passivation but also affecting the adsorption of protein on the alloy surface.

Effect of electrochemical corrosion on the subsurface microstructure evolution of a CoCrMo alloy in albumin containing environment

Energy Technology Data Exchange (ETDEWEB)

Wang, Zhongwei; Yan, Yu, E-mail: yanyu@ustb.edu.cn; Su, Yanjing; Qiao, Lijie

2017-06-01

Highlights: • Accelerated electrochemical corrosion results in severer plastic deformation with finer grains. • Lower applied potential can increase protein adsorption on sample surfaces. • The tribo-film decreases the shear stresses and relief subsurface deformation. • Tribocorrosion induced passive film can suppress the annihilation of stacking faults. - Abstract: The subsurface microstructures of metallic implants play a key role in bio-tribocorrosion. Due to wear or change of local environment, the implant surface can have inhomogeneous electrochemical corrosion properties. In this work, the effect of electrochemical corrosion conditions on the subsurface microstructure evolution of CoCrMo alloys for artificial joints was investigated. Transmission electron microscope (TEM) was employed to observe the subsurface microstructures of worn areas at different applied potentials in a simulated physiological solution. The results showed that applied potentials could affect the severity of the subsurface deformation not only by changing the surface passivation but also affecting the adsorption of protein on the alloy surface.

Enhanced wetting of Cu on ZnO by migration of subsurface oxygen vacancies

DEFF Research Database (Denmark)

Beinik, Igor; Helström, Matti; Jensen, Thomas Nørregaard

2015-01-01

is of utmost importance. The Cu/ZnO system is among the most investigated of such systems in model studies, but the presence of subsurface ZnO defects and their important role for adhesion on ZnO have been unappreciated so far. Here we reveal that the surface-directed migration of subsurface defects affects...... the Cu adhesion on polar ZnO(0001) in the technologically interesting temperature range up to 550 K. This leads to enhanced adhesion and ultimately complete wetting of ZnO(0001) by a Cu overlayer. On the basis of our experimental and computational results we demonstrate a mechanism which implies...

Final report - Microbial pathways for the reduction of mercury in saturated subsurface sediments

Energy Technology Data Exchange (ETDEWEB)

Tamar barkay; Lily Young; Gerben Zylstra

2009-08-25

Mercury is a component of mixed wastes that have contaminated vast areas of the deep subsurface as a result of nuclear weapon and energy production. While this mercury is mostly bound to soil constituents episodes of groundwater contamination are known in some cases resulting in potable water super saturated with Hg(0). Microbial processes that reduce Hg(II) to the elemental form Hg(0) in the saturated subsurface sediments may contribute to this problem. When we started the project, only one microbial pathway for the reduction of Hg(II), the one mediated by the mer operon in mercury resistant bacteria was known. As we had previously demonstrated that the mer mediated process occurred in highly contaminated environments (Schaefer et al., 2004), and mercury concentrations in the subsurface were reported to be low (Krabbenhoft and Babiarz, 1992), we hypothesized that other microbial processes might be active in reducing Hg(II) to Hg(0) in saturated subsurface environments. The specific goals of our projects were: (1) Investigating the potential for Hg(II) reduction under varying electron accepting conditions in subsurface sediments and relating these potential to mer gene distribution; and (2) Examining the physiological and biochemical characteristics of the interactions of anaerobic bacteria with mercury. The results are briefly summarized with references to published papers and manuscripts in preparation where details about our research can be found. Additional information may be found in copies of our published manuscripts and conference proceedings, and our yearly reports that were submitted through the RIMS system.

Geochemical characterization of subsurface sediments in the Netherlands

NARCIS (Netherlands)

Huisman, D.J.

1998-01-01

Traditionally, the Netherlands' subsurface is mainly used to obtain good quality drinking and industrial waters from the different aquifers. Due to the lack of space on the surface, increasing environmental problems and demand for energy, the subsurface will be used increasingly for other

Electrical Subsurface Grounding Analysis

International Nuclear Information System (INIS)

J.M. Calle

2000-01-01

The purpose and objective of this analysis is to determine the present grounding requirements of the Exploratory Studies Facility (ESF) subsurface electrical system and to verify that the actual grounding system and devices satisfy the requirements

Network optimization including gas lift and network parameters under subsurface uncertainty

Energy Technology Data Exchange (ETDEWEB)

Schulze-Riegert, R.; Baffoe, J.; Pajonk, O. [SPT Group GmbH, Hamburg (Germany); Badalov, H.; Huseynov, S. [Technische Univ. Clausthal, Clausthal-Zellerfeld (Germany). ITE; Trick, M. [SPT Group, Calgary, AB (Canada)

2013-08-01

Optimization of oil and gas field production systems poses a great challenge to field development due to complex and multiple interactions between various operational design parameters and subsurface uncertainties. Conventional analytical methods are capable of finding local optima based on single deterministic models. They are less applicable for efficiently generating alternative design scenarios in a multi-objective context. Practical implementations of robust optimization workflows integrate the evaluation of alternative design scenarios and multiple realizations of subsurface uncertainty descriptions. Production or economic performance indicators such as NPV (Net Present Value) are linked to a risk-weighted objective function definition to guide the optimization processes. This work focuses on an integrated workflow using a reservoir-network simulator coupled to an optimization framework. The work will investigate the impact of design parameters while considering the physics of the reservoir, wells, and surface facilities. Subsurface uncertainties are described by well parameters such as inflow performance. Experimental design methods are used to investigate parameter sensitivities and interactions. Optimization methods are used to find optimal design parameter combinations which improve key performance indicators of the production network system. The proposed workflow will be applied to a representative oil reservoir coupled to a network which is modelled by an integrated reservoir-network simulator. Gas-lift will be included as an explicit measure to improve production. An objective function will be formulated for the net present value of the integrated system including production revenue and facility costs. Facility and gas lift design parameters are tuned to maximize NPV. Well inflow performance uncertainties are introduced with an impact on gas lift performance. Resulting variances on NPV are identified as a risk measure for the optimized system design. A

Groundwater Salinity Simulation of a Subsurface Reservoir in Taiwan

Science.gov (United States)

Fang, H. T.

2015-12-01

The subsurface reservoir is located in Chi-Ken Basin, Pescadores (a group islands located at western part of Taiwan). There is no river in these remote islands and thus the freshwater supply is relied on the subsurface reservoir. The basin area of the subsurface reservoir is 2.14 km2 , discharge of groundwater is 1.27×106m3 , annual planning water supplies is 7.9×105m3 , which include for domestic agricultural usage. The annual average temperature is 23.3oC, average moisture is 80~85%, annual average rainfall is 913 mm, but ET rate is 1975mm. As there is no single river in the basin; the major recharge of groundwater is by infiltration. Chi-Ken reservoir is the first subsurface reservoir in Taiwan. Originally, the water quality of the reservoir is good. The reservoir has had the salinity problem since 1991 and it became more and more serious from 1992 until 1994. Possible reason of the salinity problem was the shortage of rainfall or the leakage of the subsurface barrier which caused the seawater intrusion. The present study aimed to determine the leakage position of subsurface barrier that caused the salinity problem. In order to perform the simulation for different possible leakage position of the subsurface reservoir, a Groundwater Modeling System (GMS) is used to define soils layer data, hydro-geological parameters, initial conditions, boundary conditions and the generation of three dimension meshes. A three dimension FEMWATER(Yeh , 1996) numerical model was adopted to find the possible leakage position of the subsurface barrier and location of seawater intrusion by comparing the simulation of different possible leakage with the observations. 1.By assuming the leakage position in the bottom of barrier, the simulated numerical result matched the observation better than the other assumed leakage positions. It showed that the most possible leakage position was at the bottom of the barrier. 2.The research applied three dimension FEMWATER and GMS as an interface

Abnormal resting-state connectivity of motor and cognitive networks in early manifest Huntington's disease.

Science.gov (United States)

Wolf, R C; Sambataro, F; Vasic, N; Depping, M S; Thomann, P A; Landwehrmeyer, G B; Süssmuth, S D; Orth, M

2014-11-01

Functional magnetic resonance imaging (fMRI) of multiple neural networks during the brain's 'resting state' could facilitate biomarker development in patients with Huntington's disease (HD) and may provide new insights into the relationship between neural dysfunction and clinical symptoms. To date, however, very few studies have examined the functional integrity of multiple resting state networks (RSNs) in manifest HD, and even less is known about whether concomitant brain atrophy affects neural activity in patients. Using MRI, we investigated brain structure and RSN function in patients with early HD (n = 20) and healthy controls (n = 20). For resting-state fMRI data a group-independent component analysis identified spatiotemporally distinct patterns of motor and prefrontal RSNs of interest. We used voxel-based morphometry to assess regional brain atrophy, and 'biological parametric mapping' analyses to investigate the impact of atrophy on neural activity. Compared with controls, patients showed connectivity changes within distinct neural systems including lateral prefrontal, supplementary motor, thalamic, cingulate, temporal and parietal regions. In patients, supplementary motor area and cingulate cortex connectivity indices were associated with measures of motor function, whereas lateral prefrontal connectivity was associated with cognition. This study provides evidence for aberrant connectivity of RSNs associated with motor function and cognition in early manifest HD when controlling for brain atrophy. This suggests clinically relevant changes of RSN activity in the presence of HD-associated cortical and subcortical structural abnormalities.

Scenario simulation based assessment of subsurface energy storage

Science.gov (United States)

Beyer, C.; Bauer, S.; Dahmke, A.

2014-12-01

Energy production from renewable sources such as solar or wind power is characterized by temporally varying power supply. The politically intended transition towards renewable energies in Germany („Energiewende") hence requires the installation of energy storage technologies to compensate for the fluctuating production. In this context, subsurface energy storage represents a viable option due to large potential storage capacities and the wide prevalence of suited geological formations. Technologies for subsurface energy storage comprise cavern or deep porous media storage of synthetic hydrogen or methane from electrolysis and methanization, or compressed air, as well as heat storage in shallow or moderately deep porous formations. Pressure build-up, fluid displacement or temperature changes induced by such operations may affect local and regional groundwater flow, geomechanical behavior, groundwater geochemistry and microbiology. Moreover, subsurface energy storage may interact and possibly be in conflict with other "uses" like drinking water abstraction or ecological goods and functions. An utilization of the subsurface for energy storage therefore requires an adequate system and process understanding for the evaluation and assessment of possible impacts of specific storage operations on other types of subsurface use, the affected environment and protected entities. This contribution presents the framework of the ANGUS+ project, in which tools and methods are developed for these types of assessments. Synthetic but still realistic scenarios of geological energy storage are derived and parameterized for representative North German storage sites by data acquisition and evaluation, and experimental work. Coupled numerical hydraulic, thermal, mechanical and reactive transport (THMC) simulation tools are developed and applied to simulate the energy storage and subsurface usage scenarios, which are analyzed for an assessment and generalization of the imposed THMC

A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles

DEFF Research Database (Denmark)

Southwell, Amber L; Skotte, Niels H; Villanueva, Erika B

2017-01-01

transgenes in Hu128/21 mice match the human HTT exon 1 reference sequence. Conversely, the BACHD transgene carries a floxed, synthetic exon 1 sequence. Hu128/21 mice will be useful for investigations of human HTT that cannot be addressed in Hu97/18 mice, for developing therapies targeted to exon 1......Huntington disease (HD) is a neurodegenerative disease caused by a mutation in the huntingtin (HTT) gene. HTT is a large protein, interacts with many partners and is involved in many cellular pathways, which are perturbed in HD. Therapies targeting HTT directly are likely to provide the most global......-length, genomic human HTT transgenes heterozygous for the HD mutation and polymorphisms associated with HD in populations of East Asian descent and in a minority of patients from other ethnic groups. Hu128/21 mice display a wide variety of HD-like phenotypes that are similar to YAC128 mice. Additionally, both...

The Role of Subsurface Properties on Transport of Water and Trace Gases: 1D Simulations at Selected Mars Landing Sites.

Science.gov (United States)

Karatekin, O.; Gloesener, E.; Dehant, V. M. A.

2017-12-01

In this work, water ice stability and water vapour transport through porous martian subsurface are studied using a 1D diffusive model. The role of adsorption on water transfer in martian conditions is investigated as well as the range of parameters that have the largest effect on gas transport. In addition, adsorption kinetics is considered to examine its influence on the water vapor exchange between the subsurface and the atmosphere. As methane has been detected in the martian atmosphere, the subsurface model is then used to study methane diffusion in the CH4/CO2/H2O system from variable depths under the surface. The results of subsurface gas transport at selected locations/landing sites are shown and implications for present/future observations are discussed.

Model for the prediction of subsurface strata movement due to underground mining

Science.gov (United States)

Cheng, Jianwei; Liu, Fangyuan; Li, Siyuan

2017-12-01

The problem of ground control stability due to large underground mining operations is often associated with large movements and deformations of strata. It is a complicated problem, and can induce severe safety or environmental hazards either at the surface or in strata. Hence, knowing the subsurface strata movement characteristics, and making any subsidence predictions in advance, are desirable for mining engineers to estimate any damage likely to affect the ground surface or subsurface strata. Based on previous research findings, this paper broadly applies a surface subsidence prediction model based on the influence function method to subsurface strata, in order to predict subsurface stratum movement. A step-wise prediction model is proposed, to investigate the movement of underground strata. The model involves a dynamic iteration calculation process to derive the movements and deformations for each stratum layer; modifications to the influence method function are also made for more precise calculations. The critical subsidence parameters, incorporating stratum mechanical properties and the spatial relationship of interest at the mining level, are thoroughly considered, with the purpose of improving the reliability of input parameters. Such research efforts can be very helpful to mining engineers’ understanding of the moving behavior of all strata over underground excavations, and assist in making any damage mitigation plan. In order to check the reliability of the model, two methods are carried out and cross-validation applied. One is to use a borehole TV monitor recording to identify the progress of subsurface stratum bedding and caving in a coal mine, the other is to conduct physical modelling of the subsidence in underground strata. The results of these two methods are used to compare with theoretical results calculated by the proposed mathematical model. The testing results agree well with each other, and the acceptable accuracy and reliability of the

Final report - Reduction of mercury in saturated subsurface sediments and its potential to mobilize mercury in its elemental form

Energy Technology Data Exchange (ETDEWEB)

Bakray, Tamar [Rutgers University

2013-06-13

The goal of our project was to investigate Hg(II) reduction in the deep subsurface. We focused on microbial and abiotic pathways of reduction and explored how it affected the toxicity and mobility of Hg in this unique environment. The project’s tasks included: 1. Examining the role of mer activities in the reduction of Hg(II) in denitrifying enrichment cultures; 2. Investigating the biotic/abiotic reduction of Hg(II) under iron reducing conditions; 3. Examining Hg(II) redox transformations under anaerobic conditions in subsurface sediments from DOE sites.

Heating systems for heating subsurface formations

Science.gov (United States)

Nguyen, Scott Vinh [Houston, TX; Vinegar, Harold J [Bellaire, TX

2011-04-26

Methods and systems for heating a subsurface formation are described herein. A heating system for a subsurface formation includes a sealed conduit positioned in an opening in the formation and a heat source. The sealed conduit includes a heat transfer fluid. The heat source provides heat to a portion of the sealed conduit to change phase of the heat transfer fluid from a liquid to a vapor. The vapor in the sealed conduit rises in the sealed conduit, condenses to transfer heat to the formation and returns to the conduit portion as a liquid.

Uranium Biomineralization By Natural Microbial Phosphatase Activities in the Subsurface

Energy Technology Data Exchange (ETDEWEB)

Taillefert, Martial [Georgia Tech Research Corporation, Atlanta, GA (United States)

2015-04-01

This project investigated the geochemical and microbial processes associated with the biomineralization of radionuclides in subsurface soils. During this study, it was determined that microbial communities from the Oak Ridge Field Research subsurface are able to express phosphatase activities that hydrolyze exogenous organophosphate compounds and result in the non-reductive bioimmobilization of U(VI) phosphate minerals in both aerobic and anaerobic conditions. The changes of the microbial community structure associated with the biomineralization of U(VI) was determined to identify the main organisms involved in the biomineralization process, and the complete genome of two isolates was sequenced. In addition, it was determined that both phytate, the main source of natural organophosphate compounds in natural environments, and polyphosphate accumulated in cells could also be hydrolyzed by native microbial population to liberate enough orthophosphate and precipitate uranium phosphate minerals. Finally, the minerals produced during this process are stable in low pH conditions or environments where the production of dissolved inorganic carbon is moderate. These findings suggest that the biomineralization of U(VI) phosphate minerals is an attractive bioremediation strategy to uranium bioreduction in low pH uranium-contaminated environments. These efforts support the goals of the SBR long-term performance measure by providing key information on "biological processes influencing the form and mobility of DOE contaminants in the subsurface".

Integrated geomechanical modelling at TNO for assessement of deep subsurface risks

NARCIS (Netherlands)

Orlic, B.; Fokker, P.; Zijl, W.; Scheffers, B.

2001-01-01

Public authorities, E & P and the mining industry increasingly demand fundamental insight and accurate predictions on subsurface and surface deformation and damage due to exploitation of subsurface natural resources, and subsurface storage of energy residues (e.g. CO2). At this moment deformation is

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

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

STRUCTURE AND FUNCTION OF SUBSURFACE MICROBIAL COMMUNITIES AFFECTING RADIONUCLIDE TRANSPORT AND BIOIMMOBILIZATION

Energy Technology Data Exchange (ETDEWEB)

Joel E. Kostka; Lee Kerkhof; Kuk-Jeong Chin; Martin Keller; Joseph W. Stucki

2011-06-15

The objectives of this project were to: (1) isolate and characterize novel anaerobic prokaryotes from subsurface environments exposed to high levels of mixed contaminants (U(VI), nitrate, sulfate), (2) elucidate the diversity and distribution of metabolically active metal- and nitrate-reducing prokaryotes in subsurface sediments, and (3) determine the biotic and abiotic mechanisms linking electron transport processes (nitrate, Fe(III), and sulfate reduction) to radionuclide reduction and immobilization. Mechanisms of electron transport and U(VI) transformation were examined under near in situ conditions in sediment microcosms and in field investigations at the Oak Ridge Field Research Center (ORFRC), in Oak Ridge, Tennessee, where the subsurface is exposed to mixed contamination predominated by uranium and nitrate. A total of 20 publications (16 published or 'in press' and 4 in review), 10 invited talks, and 43 contributed seminars/ meeting presentations were completed during the past four years of the project. PI Kostka served on one proposal review panel each year for the U.S. DOE Office of Science during the four year project period. The PI leveraged funds from the state of Florida to purchase new instrumentation that aided the project. Support was also leveraged by the PI from the Joint Genome Institute in the form of two successful proposals for genome sequencing. Draft genomes are now available for two novel species isolated during our studies and 5 more genomes are in the pipeline. We effectively addressed each of the three project objectives and research highlights are provided. Task I - Isolation and characterization of novel anaerobes: (1) A wide range of pure cultures of metal-reducing bacteria, sulfate-reducing bacteria, and denitrifying bacteria (32 strains) were isolated from subsurface sediments of the Oak Ridge Field Research Center (ORFRC), where the subsurface is exposed to mixed contamination of uranium and nitrate. These isolates which

Anatomy of Old Faithful from subsurface seismic imaging of the Yellowstone Upper Geyser Basin

KAUST Repository

Wu, Sin-Mei

2017-10-03

The Upper Geyser Basin in Yellowstone National Park contains one of the highest concentrations of hydrothermal features on Earth including the iconic Old Faithful geyser. Although this system has been the focus of many geological, geochemical, and geophysical studies for decades, the shallow (<200 m) subsurface structure remains poorly characterized. To investigate the detailed subsurface geologic structure including the hydrothermal plumbing of the Upper Geyser Basin, we deployed an array of densely spaced three-component nodal seismographs in November of 2015. In this study, we extract Rayleigh-wave seismic signals between 1-10 Hz utilizing non-diffusive seismic waves excited by nearby active hydrothermal features with the following results. 1) imaging the shallow subsurface structure by utilizing stationary hydrothermal activity as a seismic source, 2) characterizing how local geologic conditions control the formation and location of the Old Faithful hydrothermal system, and 3) resolving a relatively shallow (10-60 m) and large reservoir located ~100 m southwest of Old Faithful geyser.

Patterns of False Memory in Patients with Huntington's Disease.

Science.gov (United States)

Chen, I-Wen; Chen, Chiung-Mei; Wu, Yih-Ru; Hua, Mau-Sun

2017-06-01

Increased false memory recognition in patients with Huntington's disease (HD) has been widely reported; however, the underlying memory constructive processes remain unclear. The present study explored gist memory, item-specific memory, and monitoring ability in patients with HD. Twenty-five patients (including 13 patients with mild HD and 12 patients with moderate-to-severe HD) and 30 healthy comparison participants (HC) were recruited. We used the Deese-Roediger-McDermott (DRM) paradigm to investigate participants' false recognition patterns, along with neuropsychological tests to assess general cognitive function. Both mild and moderate-to-severe patients with HD showed significant executive functioning and episodic memory impairment. On the DRM tasks, both HD patient groups showed significantly impaired performance in tasks assessing unrelated false recognition and item-specific memory as compared to the HC group; moderate-to-severe patients performed more poorly than mild patients did. Only moderate-severe patients exhibited significantly poorer related false recognition index scores than HCs in the verbal DRM task; performance of HD patient groups was comparable to the HC group on the pictorial DRM task. It appears that diminished verbatim memory and monitoring ability are early signs of cognitive decline during the HD course. Conversely, gist memory is relatively robust, with only partial decline during advanced-stage HD. Our findings suggest that medial temporal lobe function is relatively preserved compared to that of frontal-related structures in early HD. Thus, gist-based memory rehabilitation programs might be beneficial for patients with HD. © The Author 2017. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

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.

Route churn: an analysis of low-cost carrier route continuity in Europe

NARCIS (Netherlands)

de Wit, J.G.; Zuidberg, J.

2016-01-01

Discontinuity of air routes is a subject that has been analysed in various ways. For example, the complex network approach focuses on network robustness and resilience due to route interruptions during a relatively short period. Also seasonal interruptions of air routes are a well-documented

Direct thermal effects of the Hadean bombardment did not limit early subsurface habitability

Science.gov (United States)

Grimm, R. E.; Marchi, S.

2018-03-01

Intense bombardment is considered characteristic of the Hadean and early Archean eons, yet some detrital zircons indicate that near-surface water was present and thus at least intervals of clement conditions may have existed. We investigate the habitability of the top few kilometers of the subsurface by updating a prior approach to thermal evolution of the crust due to impact heating, using a revised bombardment history, a more accurate thermal model, and treatment of melt sheets from large projectiles (>100 km diameter). We find that subsurface habitable volume grows nearly continuously throughout the Hadean and early Archean (4.5-3.5 Ga) because impact heat is dissipated rapidly compared to the total duration and waning strength of the bombardment. Global sterilization was only achieved using an order of magnitude more projectiles in 1/10 the time. Melt sheets from large projectiles can completely resurface the Earth several times prior to ∼4.2 Ga but at most once since then. Even in the Hadean, melt sheets have little effect on habitability because cooling times are short compared to resurfacing intervals, allowing subsurface biospheres to be locally re-established by groundwater infiltration between major impacts. Therefore the subsurface is always habitable somewhere, and production of global steam or silicate-vapor atmospheres are the only remaining avenues to early surface sterilization by bombardment.

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

Corn stover harvest increases herbicide movement to subsurface drains: RZWQM simulations

Science.gov (United States)

Shipitalo, Martin J.; Malone, Robert W.; Ma, Liwang; Nolan, Bernard T.; Kanwar, Rameshwar S.; Shaner, Dale L.; Pederson, Carl H.

2016-01-01

BACKGROUND Crop residue removal for bioenergy production can alter soil hydrologic properties and the movement of agrochemicals to subsurface drains. The Root Zone Water Quality Model (RZWQM), previously calibrated using measured flow and atrazine concentrations in drainage from a 0.4 ha chisel-tilled plot, was used to investigate effects of 50 and 100% corn (Zea mays L.) stover harvest and the accompanying reductions in soil crust hydraulic conductivity and total macroporosity on transport of atrazine, metolachlor, and metolachlor oxanilic acid (OXA). RESULTS The model accurately simulated field-measured metolachlor transport in drainage. A 3-yr simulation indicated that 50% residue removal decreased subsurface drainage by 31% and increased atrazine and metolachlor transport in drainage 4 to 5-fold when surface crust conductivity and macroporosity were reduced by 25%. Based on its measured sorption coefficient, ~ 2-fold reductions in OXA losses were simulated with residue removal. CONCLUSION RZWQM indicated that if corn stover harvest reduces crust conductivity and soil macroporosity, losses of atrazine and metolachlor in subsurface drainage will increase due to reduced sorption related to more water moving through fewer macropores. Losses of the metolachlor degradation product OXA will decrease due to the more rapid movement of the parent compound into the soil.

Nested sampling algorithm for subsurface flow model selection, uncertainty quantification, and nonlinear calibration

KAUST Repository

Elsheikh, A. H.

2013-12-01

Calibration of subsurface flow models is an essential step for managing ground water aquifers, designing of contaminant remediation plans, and maximizing recovery from hydrocarbon reservoirs. We investigate an efficient sampling algorithm known as nested sampling (NS), which can simultaneously sample the posterior distribution for uncertainty quantification, and estimate the Bayesian evidence for model selection. Model selection statistics, such as the Bayesian evidence, are needed to choose or assign different weights to different models of different levels of complexities. In this work, we report the first successful application of nested sampling for calibration of several nonlinear subsurface flow problems. The estimated Bayesian evidence by the NS algorithm is used to weight different parameterizations of the subsurface flow models (prior model selection). The results of the numerical evaluation implicitly enforced Occam\\'s razor where simpler models with fewer number of parameters are favored over complex models. The proper level of model complexity was automatically determined based on the information content of the calibration data and the data mismatch of the calibrated model.

Crystal Orientation Effect on the Subsurface Deformation of Monocrystalline Germanium in Nanometric Cutting.

Science.gov (United States)

Lai, Min; Zhang, Xiaodong; Fang, Fengzhou

2017-12-01

Molecular dynamics simulations of nanometric cutting on monocrystalline germanium are conducted to investigate the subsurface deformation during and after nanometric cutting. The continuous random network model of amorphous germanium is established by molecular dynamics simulation, and its characteristic parameters are extracted to compare with those of the machined deformed layer. The coordination number distribution and radial distribution function (RDF) show that the machined surface presents the similar amorphous state. The anisotropic subsurface deformation is studied by nanometric cutting on the (010), (101), and (111) crystal planes of germanium, respectively. The deformed structures are prone to extend along the 110 slip system, which leads to the difference in the shape and thickness of the deformed layer on various directions and crystal planes. On machined surface, the greater thickness of subsurface deformed layer induces the greater surface recovery height. In order to get the critical thickness limit of deformed layer on machined surface of germanium, the optimized cutting direction on each crystal plane is suggested according to the relevance of the nanometric cutting to the nanoindentation.

Next Generation Network Routing and Control Plane

DEFF Research Database (Denmark)

Fu, Rong

proved, the dominating Border Gateway Protocol (BGP) cannot address all the issues that in inter-domain QoS routing. Thus a new protocol or network architecture has to be developed to be able to carry the inter-domain traffic with the QoS and TE consideration. Moreover, the current network control also...... lacks the ability to cooperate between different domains and operators. The emergence of label switching transport technology such as of Multi-Protocol Label Switching (MPLS) or Generalized MPLS (GMPLS) supports the traffic transport in a finer granularity and more dedicated end-to-end Quality...... (RACF) provides the platform that enables cooperation and ubiquitous integration between networks. In this paper, we investigate in the network architecture, protocols and algorithms for inter-domain QoS routing and traffic engineering. The PCE based inter-domain routing architecture is enhanced...

Subsurface Contaminants Focus Area annual report 1997

International Nuclear Information System (INIS)

1997-01-01

In support of its vision for technological excellence, the Subsurface Contaminants Focus Area (SCFA) has identified three strategic goals. The three goals of the SCFA are: Contain and/or stabilize contamination sources that pose an imminent threat to surface and ground waters; Delineate DNAPL contamination in the subsurface and remediate DNAPL-contaminated soils and ground water; and Remove a full range of metal and radionuclide contamination in soils and ground water. To meet the challenges of remediating subsurface contaminants in soils and ground water, SCFA funded more than 40 technologies in fiscal year 1997. These technologies are grouped according to the following product lines: Dense Nonaqueous-Phase Liquids; Metals and Radionuclides; Source Term Containment; and Source Term Remediation. This report briefly describes the SCFA 1997 technologies and showcases a few key technologies in each product line

Validation of gravity data from the geopotential field model for subsurface investigation of the Cameroon Volcanic Line (Western Africa)

Science.gov (United States)

Marcel, Jean; Abate Essi, Jean Marcel; Nouck, Philippe Njandjock; Sanda, Oumarou; Manguelle-Dicoum, Eliézer

2018-03-01

Belonging to the Cameroon Volcanic Line (CVL), the western part of Cameroon is an active volcanic zone with volcanic eruptions and deadly gas emissions. The volcanic flows generally cover areas and bury structural features like faults. Terrestrial gravity surveys can hardly cover entirely this mountainous area due to difficult accessibility. The present work aims to evaluate gravity data derived from the geopotential field model, EGM2008 to investigate the subsurface of the CVL. The methodology involves upward continuation, horizontal gradient, maxima of horizontal gradient-upward continuation combination and Euler deconvolution techniques. The lineaments map inferred from this geopotential field model confirms several known lineaments and reveals new ones covered by lava flows. The known lineaments are interpreted as faults or geological contacts such as the Foumban fault and the Pan-African Belt-Congo craton contact. The lineaments highlighted coupled with the numerous maar lakes identified in this volcanic sector attest of the vulnerability of the CVL where special attention should be given for geohazard prevention.

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.

Progression of motor subtypes in Huntington's disease: a 6-year follow-up study.

Science.gov (United States)

Jacobs, M; Hart, E P; van Zwet, E W; Bentivoglio, A R; Burgunder, J M; Craufurd, D; Reilmann, R; Saft, C; Roos, R A C

2016-10-01

The objective of this study is to investigate the progression of predominantly choreatic and hypokinetic-rigid signs in Huntington's disease (HD) and their relationship with cognitive and general functioning over time. The motor signs in HD can be divided into predominantly choreatic and hypokinetic-rigid subtypes. It has been reported in cross-sectional studies that predominantly choreatic HD patients perform better on functional and cognitive assessments compared to predominantly hypokinetic-rigid HD patients. The course of these motor subtypes and their clinical profiles has not been investigated longitudinally. A total of 4135 subjects who participated in the European HD Network REGISTRY study were included and classified at baseline as either predominantly choreatic (n = 891), hypokinetic-rigid (n = 916), or mixed-motor (n = 2328), based on a previously used method. The maximum follow-up period was 6 years. The mixed-motor group was not included in the analyses. Linear mixed models were constructed to investigate changes in motor subtypes over time and their relationship with cognitive and functional decline. Over the 6-year follow-up period, the predominantly choreatic group showed a significant decrease in chorea, while hypokinetic-rigid symptoms slightly increased in the hypokinetic-rigid group. On the Total Functional Capacity, Stroop test, and Verbal fluency task the rate of change over time was significantly faster in the predominantly choreatic group, while on all other clinical assessments the decline was comparable for both groups. Our results suggest that choreatic symptoms decrease over time, whereas hypokinetic-rigid symptoms slightly increase in a large cohort of HD patients. Moreover, different motor subtypes can be related to different clinical profiles.

Translucent Radiosity: Efficiently Combining Diffuse Inter-Reflection and Subsurface Scattering.

Science.gov (United States)

Sheng, Yu; Shi, Yulong; Wang, Lili; Narasimhan, Srinivasa G

2014-07-01

It is hard to efficiently model the light transport in scenes with translucent objects for interactive applications. The inter-reflection between objects and their environments and the subsurface scattering through the materials intertwine to produce visual effects like color bleeding, light glows, and soft shading. Monte-Carlo based approaches have demonstrated impressive results but are computationally expensive, and faster approaches model either only inter-reflection or only subsurface scattering. In this paper, we present a simple analytic model that combines diffuse inter-reflection and isotropic subsurface scattering. Our approach extends the classical work in radiosity by including a subsurface scattering matrix that operates in conjunction with the traditional form factor matrix. This subsurface scattering matrix can be constructed using analytic, measurement-based or simulation-based models and can capture both homogeneous and heterogeneous translucencies. Using a fast iterative solution to radiosity, we demonstrate scene relighting and dynamically varying object translucencies at near interactive rates.

Grey matter volume loss is associated with specific clinical motor signs in Huntington's disease.

Science.gov (United States)

Coppen, Emma M; Jacobs, Milou; van den Berg-Huysmans, Annette A; van der Grond, Jeroen; Roos, Raymund A C

2018-01-01

Motor disturbances are clinical hallmarks of Huntington's disease (HD) and involve chorea, dystonia, hypokinesia and visuomotor dysfunction. Investigating the association between specific motor signs and different regional volumes is important to understand the heterogeneity of HD. To investigate the motor phenotype of HD and associations with subcortical and cortical grey matter volume loss. Structural T1-weighted MRI scans of 79 HD patients and 30 healthy controls were used to calculate volumes of seven subcortical structures including the nucleus accumbens, hippocampus, thalamus, caudate nucleus, putamen, pallidum and amygdala. Multiple linear regression analyses, corrected for age, gender, CAG, MRI scan protocol and normalized brain volume, were performed to assess the relationship between subcortical volumes and different motor subdomains (i.e. eye movements, chorea, dystonia, hypokinesia/rigidity and gait/balance). Voxel-based morphometry analysis was used to investigate the relationship between cortical volume changes and motor signs. Subcortical volume loss of the accumbens nucleus, caudate nucleus, putamen, and pallidum were associated with higher chorea scores. No other subcortical region was significantly associated with motor symptoms after correction for multiple comparisons. Voxel-based cortical grey matter volume reductions in occipital regions were related with an increase in eye movement scores. In HD, chorea is mainly associated with subcortical volume loss, while eye movements are more related to cortical volume loss. Both subcortical and cortical degeneration has an impact on motor impairment in HD. This implies that there is a widespread contribution of different brain regions resulting in the clinical motor presentation seen in HD patients. Copyright © 2017 Elsevier Ltd. All rights reserved.

Analyzing subsurface drain network performance in an agricultural monitoring site with a three-dimensional hydrological model

Science.gov (United States)

Nousiainen, Riikka; Warsta, Lassi; Turunen, Mika; Huitu, Hanna; Koivusalo, Harri; Pesonen, Liisa

2015-10-01

Effectiveness of a subsurface drainage system decreases with time, leading to a need to restore the drainage efficiency by installing new drain pipes in problem areas. The drainage performance of the resulting system varies spatially and complicates runoff and nutrient load generation within the fields. We presented a method to estimate the drainage performance of a heterogeneous subsurface drainage system by simulating the area with the three-dimensional hydrological FLUSH model. A GIS analysis was used to delineate the surface runoff contributing area in the field. We applied the method to reproduce the water balance and to investigate the effectiveness of a subsurface drainage network of a clayey field located in southern Finland. The subsurface drainage system was originally installed in the area in 1971 and the drainage efficiency was improved in 1995 and 2005 by installing new drains. FLUSH was calibrated against total runoff and drain discharge data from 2010 to 2011 and validated against total runoff in 2012. The model supported quantification of runoff fractions via the three installed drainage networks. Model realisations were produced to investigate the extent of the runoff contributing areas and the effect of the drainage parameters on subsurface drain discharge. The analysis showed that better model performance was achieved when the efficiency of the oldest drainage network (installed in 1971) was decreased. Our analysis method can reveal the drainage system performance but not the reason for the deterioration of the drainage performance. Tillage layer runoff from the field was originally computed by subtracting drain discharge from the total runoff. The drains installed in 1995 bypass the measurement system, which renders the tillage layer runoff calculation procedure invalid after 1995. Therefore, this article suggests use of a local correction coefficient based on the simulations for further research utilizing data from the study area.

Controlling Subsurface Fractures and Fluid Flow: A Basic Research Agenda

Energy Technology Data Exchange (ETDEWEB)

Pyrak-Nolte, Laura J [Purdue Univ., West Lafayette, IN (United States); DePaolo, Donald J. [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Univ. of California, Berkeley, CA (United States); Pietraß, Tanja [USDOE Office of Science, Washington, DC (United States)

2015-05-22

From beneath the surface of the earth, we currently obtain about 80-percent of the energy our nation consumes each year. In the future we have the potential to generate billions of watts of electrical power from clean, green, geothermal energy sources. Our planet’s subsurface can also serve as a reservoir for storing energy produced from intermittent sources such as wind and solar, and it could provide safe, long-term storage of excess carbon dioxide, energy waste products and other hazardous materials. However, it is impossible to underestimate the complexities of the subsurface world. These complexities challenge our ability to acquire the scientific knowledge needed for the efficient and safe exploitation of its resources. To more effectively harness subsurface resources while mitigating the impacts of developing and using these resources, the U.S. Department of Energy established SubTER – the Subsurface Technology and Engineering RD&D Crosscut team. This DOE multi-office team engaged scientists and engineers from the national laboratories to assess and make recommendations for improving energy-related subsurface engineering. The SubTER team produced a plan with the overall objective of “adaptive control of subsurface fractures and fluid flow.”This plan revolved around four core technological pillars—Intelligent Wellbore Systems that sustain the integrity of the wellbore environment; Subsurface Stress and Induced Seismicity programs that guide and optimize sustainable energy strategies while reducing the risks associated with subsurface injections; Permeability Manipulation studies that improve methods of enhancing, impeding and eliminating fluid flow; and New Subsurface Signals that transform our ability to see into and characterize subsurface systems. The SubTER team developed an extensive R&D plan for advancing technologies within these four core pillars and also identified several areas where new technologies would require additional basic research

Optimized Subsurface Irrigation System: The Future of Sugarcane Irrigation

Directory of Open Access Journals (Sweden)

M. H. J. P. Gunarathna

2018-03-01

Full Text Available Climate change may harm the growth and yield of sugarcane (Saccharum officinarum L. without the introduction of appropriate irrigation facilities. Therefore, new irrigation methods should be developed to maximize water use efficiency and reduce operational costs. OPSIS (optimized subsurface irrigation system is a new solar-powered automatic subsurface irrigation system that creates a phreatic zone below crop roots and relies on capillarity to supply water to the root zone. It is designed for upland crops such as sugarcane. We investigated the performance of OPSIS for irrigating sugarcane and evaluated its performance against sprinkler irrigation under subtropical conditions. We conducted field experiments in Okinawa, Japan, over the period from 2013 to 2016 and took measurements during spring- and summer-planted main crops and two ratoon crops of the spring-planted crop. Compared with sprinkler irrigation, OPSIS produced a significantly higher fresh cane yield, consumed less irrigation water and provided a higher irrigation water use efficiency. We conclude that OPSIS could be adopted as a sustainable solution to sugarcane irrigation in Okinawa and similar environments.

Hazmat Routes (National)

Data.gov (United States)

Department of Transportation — The Federal Motor Carrier Safety Administration (FMCSA) Hazardous Material Routes (NTAD) were developed using the 2004 First Edition TIGER/Line files. The routes are...

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.

Subsurface injection of dissolved ferric chloride to form a chemical barrier: Laboratory investigations

International Nuclear Information System (INIS)

Morrison, S.J.; Spangler, R.R.; Morris, S.A.

1996-01-01

A chemical barrier is a permeable zone of reactive materials emplaced in the subsurface to remove ground-water contaminants while allowing clean ground water to pass through. Because dissolved ferric chloride hydrolyzes to amorphous ferric oxyhydroxide when it contacts calcite (CaCO 3 ), it may be viable to emplace a zone of amorphous ferric oxyhydroxide (an absorbent for U, Mo, and other inorganic contaminants) into calcite-bearing geologic units by injecting ferric chloride through wells. For a chemical barrier to be successful, it must remain permeable and must be immobile. This investigation monitored chemical compositions, hydraulic conductivity, and iron mobility in laboratory columns and in a two-dimensional tank to determine the viability of injecting ferric chloride to form an amorphous ferric oxyhydroxide chemical barrier. The authors introduced a ferric chloride solution (1,345 mg/1[0.024 m] Fe) to calcite-bearing alluvial gravel to form a chemical barrier of amorphous ferric oxyhydroxide, followed by solutions contaminated with U and Mo. The simulated chemical barriers decreased U and Mo concentrations to less than 0.05 mg/l (2.1 x 10 -7 m) and 0.01 (1.0 x 10 -7 m), respectively; however, the breakthrough front is spread out with concentrations increasing to more than regulatory guideline values sooner than predicted. The hydraulic conductivity of calcite-bearing alluvial gravel decreased substantially during ferric chloride introduction because of the formation of carbon dioxide but increased to within factors of 1 to 5 of the original value as synthetic ground water flowed through the system. Amorphous ferric oxyhydroxide that formed in these experiments remained immobile at flow rates exceeding those typical of ground water. These laboratory results, in conjunction with site-specific characterization data, can be used to design chemical barriers emplaced by injection of ferric chloride

Bacterial communities associated with subsurface geochemical processes in continental serpentinite springs.

Science.gov (United States)

Brazelton, William J; Morrill, Penny L; Szponar, Natalie; Schrenk, Matthew O

2013-07-01

Reactions associated with the geochemical process of serpentinization can generate copious quantities of hydrogen and low-molecular-weight organic carbon compounds, which may provide energy and nutrients to sustain subsurface microbial communities independently of the photosynthetically supported surface biosphere. Previous microbial ecology studies have tested this hypothesis in deep sea hydrothermal vents, such as the Lost City hydrothermal field. This study applied similar methods, including molecular fingerprinting and tag sequencing of the 16S rRNA gene, to ultrabasic continental springs emanating from serpentinizing ultramafic rocks. These molecular surveys were linked with geochemical measurements of the fluids in an interdisciplinary approach designed to distinguish potential subsurface organisms from those derived from surface habitats. The betaproteobacterial genus Hydrogenophaga was identified as a likely inhabitant of transition zones where hydrogen-enriched subsurface fluids mix with oxygenated surface water. The Firmicutes genus Erysipelothrix was most strongly correlated with geochemical factors indicative of subsurface fluids and was identified as the most likely inhabitant of a serpentinization-powered subsurface biosphere. Both of these taxa have been identified in multiple hydrogen-enriched subsurface habitats worldwide, and the results of this study contribute to an emerging biogeographic pattern in which Betaproteobacteria occur in near-surface mixing zones and Firmicutes are present in deeper, anoxic subsurface habitats.

Evaluating Recall and Recognition Memory Using the Montreal Cognitive Assessment: Applicability for Alzheimer's and Huntington's Diseases.

Science.gov (United States)

Van Liew, Charles; Santoro, Maya S; Goldstein, Jody; Gluhm, Shea; Gilbert, Paul E; Corey-Bloom, Jody

2016-12-01

We sought to investigate whether the Montreal Cognitive Assessment (MoCA) could provide a brief assessment of recall and recognition using Huntington disease (HD) and Alzheimer disease (AD) as disorders characterized by different memory deficits. This study included 80 participants with HD, 64 participants with AD, and 183 community-dwelling control participants. Random-effects hierarchical logistic regressions were performed to assess the relative performance of the normal control (NC), participants with HD, and participants with AD on verbal free recall, cued recall, and multiple-choice recognition on the MoCA. The NC participants performed significantly better than participants with AD at all the 3 levels of assessment. No difference existed between participants with HD and NC for cued recall, but NC participants performed significantly better than participants with HD on free recall and recognition. The participants with HD performed significantly better than participants with AD at all the 3 levels of assessment. The MoCA appears to be a valuable, brief cognitive assessment capable of identifying specific memory deficits consistent with known differences in memory profiles. © The Author(s) 2016.

Theory of mind and empathy in preclinical and clinical Huntington's disease.

Science.gov (United States)

Adjeroud, Najia; Besnard, Jérémy; El Massioui, Nicole; Verny, Christophe; Prudean, Adriana; Scherer, Clarisse; Gohier, Bénédicte; Bonneau, Dominique; Allain, Philippe

2016-01-01

We investigated cognitive and affective Theory of Mind (ToM) and empathy in patients with premanifest and manifest Huntington's disease (HD). The relationship between ToM performance and executive skills was also examined. Sixteen preclinical and 23 clinical HD patients, and 39 healthy subjects divided into 2 control groups were given a French adaptation of the Yoni test (Shamay-Tsoory, S.G., Aharon-Peretz, J. (2007). Dissociable prefrontal networks for cognitive and affective theory of mind: a lesion study. Neuropsychologia, 45(3), 3054-67) that examines first- and second-order cognitive and affective ToM processing in separate conditions with a physical control condition. Participants were also given questionnaires of empathy and cognitive tests which mainly assessed executive functions (inhibition and mental flexibility). Clinical HD patients made significantly more errors than their controls in the first- and second-order cognitive and affective ToM conditions of the Yoni task, but exhibited no empathy deficits. However, there was no evidence that ToM impairment was related to cognitive deficits in these patients. Preclinical HD patients were unimpaired in ToM tasks and empathy measures compared with their controls. Our results are consistent with the idea that impaired affective and cognitive mentalizing emerges with the clinical manifestation of HD, but is not necessarily part of the preclinical stage. Furthermore, these impairments appear independent of executive dysfunction and empathy. © The Author (2015). Published by Oxford University Press. For Permissions, please email: journals.permissions@oup.com.

On the Capacity of Hybrid Wireless Networks with Opportunistic Routing

Directory of Open Access Journals (Sweden)

Le Tan

2010-01-01

Full Text Available This paper studies the capacity of hybrid wireless networks with opportunistic routing (OR. We first extend the opportunistic routing algorithm to exploit high-speed data transmissions in infrastructure network through base stations. We then develop linear programming models to calculate the end-to-end throughput bounds from multiple source nodes to single as well as multiple destination nodes. The developed models are applied to study several hybrid wireless network examples. Through case studies, we investigate several factors that have significant impacts on the hybrid wireless network capacity under opportunistic routing, such as node transmission range, density and distribution pattern of base stations (BTs, and number of wireless channels on wireless nodes and base stations. Our numerical results demonstrate that opportunistic routing could achieve much higher throughput on both ad hoc and hybrid networks than traditional unicast routing (UR. Moreover, opportunistic routing can efficiently utilize base stations and achieve significantly higher throughput gains in hybrid wireless networks than in pure ad hoc networks especially with multiple-channel base stations.

GPR applied to mapping utilities along the route of the Line 4 (yellow) subway tunnel construction in São Paulo City, Brazil

Science.gov (United States)

Porsani, Jorge Luís; Ruy, Yang Boo; Ramos, Fernanda Pereira; Yamanouth, Gisele R. B.

2012-05-01

The rapid industrial development and disorganized population growth in huge cities bring about various urban problems due to intense use of physical space on and below the surface. Subsurface problems in metropolitan areas are caused by subway line construction, which often follows the routes of utility networks, such as electric and telephone cables, water and gas pipes, storm sewers, etc. Usually, the main problems are related to damage or destruction of preexisting utilities, often putting human lives at risk. With the purpose of minimizing risks, GPR-profiling with 200 MHz antennae was done at two sites, both located in downtown São Paulo, Brazil. The objectives of this work were to map utilities or existing infrastructure in the subsurface in order to orient the construction of the Line 4 (yellow) subway tunnel in São Paulo. GPR profiles can detect water pipes, utility networks in the subsurface, and concrete foundation columns or pilings in subsoil up to 2 m depth. In addition, the GPR profiles also provided details of the target shapes in the subsurface. GPR interpretations combined with lithological information from boreholes and trenches opened in the study areas were extremely important in mapping of the correct spatial distribution of buried utilities at these two sites in São Paulo. This information improves and updates maps of utility placement, serves as a basis for planning of the geotechnical excavation of the Line 4 (yellow) subway tunnel in São Paulo, helps minimize problems related to destruction of preexisting utilities in the subsoil, and avoids risk of dangerous accidents.

Reconfigurable Robust Routing for Mobile Outreach Network

Science.gov (United States)

Lin, Ching-Fang

2010-01-01

The Reconfigurable Robust Routing for Mobile Outreach Network (R3MOO N) provides advanced communications networking technologies suitable for the lunar surface environment and applications. The R3MOON techn ology is based on a detailed concept of operations tailored for luna r surface networks, and includes intelligent routing algorithms and wireless mesh network implementation on AGNC's Coremicro Robots. The product's features include an integrated communication solution inco rporating energy efficiency and disruption-tolerance in a mobile ad h oc network, and a real-time control module to provide researchers an d engineers a convenient tool for reconfiguration, investigation, an d management.

Subsurface event detection and classification using Wireless Signal Networks.

Science.gov (United States)

Yoon, Suk-Un; Ghazanfari, Ehsan; Cheng, Liang; Pamukcu, Sibel; Suleiman, Muhannad T

2012-11-05

Subsurface environment sensing and monitoring applications such as detection of water intrusion or a landslide, which could significantly change the physical properties of the host soil, can be accomplished using a novel concept, Wireless Signal Networks (WSiNs). The wireless signal networks take advantage of the variations of radio signal strength on the distributed underground sensor nodes of WSiNs to monitor and characterize the sensed area. To characterize subsurface environments for event detection and classification, this paper provides a detailed list and experimental data of soil properties on how radio propagation is affected by soil properties in subsurface communication environments. Experiments demonstrated that calibrated wireless signal strength variations can be used as indicators to sense changes in the subsurface environment. The concept of WSiNs for the subsurface event detection is evaluated with applications such as detection of water intrusion, relative density change, and relative motion using actual underground sensor nodes. To classify geo-events using the measured signal strength as a main indicator of geo-events, we propose a window-based minimum distance classifier based on Bayesian decision theory. The window-based classifier for wireless signal networks has two steps: event detection and event classification. With the event detection, the window-based classifier classifies geo-events on the event occurring regions that are called a classification window. The proposed window-based classification method is evaluated with a water leakage experiment in which the data has been measured in laboratory experiments. In these experiments, the proposed detection and classification method based on wireless signal network can detect and classify subsurface events.

Subsurface characterization and geohydrologic site evaluation West Chestnut Ridge site

International Nuclear Information System (INIS)

1984-01-01

The West Chestnut Ridge Site at the Oak Ridge National Laboratory is being considered for use as a repository for low-level radioactive waste. The purposes of this study were to provide a geohydrological characterization of the site for use in pathways analysis, and to provide preliminary geotechnical recommendations that would be used for development of a site utilization plan. Subsurface conditions were investigated at twenty locations and observation wells were installed. Field testing at each location included the Standard Penetration Test and permeability tests in soil and rock. A well pumping test was ocmpleted at one site. Laboratory testing included permeability, deformability, strength and compaction tests, as well as index and physical property tests. The field investigations showed that the subsurface conditions include residual soil overlying a weathered zone of dolomite which grades into relatively unweathered dolomite at depth. The thickness of residual soil is typically 80 ft (24 m) on the ridges, but can be as little as 10 ft (3 m) in the valleys. Trench excavations to depths of 30 ft (9 m) should not present serious slope stability problems above the water table. On-site soils can be used for liners or trench backfill but these soils may require moisture conditioning to achieve required densities. 19 figures, 8 tables

Chemical Routes to Colloidal Chalcogenide Nanosheets

Energy Technology Data Exchange (ETDEWEB)

Schaak, Raymond E. [Pennsylvania State Univ., University Park, PA (United States)

2014-11-25

This project began with an emphasis on developing new low-­temperature synthetic routes to intermetallic alloys in order to advance the synthesis and processing science of superconducting materials, as well as to potentially discover new materials that would be inaccessible using more traditional higher-­temperature methods. Our initial target materials, chosen as model systems for developing new low-­temperature solution-­based synthetic platforms, were based on well known superconducting alloys, including Nb₃M (M = Ga, Ge, Sn), Bi-­M (M = In, Cu), and MgCNi₃. Several key, fundamental synthetic challenges that underpinned the formation of these materials using solution chemistry routes were identified and investigated.

Investigation of a subsidence event near Flushing, Ohio

International Nuclear Information System (INIS)

Ledney, C.M.; Hawk, J.L.

1994-01-01

An investigation was undertaken to determine the cause and extent of events which caused problems to a number of residences along State Route 149 near Flushing, Belmont County, Ohio. The events began in 1988 and continued through 1991 and affected nine homes. The type of problems occurring, as well as surface effects, compared to available mine maps of the area, indicated the problems were caused by subsidence from coal mining. The mining occurred in the Pittsburgh seam at a depth of between 180 and 220 feet. The mining beneath the site took place between 1975 and 1977 and was of the room and pillar type. A subsurface investigation was performed, along with ''down the hole'' video camera inspections to provide necessary subsurface information for analysis of the subsidence event. Factors of safety were calculated for pillars throughout the mine. Based on this analysis, it was determined that pillar failure caused the subsidence event. Once a determination was made as to the likely cause of the subsidence, the data was re-examined to determine the possible location of pillar failure, as well as the type and extent of subsidence. This analysis involved the use of RQD versus depth plots and the compilation of isopach maps of the mine overburden and the Sewickley Sandstone. The trend of the two maps suggested that a relationship existed between the sandstone thickness, the overburden and the surface expression of the subsidence. In order to determine this relationship, the two maps were combined into a second order map showing the mine overburden--Sewickley Sandstone thickness ratios. The combination was accomplished by computer matrix operations using the grid values of the two previous maps that were generated by kriging. It was concluded that the ratio of the Sewickley Sandstone thickness to the mine overburden had a tremendous effect on the amount of damage that occurred to specific residences

The Mojave Subsurface Bio-Geochemistry Explorer (MOSBE)

Science.gov (United States)

Guerrero, J.; Beegle, L.; Abbey, W.; Bhartia, R.; Kounaves, S.; Russell, M.; Towles, D.

2012-01-01

The MOSBE Team has developed a terrestrial field campaign to explore two subsurface biological habitats under the Mojave Desert. This field campaign will not only help us understand terrestrial desert biology, but also will develop methodologies and strategies for potential future Mars missions that would seek to explore the Martian subsurface. We have proposed to the ASTEP program to integrate a suite of field demonstrated instruments with a 20 m subsurface drill as a coherent unit, the Mojave Subsurface Bio-geochemistry Explorer. The ATK Space Modular Planetary Drill System (MPDS) requires no drilling fluid, which allows aseptic sampling, can penetrate lithic ground up to 20 meters of depth, and utilizes less than 100 Watts throughout the entire depth. The drill has been developed and demonstrated in field testing to a depth of 10 meters in Arizona, December 2002. In addition to caching a continuous core throughout the drilling depth, it also generates and caches cuttings and fines that are strata-graphically correlated with the core. As a core segment is brought to the surface, it will be analyzed for texture and structure by a color microscopic imager and for relevant chemistry and mineralogy with a UV fluorescence/Raman spectrometer. Organic and soluble ionic species will be identified through two instruments -- a microcapillary electrophoresis, and an ion trap mass spectrometer that have been developed under PIDDP, ASTID and MIDP funding.

Geophysical characterization of subsurface barriers

International Nuclear Information System (INIS)

Borns, D.J.

1995-08-01

An option for controlling contaminant migration from plumes and buried waste sites is to construct a subsurface barrier of a low-permeability material. The successful application of subsurface barriers requires processes to verify the emplacement and effectiveness of barrier and to monitor the performance of a barrier after emplacement. Non destructive and remote sensing techniques, such as geophysical methods, are possible technologies to address these needs. The changes in mechanical, hydrologic and chemical properties associated with the emplacement of an engineered barrier will affect geophysical properties such a seismic velocity, electrical conductivity, and dielectric constant. Also, the barrier, once emplaced and interacting with the in situ geologic system, may affect the paths along which electrical current flows in the subsurface. These changes in properties and processes facilitate the detection and monitoring of the barrier. The approaches to characterizing and monitoring engineered barriers can be divided between (1) methods that directly image the barrier using the contrasts in physical properties between the barrier and the host soil or rock and (2) methods that reflect flow processes around or through the barrier. For example, seismic methods that delineate the changes in density and stiffness associated with the barrier represents a direct imaging method. Electrical self potential methods and flow probes based on heat flow methods represent techniques that can delineate the flow path or flow processes around and through a barrier

Geosystem services:A concept in support of sustainable development of the subsurface

NARCIS (Netherlands)

van Ree, C.C.D.F.; van Beukering, P.J.H.

2016-01-01

Because functions of the subsurface are hidden from view, its important role in society is often taken for granted. Underground use in cities and subsurface resource extraction rapidly increase. Ensuring sustainability of the subsurface role requires balancing between exploitation and conservation,

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...

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.

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.

Considerations in the development of subsurface containment barrier performance standards

International Nuclear Information System (INIS)

Dunstan, S.; Zdinak, A.P.; Lodman, D.

1997-01-01

The U.S. Department of Energy (DOE) is supporting subsurface barriers as an alternative remedial option for management of contamination problems at their facilities. Past cleanup initiatives have sometimes proven ineffective or extremely expensive. Economic considerations coupled with changing public and regulatory philosophies regarding remediation techniques makes subsurface barriers a promising technology for future cleanup efforts. As part of the initiative to develop subsurface containment barriers as an alternative remedial option, DOE funded MSE Technology Applications, Inc. (MSE) to conduct a comprehensive review to identify performance considerations for the acceptability of subsurface barrier technologies as a containment method. Findings from this evaluation were intended to provide a basis for selection and application of containment technologies to address waste problems at DOE sites. Based on this study, the development of performance standards should consider: (1) sustainable low hydraulic conductivity; (2) capability to meet applicable regulations; (3) compatibility with subsurface environmental conditions; (4) durability and long-term stability; (5) repairability; and (6) verification and monitoring. This paper describes the approach for determining considerations for performance standards

Peeking Beneath the Caldera: Communicating Subsurface Knowledge of Newberry Volcano

Science.gov (United States)

Mark-Moser, M.; Rose, K.; Schultz, J.; Cameron, E.

2016-12-01

"Imaging the Subsurface: Enhanced Geothermal Systems and Exploring Beneath Newberry Volcano" is an interactive website that presents a three-dimensional subsurface model of Newberry Volcano developed at National Energy Technology Laboratory (NETL). Created using the Story Maps application by ArcGIS Online, this format's dynamic capabilities provide the user the opportunity for multimedia engagement with the datasets and information used to build the subsurface model. This website allows for an interactive experience that the user dictates, including interactive maps, instructive videos and video capture of the subsurface model, and linked information throughout the text. This Story Map offers a general background on the technology of enhanced geothermal systems and the geologic and development history of Newberry Volcano before presenting NETL's modeling efforts that support the installation of enhanced geothermal systems. The model is driven by multiple geologic and geophysical datasets to compare and contrast results which allow for the targeting of potential EGS sites and the reduction of subsurface uncertainty. This Story Map aims to communicate to a broad audience, and provides a platform to effectively introduce the model to researchers and stakeholders.

Surface and subsurface conditions in permafrost areas - a literature review

International Nuclear Information System (INIS)

Vidstrand, Patrik

2003-02-01

This report contains a summary of some of the information within existing technical and scientific literature on permafrost. Permafrost is viewed as one of the future climate driven process domains that may exist in Scandinavia, and that may give rise to significantly different surface and subsurface conditions than the present. Except for changes in the biosphere, permafrost may impact hydraulic, mechanical, and chemical subsurface processes and conditions. Permafrost and its influences on the subsurface conditions are thus of interest for the performance and safety assessments of deep geological waste repositories. The definition of permafrost is 'ground that stays at or below 0 deg C for at least two consecutive years'. Permafrost will effect the geological subsurface to some depth. How deep the permafrost may grow is a function of the heat balance, thermal conditions at the surface and within the ground, and the geothermal heat flux from the Earth's inner parts. The main chapters of the report summaries the knowledge on permafrost evolution, occurrence and distribution, and extracts information concerning hydrology and mechanical and chemical impacts due to permafrost related conditions. The results of a literature review are always dependent on the available literature. Concerning permafrost there is some literature available from investigations in the field of long-term repositories and some from mining industries. However, reports of these investigations are few and the bulk of permafrost literature comes from the science departments concerned with surficial processes (e.g. geomorphology, hydrology, agriculture, etc) and from engineering concerns, such as foundation of constructions and pipeline design. This focus within the permafrost research inevitably yields a biased but also an abundant amount of information on localised surficial processes and a limited amount on regional and deep permafrost characteristics. Possible conclusions are that there is

Surface and subsurface conditions in permafrost areas - a literature review

Energy Technology Data Exchange (ETDEWEB)

Vidstrand, Patrik [Bergab, Goeteborg (Sweden)

2003-02-01

This report contains a summary of some of the information within existing technical and scientific literature on permafrost. Permafrost is viewed as one of the future climate driven process domains that may exist in Scandinavia, and that may give rise to significantly different surface and subsurface conditions than the present. Except for changes in the biosphere, permafrost may impact hydraulic, mechanical, and chemical subsurface processes and conditions. Permafrost and its influences on the subsurface conditions are thus of interest for the performance and safety assessments of deep geological waste repositories. The definition of permafrost is 'ground that stays at or below 0 deg C for at least two consecutive years'. Permafrost will effect the geological subsurface to some depth. How deep the permafrost may grow is a function of the heat balance, thermal conditions at the surface and within the ground, and the geothermal heat flux from the Earth's inner parts. The main chapters of the report summaries the knowledge on permafrost evolution, occurrence and distribution, and extracts information concerning hydrology and mechanical and chemical impacts due to permafrost related conditions. The results of a literature review are always dependent on the available literature. Concerning permafrost there is some literature available from investigations in the field of long-term repositories and some from mining industries. However, reports of these investigations are few and the bulk of permafrost literature comes from the science departments concerned with surficial processes (e.g. geomorphology, hydrology, agriculture, etc) and from engineering concerns, such as foundation of constructions and pipeline design. This focus within the permafrost research inevitably yields a biased but also an abundant amount of information on localised surficial processes and a limited amount on regional and deep permafrost characteristics. Possible conclusions are that

Shot Peening Effects on Subsurface Layer Properties and Fatigue Performance of Case-Hardened 18CrNiMo7-6 Steel

Directory of Open Access Journals (Sweden)

H. S. Ho

2018-01-01

Full Text Available The present study is conducted with a dual-aim: firstly, to examine the effect of several single shot peening conditions on the subsurface layer properties and fatigue performance of the case-hardened 18CrNiMo7-6 steel, and secondly, to propose an optimized peening condition for improved fatigue performance. By carrying out the subsurface integrity analysis and fatigue testing, the underlying relationships among the peening process, subsurface layer property and fatigue performance are investigated, the way peening conditions affect the fatigue life and its associated scatter for the case-hardened 18CrNiMo7-6 steel is quantitatively assessed. The in-depth study shows that dual peening can be an optimized solution, for it is able to produce a subsurface layer with enhanced properties and eventually gain a significant improvement in fatigue performance.

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.

300 Area Integrated Field-Scale Subsurface Research Challenge (IFRC) Field Site Management Plan

Energy Technology Data Exchange (ETDEWEB)

Freshley, Mark D.

2008-12-31

Pacific Northwest National Laboratory (PNNL) has established the 300 Area Integrated Field-Scale Subsurface Research Challenge (300 Area IFRC) on the Hanford Site in southeastern Washington State for the U.S. Department of Energy’s (DOE) Office of Biological and Environmental Research (BER) within the Office of Science. The project is funded by the Environmental Remediation Sciences Division (ERSD). The purpose of the project is to conduct research at the 300 IFRC to investigate multi-scale mass transfer processes associated with a subsurface uranium plume impacting both the vadose zone and groundwater. The management approach for the 300 Area IFRC requires that a Field Site Management Plan be developed. This is an update of the plan to reflect the installation of the well network and other changes.

Natural biological variation of white matter microstructure is accentuated in Huntington's disease.

Science.gov (United States)

Gregory, Sarah; Crawford, Helen; Seunarine, Kiran; Leavitt, Blair; Durr, Alexandra; Roos, Raymund A C; Scahill, Rachael I; Tabrizi, Sarah J; Rees, Geraint; Langbehn, Douglas; Orth, Michael

2018-04-22

Huntington's disease (HD) is a monogenic neurodegenerative disorder caused by a CAG-repeat expansion in the Huntingtin gene. Presence of this expansion signifies certainty of disease onset, but only partly explains age at which onset occurs. Genome-wide association studies have shown that naturally occurring genetic variability influences HD pathogenesis and disease onset. Investigating the influence of biological traits in the normal population, such as variability in white matter properties, on HD pathogenesis could provide a complementary approach to understanding disease modification. We have previously shown that while white matter diffusivity patterns in the left sensorimotor network were similar in controls and HD gene-carriers, they were more extreme in the HD group. We hypothesized that the influence of natural variation in diffusivity on effects of HD pathogenesis on white matter is not limited to the sensorimotor network but extends to cognitive, limbic, and visual networks. Using tractography, we investigated 32 bilateral pathways within HD-related networks, including motor, cognitive, and limbic, and examined diffusivity metrics using principal components analysis. We identified three independent patterns of diffusivity common to controls and HD gene-carriers that predicted HD status. The first pattern involved almost all tracts, the second was limited to sensorimotor tracts, and the third encompassed cognitive network tracts. Each diffusivity pattern was associated with network specific performance. The consistency in diffusivity patterns across both groups coupled with their association with disease status and task performance indicates that naturally-occurring patterns of diffusivity can become accentuated in the presence of the HD gene mutation to influence clinical brain function. © 2018 The Authors Human Brain Mapping Published by Wiley Periodicals, Inc.

Indoor Semantic Modelling for Routing: The Two-Level Routing Approach for Indoor Navigation

Directory of Open Access Journals (Sweden)

Liu Liu

2017-11-01

Full Text Available Humans perform many activities indoors and they show a growing need for indoor navigation, especially in unfamiliar buildings such as airports, museums and hospitals. Complexity of such buildings poses many challenges for building managers and visitors. Indoor navigation services play an important role in supporting these indoor activities. Indoor navigation covers extensive topics such as: 1 indoor positioning and localization; 2 indoor space representation for navigation model generation; 3 indoor routing computation; 4 human wayfinding behaviours; and 5 indoor guidance (e.g., textual directories. So far, a large number of studies of pedestrian indoor navigation have presented diverse navigation models and routing algorithms/methods. However, the major challenge is rarely referred to: how to represent the complex indoor environment for pedestrians and conduct routing according to the different roles and sizes of users. Such complex buildings contain irregular shapes, large open spaces, complicated obstacles and different types of passages. A navigation model can be very complicated if the indoors are accurately represented. Although most research demonstrates feasible indoor navigation models and related routing methods in regular buildings, the focus is still on a general navigation model for pedestrians who are simplified as circles. In fact, pedestrians represent different sizes, motion abilities and preferences (e.g., described in user profiles, which should be reflected in navigation models and be considered for indoor routing (e.g., relevant Spaces of Interest and Points of Interest. In order to address this challenge, this thesis proposes an innovative indoor modelling and routing approach – two-level routing. It specially targets the case of routing in complex buildings for distinct users. The conceptual (first level uses general free indoor spaces: this is represented by the logical network whose nodes represent the spaces and edges

Martian sub-surface ionising radiation: biosignatures and geology

Directory of Open Access Journals (Sweden)

J. M. Ward

2007-07-01

Full Text Available The surface of Mars, unshielded by thick atmosphere or global magnetic field, is exposed to high levels of cosmic radiation. This ionising radiation field is deleterious to the survival of dormant cells or spores and the persistence of molecular biomarkers in the subsurface, and so its characterisation is of prime astrobiological interest. Here, we present modelling results of the absorbed radiation dose as a function of depth through the Martian subsurface, suitable for calculation of biomarker persistence. A second major implementation of this dose accumulation rate data is in application of the optically stimulated luminescence technique for dating Martian sediments.

We present calculations of the dose-depth profile in the Martian subsurface for various scenarios: variations of surface composition (dry regolith, ice, layered permafrost, solar minimum and maximum conditions, locations of different elevation (Olympus Mons, Hellas basin, datum altitude, and increasing atmospheric thickness over geological history. We also model the changing composition of the subsurface radiation field with depth compared between Martian locations with different shielding material, determine the relative dose contributions from primaries of different energies, and discuss particle deflection by the crustal magnetic fields.

Cultivation Of Deep Subsurface Microbial Communities

Science.gov (United States)

Obrzut, Natalia; Casar, Caitlin; Osburn, Magdalena R.

2018-01-01

The potential habitability of surface environments on other planets in our solar system is limited by exposure to extreme radiation and desiccation. In contrast, subsurface environments may offer protection from these stressors and are potential reservoirs for liquid water and energy that support microbial life (Michalski et al., 2013) and are thus of interest to the astrobiology community. The samples used in this project were extracted from the Deep Mine Microbial Observatory (DeMMO) in the former Homestake Mine at depths of 800 to 2000 feet underground (Osburn et al., 2014). Phylogenetic data from these sites indicates the lack of cultured representatives within the community. We used geochemical data to guide media design to cultivate and isolate organisms from the DeMMO communities. Media used for cultivation varied from heterotrophic with oxygen, nitrate or sulfate to autotrophic media with ammonia or ferrous iron. Environmental fluid was used as inoculum in batch cultivation and strains were isolated via serial transfers or dilution to extinction. These methods resulted in isolating aerobic heterotrophs, nitrate reducers, sulfate reducers, ammonia oxidizers, and ferric iron reducers. DNA sequencing of these strains is underway to confirm which species they belong to. This project is part of the NASA Astrobiology Institute Life Underground initiative to detect and characterize subsurface microbial life; by characterizing the intraterrestrials, the life living deep within Earth’s crust, we aim to understand the controls on how and where life survives in subsurface settings. Cultivation of terrestrial deep subsurface microbes will provide insight into the survival mechanisms of intraterrestrials guiding the search for these life forms on other planets.

Molecular analysis of deep subsurface bacteria

International Nuclear Information System (INIS)

Jimenez Baez, L.E.

1989-09-01

Deep sediments samples from site C10a, in Appleton, and sites, P24, P28, and P29, at the Savannah River Site (SRS), near Aiken, South Carolina were studied to determine their microbial community composition, DNA homology and mol %G+C. Different geological formations with great variability in hydrogeological parameters were found across the depth profile. Phenotypic identification of deep subsurface bacteria underestimated the bacterial diversity at the three SRS sites, since bacteria with the same phenotype have different DNA composition and less than 70% DNA homology. Total DNA hybridization and mol %G+C analysis of deep sediment bacterial isolates suggested that each formation is comprised of different microbial communities. Depositional environment was more important than site and geological formation on the DNA relatedness between deep subsurface bacteria, since more 70% of bacteria with 20% or more of DNA homology came from the same depositional environments. Based on phenotypic and genotypic tests Pseudomonas spp. and Acinetobacter spp.-like bacteria were identified in 85 million years old sediments. This suggests that these microbial communities might have been adapted during a long period of time to the environmental conditions of the deep subsurface

Multihop Wireless Networks Opportunistic Routing

CERN Document Server

Zeng, Kai; Li, Ming

2011-01-01

This book provides an introduction to opportunistic routing an emerging technology designed to improve the packet forwarding reliability, network capacity and energy efficiency of multihop wireless networks This book presents a comprehensive background to the technological challenges lying behind opportunistic routing. The authors cover many fundamental research issues for this new concept, including the basic principles, performance limit and performance improvement of opportunistic routing compared to traditional routing, energy efficiency and distributed opportunistic routing protocol desig

On routing strategy with finite-capacity effect on scale-free networks

International Nuclear Information System (INIS)

Tang, S.; Jiang, X.; Ma, L.; Zhang, Z.; Zheng, Z.

2010-01-01

We propose a class of systems with finite-capacity effect to investigate routing-strategy optimization. The local topology and the variable capacity, two crucial elements for routing, are naturally coupled by considering the interactions among packets. We show how the combination of these two elements controls the normal and efficient functioning of routing in the frame of condensation and coverage, respectively. Specifically, it is shown that the dynamic behaviors of diffusing packets exhibit condensation, for which exact results of the stationary state and phase transition are given. Further, we explore the diffusion coverage of routed packets through simulation. Various alternatives for the strategy parameters are illustrated to apply standard techniques to alleviate condensation and accelerate coverage. Our results provide a practical way for the design of optimal routing strategies in complex networks by the manipulation of a few parameters. (author)

Agriculture and wildlife: ecological implications of subsurface irrigation drainage

Science.gov (United States)

A. Dennis Lemly

1994-01-01

Subsurface agricultural irrigation drainage is a wastewater with the potential to severely impact wetlands and wildlife populations. Widespread poisoning of migratory birds by drainwater contaminants has occurred in the western United States and waterfowl populations are threatened in the Pacific and Central flyways. Irrigated agriculture could produce subsurface...

Echolalia or functional repetition in conversation--a case study of an individual with Huntington's disease.

Science.gov (United States)

Saldert, Charlotta; Hartelius, Lena

2011-01-01

In this case study, we investigated the use of repetition in an individual with a neurogenic communication disorder. We present an analysis of interaction in natural conversations between a woman with advanced Huntington's disease (HD), whose speech had been described as sometimes characterised by echolalia, and her personal assistant. The conversational interaction is analysed on a sequential level, and recurrent patterns are explored. Although the ability of the person with HD to interact is affected by chorea, word retrieval problems and reduced comprehension, she takes an active part in conversation. The conversational partner's contributions are often adapted to her communicative ability as they are formulated as questions or suggestions that can be elaborated on or responded to with a simple 'yes' or 'no'. The person with HD often repeats the words of her conversational partner in a way that extends her contributions and shows listenership, and this use of repetition is also frequent in ordinary conversations between non-brain-damaged individuals. The results show that the conversation partners in this case cooperate in making the conversation proceed and evolve, and that verbal repetition is used in a way that works as a strategy for compensating for the impairment.

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.

Nitrate and nitrite contamination of sub-surface water in some areas of North West Frontier Province (N.W.F.P.) Pakistan

International Nuclear Information System (INIS)

Khan, M.; Khawaja, M.A.; Imdadullah

1998-01-01

Over the past few years, nitrate and nitrite contamination of sub-surface water samples from Peshawar, Charsada, Mardan and Nowshera districts of NWFP has been studied. In all the areas under study, nitrate concentration of sub-surface water was found to be below WHO approved limit of 45 mg/l. Whereas city area after 1987 showed a decreasing level of nitrate contamination of sub-surface water, it appeared to be on the increase in water samples from the outskirts of Peshawar-Charsada road. No uniform increasing or decreasing patterns of nitrate contamination were observed for water samples from cantonment, University and Hayatabad, areas of Mardan, Charsada and Nowshera under study. The nitrate contamination of sub-surface water appeared to be due to both the agricultural activities as well as human and animal wastes. A few sub-surface water samples from Peshawar city, Mardan and Nowshera areas indicated high concentration of nitrite, which is alarming in view of the earlier reports showing absence of nitrite in water samples from these areas. However, since 1993, nitrite presence has not been detected in sub-surface water samples from all the areas under present investigation. (author)

Subsurface Event Detection and Classification Using Wireless Signal Networks

Directory of Open Access Journals (Sweden)

Muhannad T. Suleiman

2012-11-01

Full Text Available Subsurface environment sensing and monitoring applications such as detection of water intrusion or a landslide, which could significantly change the physical properties of the host soil, can be accomplished using a novel concept, Wireless Signal Networks (WSiNs. The wireless signal networks take advantage of the variations of radio signal strength on the distributed underground sensor nodes of WSiNs to monitor and characterize the sensed area. To characterize subsurface environments for event detection and classification, this paper provides a detailed list and experimental data of soil properties on how radio propagation is affected by soil properties in subsurface communication environments. Experiments demonstrated that calibrated wireless signal strength variations can be used as indicators to sense changes in the subsurface environment. The concept of WSiNs for the subsurface event detection is evaluated with applications such as detection of water intrusion, relative density change, and relative motion using actual underground sensor nodes. To classify geo-events using the measured signal strength as a main indicator of geo-events, we propose a window-based minimum distance classifier based on Bayesian decision theory. The window-based classifier for wireless signal networks has two steps: event detection and event classification. With the event detection, the window-based classifier classifies geo-events on the event occurring regions that are called a classification window. The proposed window-based classification method is evaluated with a water leakage experiment in which the data has been measured in laboratory experiments. In these experiments, the proposed detection and classification method based on wireless signal network can detect and classify subsurface events.

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...

Imaging subsurface geology and volatile organic compound plumes

International Nuclear Information System (INIS)

Qualheim, B.J.; Daley, P.F.; Johnson, V.; McPherrin, R.V.; Laguna, G.

1992-03-01

Lawrence Livermore National Laboratory (LLNL) (Fig. 1) is in the final stages of the Superfund decisionmaking process for site remediation and restoration. In the process of characterizing the subsurface of the LLNL site, we have developed unique methods of collecting, storing, retrieving, and imaging geologic and chemical data from more than 350 drill holes. The lateral and vertical continuity of subsurface paleostream channels were mapped for the entire LLNL site using geologic descriptions from core samples, cuttings, and interpretations from geophysical logs. A computer-aided design and drafting program, SLICE, written at LLNL, was used to create two-dimensional maps of subsurface sediments, and state-of-the-art software produced three-dimensional images of the volatile organic compound (VOC) plumes using data from water and core fluid analyses

Understanding individual routing behaviour.

Science.gov (United States)

Lima, Antonio; Stanojevic, Rade; Papagiannaki, Dina; Rodriguez, Pablo; González, Marta C

2016-03-01

Knowing how individuals move between places is fundamental to advance our understanding of human mobility (González et al. 2008 Nature 453, 779-782. (doi:10.1038/nature06958)), improve our urban infrastructure (Prato 2009 J. Choice Model. 2, 65-100. (doi:10.1016/S1755-5345(13)70005-8)) and drive the development of transportation systems. Current route-choice models that are used in transportation planning are based on the widely accepted assumption that people follow the minimum cost path (Wardrop 1952 Proc. Inst. Civ. Eng. 1, 325-362. (doi:10.1680/ipeds.1952.11362)), despite little empirical support. Fine-grained location traces collected by smart devices give us today an unprecedented opportunity to learn how citizens organize their travel plans into a set of routes, and how similar behaviour patterns emerge among distinct individual choices. Here we study 92 419 anonymized GPS trajectories describing the movement of personal cars over an 18-month period. We group user trips by origin-destination and we find that most drivers use a small number of routes for their routine journeys, and tend to have a preferred route for frequent trips. In contrast to the cost minimization assumption, we also find that a significant fraction of drivers' routes are not optimal. We present a spatial probability distribution that bounds the route selection space within an ellipse, having the origin and the destination as focal points, characterized by high eccentricity independent of the scale. While individual routing choices are not captured by path optimization, their spatial bounds are similar, even for trips performed by distinct individuals and at various scales. These basic discoveries can inform realistic route-choice models that are not based on optimization, having an impact on several applications, such as infrastructure planning, routing recommendation systems and new mobility solutions. © 2016 The Author(s).

Water and nitrogen requirements of subsurface drip irrigated pomegranate

Science.gov (United States)

Surface drip irrigation is a well-developed practice for both annual and perennial crops. The use of subsurface drip is a well-established practice in many annual row crops, e.g. tomatoes, strawberries, lettuce. However, the use of subsurface drip on perennial crops has been slow to develop. With th...

Subsurface chlorophyll maxima in the north-western Bay of Bengal

Digital Repository Service at National Institute of Oceanography (India)

Sarma, V.V.; Aswanikumar, V.

of thermocline suggests that the formation of the subsurface maximum is influencEd. by the presence of seasonal thermocline. Further the subsurface chlorophyll maximum is noticed within the depth ranges of ammonium maximum and nitracline, suggesting...

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

Novel routes to nanodispersed semiconductors

International Nuclear Information System (INIS)

Green, M.A.

1999-01-01

Novel synthetic routes to nanodispersed compound semiconductors using organometallic precursors have been developed. The quantum dots have been studied by optical absorption spectroscopy, photoluminescence spectroscopy, transmission electron microscopy, high resolution transmission electron microscopy, infra red spectroscopy and nuclear magnetic resonance. Polar Lewis base solvents such as tri-n-octylphosphine oxide and 4-ethylpyridine were utilized as both passivating agent and dispersing medium. In the the search for new solvent systems and passivating agents, and investigation was also made into the use of dimethyl sulfoxide as a reaction solvent and capping agent in the preparation of nanocrystalline CdS. Existing routes using metal alkyls and silylated precursors in hot TOPO were improved by substituting the metal alkyl with an metal alkyl adduct. Cadmium monothiocarbamate and a related precursor, cadmium thioacetate were investigated as possible single source precursors to nanometer sized CdS. The thermolysis of diorganophosphides in the Lewis bases coordinating solvent (4-ethylpridine) has been investigated, including studies of decompositon mechanisms, and quantum dots of Cd 3 P 2 , Zn 3 P 2 , Inp and GaP have been prepared. The synthesis of InAs using the metal chloride and an aminoarsenide precursor in 4-ethylpridine has also been developed. A simple method for the organization of III-V materials into glass like aggregates has been described. (author)

Relationship Between Storm Hydrograph Components and Subsurface Flow Processes in a Hilly Headwater Basin, Toyota, Japan

Science.gov (United States)

Tsujimura, M.; Asai, K.; Takei, R.

2001-05-01

Temporal and spatial distribution of tracer elements and subsurface flow processes were investigated to study relationship between storm hydrograph components and behavior of subsurface water in a headwater catchment of Toyota Hill, Aichi prefecture, central Japan. The catchment has an area of 0.857 ha with an altitude of 60 to 100 m, and is underlain by granite. The soil depth revealed by sounding test ranges from 0.5 to 4.0 m. Rain, stream, soil and ground waters were sampled once in a week, and the stream water was sampled at 5 to 60 minute intervals during rainstorms. The pressure head of subsurface water was monitored using tensiometers and piezometers nests, and the stream flow was monitored using V-notch weir. The stable isotopic ratios of deuterium and oxygen 18 and inorganic ion concentrations were determined on all water samples. The oxygen 18 isotopic ratio in stream water decreased with rainfall during the rainstorms. The ratio of event water component to the total runoff water at the peak discharge ranged from 16 to 92 %, and the event water ratio correlated with the peak discharge rate and rainfall intensity. The tesiometric data showed that the shallow subsurface water with low isotopic ratios at the lower slope discharged directly to the stream during the heavy rainstorms. The shallow subsurface flow at the lower slope and overland flow on the raiparian zone contributed much to the stream water chemistry during heavy rainstorms.

Progressive microstructural changes of the occipital cortex in Huntington's disease.

Science.gov (United States)

Odish, Omar F F; Reijntjes, Robert H A M; van den Bogaard, Simon J A; Roos, Raymund A C; Leemans, Alexander

2018-02-28

In this study we longitudinally investigated the rate of microstructural alterations in the occipital cortex in different stages of Huntington's disease (HD) by applying an automated atlas-based approach to diffusion MRI data. Twenty-two premanifest (preHD), 10 early manifest HD (early HD) and 24 healthy control subjects completed baseline and two year follow-up scans. The preHD group was stratified based on the predicted years to disease onset into a far (preHD-A) and near (preHD-B) to disease onset group. Clinical and behavioral measures were collected per assessment time point. An automated atlas-based DTI analysis approach was used to obtain the mean, axial and radial diffusivities of the occipital cortex. We found that the longitudinal rate of diffusivity change in the superior occipital gyrus (SOG), middle occipital gyrus (MOG), and inferior occipital gyrus (IOG) was significantly higher in early HD compared to both preHD and controls (all p's ≤ 0.005), which can be interpreted as an increased rate of microstructural degeneration. Furthermore, the change rate in the diffusivity of the MOG could significantly discriminate between preHD-B compared to preHD-A and the other groups (all p's ≤ 0.04). Finally, we found an inverse correlation between the Stroop Word Reading task and diffusivities in the SOG and MOG (all p's ≤ 0.01). These findings suggest that measures obtained from the occipital cortex can serve as sensitive longitudinal biomarkers for disease progression in preHD-B and early HD. These could in turn be used to assess potential effects of proposed disease modifying therapies.

Transport of Chemical Vapors from Subsurface Sources to Atmosphere as Affected by Shallow Subsurface and Atmospheric Conditions

Science.gov (United States)

Rice, A. K.; Smits, K. M.; Hosken, K.; Schulte, P.; Illangasekare, T. H.

2012-12-01

Understanding the movement and modeling of chemical vapor through unsaturated soil in the shallow subsurface when subjected to natural atmospheric thermal and mass flux boundary conditions at the land surface is of importance to applications such as landmine detection and vapor intrusion into subsurface structures. New, advanced technologies exist to sense chemical signatures at the land/atmosphere interface, but interpretation of these sensor signals to make assessment of source conditions remains a challenge. Chemical signatures are subject to numerous interactions while migrating through the unsaturated soil environment, attenuating signal strength and masking contaminant source conditions. The dominant process governing movement of gases through porous media is often assumed to be Fickian diffusion through the air phase with minimal or no quantification of other processes contributing to vapor migration, such as thermal diffusion, convective gas flow due to the displacement of air, expansion/contraction of air due to temperature changes, temporal and spatial variations of soil moisture and fluctuations in atmospheric pressure. Soil water evaporation and interfacial mass transfer add to the complexity of the system. The goal of this work is to perform controlled experiments under transient conditions of soil moisture, temperature and wind at the land/atmosphere interface and use the resulting dataset to test existing theories on subsurface gas flow and iterate between numerical modeling efforts and experimental data. Ultimately, we aim to update conceptual models of shallow subsurface vapor transport to include conditionally significant transport processes and inform placement of mobile sensors and/or networks. We have developed a two-dimensional tank apparatus equipped with a network of sensors and a flow-through head space for simulation of the atmospheric interface. A detailed matrix of realistic atmospheric boundary conditions was applied in a series of

Subsurface Noble Gas Sampling Manual

Energy Technology Data Exchange (ETDEWEB)

Carrigan, C. R. [Lawrence Livermore National Lab. (LLNL), Livermore, CA (United States); Sun, Y. [Lawrence Livermore National Lab. (LLNL), Livermore, CA (United States)

2017-09-18

The intent of this document is to provide information about best available approaches for performing subsurface soil gas sampling during an On Site Inspection or OSI. This information is based on field sampling experiments, computer simulations and data from the NA-22 Noble Gas Signature Experiment Test Bed at the Nevada Nuclear Security Site (NNSS). The approaches should optimize the gas concentration from the subsurface cavity or chimney regime while simultaneously minimizing the potential for atmospheric radioxenon and near-surface Argon-37 contamination. Where possible, we quantitatively assess differences in sampling practices for the same sets of environmental conditions. We recognize that all sampling scenarios cannot be addressed. However, if this document helps to inform the intuition of the reader about addressing the challenges resulting from the inevitable deviations from the scenario assumed here, it will have achieved its goal.

Yucca Mountain Project Subsurface Facilities Design

International Nuclear Information System (INIS)

Linden, A.; Saunders, R.S.; Boutin, R.J.; Harrington, P.G.; Lachman, K.D.; Trautner, L.J.

2002-01-01

Four units of the Topopah Springs formation (volcanic tuff) are considered for the proposed repository: the upper lithophysal, the middle non-lithophysal, the lower lithophysal, and the lower non-lithophysal. Yucca Mountain was recently designated the site for a proposed repository to dispose of spent nuclear fuel and high-level radioactive waste. Work is proceeding to advance the design of subsurface facilities to accommodate emplacing waste packages in the proposed repository. This paper summarized recent progress in the design of subsurface layout of the proposed repository. The original Site Recommendation (SR) concept for the subsurface design located the repository largely within the lower lithophysal zone (approximately 73%) of the Topopah The Site Recommendation characterized area suitable for emplacement consisted of the primary upper block, the lower block and the southern upper block extension. The primary upper block accommodated the mandated 70,000 metric tons of heavy metal (MTHM) at a 1.45 kW/m hear heat load. Based on further study of the Site Recommendation concept, the proposed repository siting area footprint was modified to make maximum use of available site characterization data, and thus, reduce uncertainties associated with performance assessment. As a result of this study, a modified repository footprint has been proposed and is presently being review for acceptance by the DOE. A panel design concept was developed to reduce overall costs and reduce the overall emplacement schedule. This concept provides flexibility to adjust the proposed repository subsurface layout with time, as it makes it unnecessary to ''commit'' to development of a large single panel at the earliest stages of construction. A description of the underground layout configuration and influencing factors that affect the layout configuration are discussed in the report

A study of Huntington disease-like syndromes in black South African patients reveals a single SCA2 mutation and a unique distribution of normal alleles across five repeat loci.

Science.gov (United States)

Baine, Fiona K; Peerbhai, Nabeelah; Krause, Amanda

2018-07-15

Huntington disease (HD) is a progressive neurodegenerative disease, characterised by a triad of movement disorder, emotional and behavioural disturbances and cognitive impairment. The underlying cause is an expanded CAG repeat in the huntingtin gene. For a small proportion of patients presenting with HD-like symptoms, the mutation in this gene is not identified and they are said to have a HD "phenocopy". South Africa has the highest number of recorded cases of an African-specific phenocopy, Huntington disease-like 2 (HDL2), caused by a repeat expansion in the junctophilin-3 gene. However, a significant proportion of black patients with clinical symptoms suggestive of HD still test negative for HD and HDL2. This study thus aimed to investigate five other loci associated with HD phenocopy syndromes - ATN1, ATXN2, ATXN7, TBP and C9orf72. In a sample of patients in whom HD and HDL2 had been excluded, a single expansion was identified in the ATXN2 gene, confirming a diagnosis of Spinocerebellar ataxia 2. The results indicate that common repeat expansion disorders do not contribute significantly to the HD-like phenotype in black South African patients. Importantly, allele sizing reveals unique distributions of normal repeat lengths across the associated loci in the African population studied. Copyright © 2018 Elsevier B.V. All rights reserved.

DEMONSTRATION BULLETIN: SUBSURFACE VOLATILIZATION AND VENTILATION SYSTEM - BROWN & ROOT ENVIRONMENTAL

Science.gov (United States)

The Subsurface Volatilization and Ventilation System (SVVS*) is an in-situ vacuum extraction/air sparging and bioremediation technology for the treatment of subsurface organic contamination in soil and groundwater. The technology, developed by Billings and Associates, Inc., and o...

Fiber Optic Bragg Grating Sensors for Thermographic Detection of Subsurface Anomalies

Science.gov (United States)

Allison, Sidney G.; Winfree, William P.; Wu, Meng-Chou

2009-01-01

Conventional thermography with an infrared imager has been shown to be an extremely viable technique for nondestructively detecting subsurface anomalies such as thickness variations due to corrosion. A recently developed technique using fiber optic sensors to measure temperature holds potential for performing similar inspections without requiring an infrared imager. The structure is heated using a heat source such as a quartz lamp with fiber Bragg grating (FBG) sensors at the surface of the structure to detect temperature. Investigated structures include a stainless steel plate with thickness variations simulated by small platelets attached to the back side using thermal grease. A relationship is shown between the FBG sensor thermal response and variations in material thickness. For comparison, finite element modeling was performed and found to agree closely with the fiber optic thermography results. This technique shows potential for applications where FBG sensors are already bonded to structures for Integrated Vehicle Health Monitoring (IVHM) strain measurements and can serve dual-use by also performing thermographic detection of subsurface anomalies.

SAFEBIKE: A Bike-sharing Route Recommender with Availability Prediction and Safe Routing

OpenAIRE

Zhong, Weisheng; Chen, Fanglan; Fu, Kaiqun; Lu, Chang-Tien

2017-01-01

This paper presents SAFEBIKE, a novel route recommendation system for bike-sharing service that utilizes station information to infer the number of available bikes in dock and recommend bike routes according to multiple factors such as distance and safety level. The system consists of a station level availability predictor that predicts bikes and docks amount at each station, and an efficient route recommendation service that considers safety and bike/dock availability factors. It targets use...

Diffusion and aggregation of subsurface radiation defects in lithium fluoride nanocrystals

Science.gov (United States)

Voitovich, A. P.; Kalinov, V. S.; Martynovich, E. F.; Stupak, A. P.; Runets, L. P.

2015-09-01

Lithium fluoride nanocrystals were irradiated by gamma rays at a temperature below the temperature corresponding to the mobility of anion vacancies. The kinetics of the aggregation of radiation-induced defects in subsurface layers of nanocrystals during annealing after irradiation was elucidated. The processes that could be used to determine the activation energy of the diffusion of anion vacancies were revealed. The value of this energy in subsurface layers was obtained. For subsurface layers, the concentrations ratio of vacancies and defects consisting of one vacancy and two electrons was found. The factors responsible for the differences in the values of the activation energies and concentration ratios in subsurface layers and in the bulk of the crystals were discussed.

Personal continuous route pattern mining

Institute of Scientific and Technical Information of China (English)

Qian YE; Ling CHEN; Gen-cai CHEN

2009-01-01

In the daily life, people often repeat regular routes in certain periods. In this paper, a mining system is developed to find the continuous route patterns of personal past trips. In order to count the diversity of personal moving status, the mining system employs the adaptive GPS data recording and five data filters to guarantee the clean trips data. The mining system uses a client/server architecture to protect personal privacy and to reduce the computational load. The server conducts the main mining procedure but with insufficient information to recover real personal routes. In order to improve the scalability of sequential pattern mining, a novel pattern mining algorithm, continuous route pattern mining (CRPM), is proposed. This algorithm can tolerate the different disturbances in real routes and extract the frequent patterns. Experimental results based on nine persons' trips show that CRPM can extract more than two times longer route patterns than the traditional route pattern mining algorithms.

Transformation of zinc-concentrate in surface and subsurface environments: Implications for assessing zinc mobility/toxicity and choosing an optimal remediation strategy

International Nuclear Information System (INIS)

Kwon, Man Jae; Boyanov, Maxim I.; Yang, Jung-Seok; Lee, Seunghak; Hwang, Yun Ho; Lee, Ju Yeon; Mishra, Bhoopesh; Kemner, Kenneth M.

2017-01-01

Zinc contamination in near- and sub-surface environments is a serious threat to many ecosystems and to public health. Sufficient understanding of Zn speciation and transport mechanisms is therefore critical to evaluating its risk to the environment and to developing remediation strategies. The geochemical and mineralogical characteristics of contaminated soils in the vicinity of a Zn ore transportation route were thoroughly investigated using a variety of analytical techniques (sequential extraction, XRF, XRD, SEM, and XAFS). Imported Zn-concentrate (ZnS) was deposited in a receiving facility and dispersed over time to the surrounding roadside areas and rice-paddy soils. Subsequent physical and chemical weathering resulted in dispersal into the subsurface. The species identified in the contaminated areas included Zn-sulfide, Zn-carbonate, other O-coordinated Zn-minerals, and Zn species bound to Fe/Mn oxides or clays, as confirmed by XAFS spectroscopy and sequential extraction. The observed transformation from S-coordinated Zn to O-coordinated Zn associated with minerals suggests that this contaminant can change into more soluble and labile forms as a result of weathering. For the purpose of developing a soil washing remediation process, the contaminated samples were extracted with dilute acids. The extraction efficiency increased with the increase of O-coordinated Zn relative to S-coordinated Zn in the sediment. This study demonstrates that improved understanding of Zn speciation in contaminated soils is essential for well-informed decision making regarding metal mobility and toxicity, as well as for choosing an appropriate remediation strategy using soil washing. - Graphical abstract: Graphical Abstract. Conceptual model of the apparent physical and geochemical processes controlling surface-subsurface partitioning of Zn in the study area. - Highlights: • Zn-concentrate accumulated in soils transformed to Zn species of various stability. • Zn species at our

A patient with aphasia using the nonsemantic lexical route for Kanji reading.

Science.gov (United States)

Hashimoto, Kosei; Uno, Akira; Sambai, Ami; Mizumoto, Go

We report a patient with aphasia, caused by cerebral hemorrhage, who probably used the nonsemantic lexical route when reading words aloud. To investigate the mechanisms underlying her reading dysfunction, we analyzed her reading abilities using the Dual-Route Cascaded Model. Language tests resulted in low correct percentages for both reading comprehension and reading nonwords aloud, suggesting problems in the semantic system and the nonlexical route. Conversely, the patient showed high scores on the reading words aloud task. Although she failed to understand many inconsistent-atypical words in the reading comprehension test, she correctly read most words aloud, suggesting that she used the nonsemantic lexical route. In addition, the lexical reading route was analyzed in detail by using inconsistent-atypical Kanji words as stimuli. Finally, we analyzed her reading dysfunction compared with previous cases.

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.)

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.

Quantitative subsurface analysis using frequency modulated thermal wave imaging

Science.gov (United States)

Subhani, S. K.; Suresh, B.; Ghali, V. S.

2018-01-01

Quantitative depth analysis of the anomaly with an enhanced depth resolution is a challenging task towards the estimation of depth of the subsurface anomaly using thermography. Frequency modulated thermal wave imaging introduced earlier provides a complete depth scanning of the object by stimulating it with a suitable band of frequencies and further analyzing the subsequent thermal response using a suitable post processing approach to resolve subsurface details. But conventional Fourier transform based methods used for post processing unscramble the frequencies with a limited frequency resolution and contribute for a finite depth resolution. Spectral zooming provided by chirp z transform facilitates enhanced frequency resolution which can further improves the depth resolution to axially explore finest subsurface features. Quantitative depth analysis with this augmented depth resolution is proposed to provide a closest estimate to the actual depth of subsurface anomaly. This manuscript experimentally validates this enhanced depth resolution using non stationary thermal wave imaging and offers an ever first and unique solution for quantitative depth estimation in frequency modulated thermal wave imaging.

Complex Systems Science for Subsurface Fate and Transport Report from the August 2009 Workshop

Energy Technology Data Exchange (ETDEWEB)

None

2010-03-01

in models can provide a basis for testing hypotheses, guiding experiment design, integrating scientific knowledge on multiple environmental systems into a common framework, and translating this information to support informed decision making and policies. Subsurface behavior typically has been investigated using reductionist, or bottom-up approaches. In these approaches, mechanisms of small-scale processes are quantified, and key aspects of their behaviors are moved up to the prediction scale using scaling laws and models. Reductionism has and will continue to yield essential and comprehensive understanding of the molecular and microscopic underpinnings of component processes. However, system-scale predictions cannot always be made with bottom-up approaches because the behaviors of subsurface environments often simply do not result from the sum of smaller-scale process interactions. Systems exhibiting such behavior are termed complex and can range from the molecular to field scale in size. Complex systems contain many interactive parts and display collective behavior including emergence, feedback, and adaptive mechanisms. Microorganisms - key moderators of subsurface chemical processes - further challenge system understanding and prediction because they are adaptive life forms existing in an environment difficult to observe and measure. A new scientific approach termed complex systems science has evolved from the critical need to understand and model these systems, whose distinguishing features increasingly are found to be common in the natural world. In contrast to reductionist approaches, complexity methods often use a top-down approach to identify key interactions controlling diagnostic variables at the prediction scale; general macroscopic laws controlling system-scale behavior; and essential, simplified models of subsystem interactions that enable prediction. This approach is analogous to systems biology, which emphasizes the tight coupling between

Complex Systems Science for Subsurface Fate and Transport Report from the August 2009 Workshop

International Nuclear Information System (INIS)