A Metabolic Study of Huntington's Disease.

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Rajasree Nambron

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

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

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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.

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...

GIS-Assisted Spatial Data Management for Corps of Engineers Real Estate Activities: Spatial Data Conversion Options

National Research Council Canada - National Science Library

Dove, Linda

2002-01-01

...; and scanning hardcopy maps and then using heads up digitizing or automated vectorization. These methods are discussed in this report. Four Corps District Offices have provided examples of their spatial data conversion methods.

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

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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?

Science.gov (United States)

Coon, Elizabeth A; Hassan, Anhar

2015-01-01

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

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...

Army Engineers in Memphis District: A Documentary Chronicle

Science.gov (United States)

1982-05-01

of levees in the St. Francis Levee District, stretching from just above Point Pleasant, Missouri, to Pecan Point, Arkansas. Meanwhile, the...troops in Trieste, Italy , 1948-1951 Commanding General, 9th Corps, Korea, 1951 Commanding General, 4th U.S. Army, 1952-1953 Commanding General

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

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

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

2012-04-01

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

Psychiatric symptoms and CAG expansion in Huntington`s disease

Energy Technology Data Exchange (ETDEWEB)

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

1996-02-16

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

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

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

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

2013-11-01

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

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

Science.gov (United States)

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

2018-01-01

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

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.

The use of olanzapine in Huntington disease accompanied by psychotic symptoms

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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

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

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

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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.

Clinical and genetic study of a juvenile-onset Huntington disease

Directory of Open Access Journals (Sweden)

HAO Ying

2012-06-01

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

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

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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

[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

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.

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.

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...

CORP

DEFF Research Database (Denmark)

Siebenmann, Christoph; Keiser, Stefanie; Robach, Paul

2017-01-01

In this Cores of Reproducibility in Physiology (CORP) article, we present the theory and practical aspects of the carbon monoxide (CO) rebreathing method for the determination of total hemoglobin mass in humans. With CO rebreathing, a small quantity of CO is diluted in O2 and rebreathed for a spe......In this Cores of Reproducibility in Physiology (CORP) article, we present the theory and practical aspects of the carbon monoxide (CO) rebreathing method for the determination of total hemoglobin mass in humans. With CO rebreathing, a small quantity of CO is diluted in O2 and rebreathed...

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...

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

Science.gov (United States)

2012-12-03

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

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...

[Sporadic juvenile forms of Huntington's chorea].

Science.gov (United States)

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

1980-01-01

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

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.

76 FR 42154 - BioMETRX, Inc., Biopure Corp. (n/k/a PBBPC, Inc.), Distributed Energy Systems Corp., Fortified...

Science.gov (United States)

2011-07-18

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] BioMETRX, Inc., Biopure Corp. (n/k/a PBBPC, Inc.), Distributed Energy Systems Corp., Fortified Holdings Corp., Knobias, Inc., and One IP Voice... securities of Distributed Energy Systems Corp. because it has not filed any periodic reports since the period...

45 CFR 2522.240 - What financial benefits do AmeriCorps participants serving in approved AmeriCorps positions receive?

Science.gov (United States)

2010-10-01

... financial benefits do AmeriCorps participants serving in approved AmeriCorps positions receive? (a) Ameri... 45 Public Welfare 4 2010-10-01 2010-10-01 false What financial benefits do AmeriCorps participants serving in approved AmeriCorps positions receive? 2522.240 Section 2522.240 Public Welfare Regulations...

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.

An innovative program to address learning barriers in small schools: Washington State School Nurse Corps.

Science.gov (United States)

Fast, Gail Ann; Gray, Lorali; Miles-Koehler, Mona

2013-01-01

While all schools in Washington State have had to deal with shrinking financial resources, small, rural school districts, with fewer than 2,000 students, face unique circumstances that further challenge their ability to meet rising student health needs. This article will explore how small districts utilize the services of the Washington State School Nurse Corps (SNC), an innovative program that supports student health and safety while reducing barriers to learning. Through direct registered nursing services and regional nurse administrative consultation and technical assistance, the SNC strengthens rural school districts' capacity to provide a safe and healthy learning environment. In addition, we will examine current research that links health and learning to discover how the SNC model is successful in addressing health risks as barriers to learning. Lastly, as resources continue to dwindle, partnerships between schools, the SNC, and state and local health and education organizations will be critical in maintaining health services and learning support to small, rural schools.

Le devenir actif du corps affectif

Directory of Open Access Journals (Sweden)

Pascal Séverac

2005-09-01

Full Text Available Le but de cet article est de saisir ce que signifie, pour le corps, être actif. À partir de la proposition 49 de la partie IV de l’Éthique, on propose de distinguer deux manières d’appréhender le corps – soit comme corps organique, soit comme corps affectif -, et l’on montre que la question éthique du devenir actif s’adresse à la dimension affective du corps. Il faut penser le devenir actif du corps affectif comme augmentation de son aptitude non seulement à affecter, mais aussi à être affecté. En effet, être affecté pour le corps n’est pas identique à pâtir : au contraire, plus est grande l’ouverture sensible d’un corps aux autres corps, plus est grande son activité éthique.The aim of this paper is to understand how the body can be active. With the proposition 49 of the fourth part of Ethics, two ways of conceiving of the body are distinguished : like an organic body or like an affective body. The ethics question of becoming active is about the affective body. This becoming active must be understood as increasing of the ability to affect, as well as to be affected. To be affected is different from to be passive. On the contrary, the more the body is able to be affected, the more he becomes active.

Le corps poétique ou la poétique du corps

Directory of Open Access Journals (Sweden)

Yvonne Goga

2007-05-01

Full Text Available Les réflexions sur la poésie de Valéry sont illustrées dans deux volumes : Album de vers anciens et Charmes. Parmi les images les plus représentatives est celle du corps de la femme. Par l’analyse des images sur le corps de la femme, le but de notre étude est celui de souligner l’un des principes de la poétique de Valéry : la relation entre le monde extérieur, le corps et l’esprit, pour créer le poème et le poète.

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.

Digital Corp(s. Identidad y ciberespacio

Directory of Open Access Journals (Sweden)

Verónica Perales Blanco

2012-04-01

Full Text Available El título de este artículo, la suma del término inglés digital con el francés corps (cuerpo digital es un guiño que hace referencia a la relación existente entre nuestra búsqueda identitaria en el ciberespacio y el derivado carácter económico de la misma. Corp es el término abreviado de corporation, procedente del latín corpus, se entiende como “cuerpo de gente” y se utiliza fundamentalmente para referirse a la estructura de gran parte de los negocios en Norteamérica y el mundo entero.Este artículo analiza -desde una perspectiva de género- algunas de las proyecciones identitarias actuales en internet con especial atención a las vinculadas a los espacios lúdicos.

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 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.

77 FR 4389 - In the Matter of Tornado Gold International Corp., Twin Faces East Entertainment Corp., Universal...

Science.gov (United States)

2012-01-27

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] In the Matter of Tornado Gold International Corp., Twin Faces East Entertainment Corp., Universal Ice Blast, Inc., US Farms, Inc., US Microbics... concerning the securities of Tornado Gold International Corp. because it has not filed any periodic reports...

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 ...

Nippon Steel Corp.: Carbon fiber seat business of Tonen Corp., formal bribery Nippon Steel Corp; Tonen no tanso seni shito jigyo. Shinnittetsu ga seishiki baishu

Energy Technology Data Exchange (ETDEWEB)

NONE

1998-02-28

Nippon Steel Corp. and Tonen Corp. announced that it signed the transfer contract of carbon fiber seat business on December 1, 1998. (Nittetsu composite) is established as a business company of carbon fiber compound material fitted to the existent business, and it makes use of the multiplier effect of the business integration, and the new day iron which purchased a business from Tonen Corp. works for the scale expansion. (translated by NEDO)

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

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...

Energy and Greenhouse Gas Emission Reduction Opportunities for Civil Works Projects Unique to the US Army Corps of Engineers

Science.gov (United States)

2012-10-26

petroleum gas ( LPG ) (4% of emissions). Figure 1 shows Tableau Software results for USACE MSCs GE GHG emissions. MVD, ERDC, and NAD (including Washington...ventilating, and air-conditioning LED light emitting diode LPG liquid petroleum gas LRC US Army Corps of Engineers — Chicago District MMBTU million...ER D C/ CE RL T R- 12 -1 9 Center for the Advancement of Sustainability Innovations (CASI) Energy and Greenhouse Gas Emission Reduction

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...

Human Capital. Corps of Engineers Needs to Update Its Workforce Planning Process to More Effectively Address Its Current and Future Workforce Needs

Science.gov (United States)

2008-05-01

Subcommittee on Oversight of Government Management, the Federal Workforce, and the District of Columbia Committee on Homeland Security and...allows flex-time, telecommuting , or alternative work schedules. Page 17 GAO-08-596 Corps of Engineers Table 1: Examples of Human Capital...programs and policies; and provides analyses, recommendations, and other assistance to help Congress make informed oversight , policy, and funding

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 ...

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.

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.

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.

Bibliographie sélective sur le corps

Directory of Open Access Journals (Sweden)

Anne-Claire Rebreyend

2007-11-01

Full Text Available Ouvrages générauxBruit Zaidman Louise, Houbre Gabrielle, Klapisch-Zuber Christiane, Schmitt Pantel Pauline (dir., Le corps des jeunes filles de l'Antiquité à nos jours, Paris, Perrin, 2001.Vigarello Georges, Le propre et le sale. L'hygiène du corps depuis le Moyen Âge, Paris, Seuil, 1985.—, Le sain et le malsain : santé et mieux-être depuis le Moyen Âge, Paris, Seuil, 1993.—, Histoire de la beauté. Le corps et l'art d'embellir de la Renaissance à nos jours, Paris, Seuil, 2004.Le corps au Moy...

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.

78 FR 56263 - HydroGen Corp., QueryObject Systems Corp., Security Intelligence Technologies, Inc., Skins, Inc...

Science.gov (United States)

2013-09-12

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] HydroGen Corp., QueryObject Systems Corp., Security Intelligence Technologies, Inc., Skins, Inc., SLM Holdings, Inc., Spring Creek Healthcare Systems... securities of Security Intelligence Technologies, Inc. because it has not filed any periodic reports since...

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

Le corps dans l'Antiquité

Directory of Open Access Journals (Sweden)

Sophie Lalanne

2008-06-01

Full Text Available Ouvrages générauxLaqueur Thomas, La fabrique du sexe. Essai sur le corps et le genre en Occident, trad. fr., Paris, Gallimard, 1992.Feher Michel, Naddaff Ramona, Tazi Nadia, Fragments for a History of the Human Body, 3 volumes, New York, Zone Books, 1989.Le Corps dans l’AntiquitéBodiou Lydie, Frère Dominique, Mehl Véronique dir., L’expression des corps. Gestes, attitudes, regards dans l’iconographie antique, Rennes, Presses Universitaires de Rennes, 2006.Bonnard Jean-Baptiste, Le complexe de ...

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

78 FR 31997 - Greatmat Technology Corp., Kentucky USA Energy, Inc., Solar Energy Ltd., and Visiphor Corp...

Science.gov (United States)

2013-05-28

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] Greatmat Technology Corp., Kentucky USA Energy, Inc., Solar Energy Ltd., and Visiphor Corp., Order of Suspension of Trading May 23, 2013. It appears... concerning the securities of Solar Energy Ltd. because it has not filed any periodic reports since the period...

20 CFR 638.541 - Job Corps training opportunities.

Science.gov (United States)

2010-04-01

... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false Job Corps training opportunities. 638.541 Section 638.541 Employees' Benefits EMPLOYMENT AND TRAINING ADMINISTRATION, DEPARTMENT OF LABOR JOB CORPS PROGRAM UNDER TITLE IV-B OF THE JOB TRAINING PARTNERSHIP ACT Center Operations § 638.541 Job Corps...

A Guideline for Marine Corps Financial Managers

National Research Council Canada - National Science Library

Wright, Anthone

1998-01-01

...), and Marine Corps orders, publications and directives to determine those keys areas considered most essential to Marine Corps financial management specialists in the performance of their duties...

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.

Marine Corps Budgetary Reprogramming Effectiveness

Science.gov (United States)

2015-03-01

infrastructure (Appropriations Act of Congress, 2008). The environmental restoration is a transfer account controlled by the DOD. Usually in the case of...at an average just over 11 percent and the Marine Corps encircle the backend of the DOD portion of reprogramming with the Marine Corps reprogramming...blue force tracker (BFT), radio systems, high mobility multipurpose wheeled vehicle (HMMWV), medium tactical vehicle replacement (MTVR), and

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.

Engaged Learning and Peace Corps Service in Tanzania: An Autoethnography

Science.gov (United States)

Darling, Brianna; Thorp, Laurie; Chung, Kimberly

2014-01-01

The Peace Corps Masters International program offers students the opportunity to combine their Peace Corps service with their master's education. This article demonstrates how classroom learning strengthened the author's Peace Corps service in Tanzania, which in turn strengthened her master's thesis. Peace Corps supports an approach to community…

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....

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

CULTURAL FRONTIERS OF THE PEACE CORPS.

Science.gov (United States)

TEXTOR, ROBERT B., ED.

THE PRESENT WORK CONTAINS ANALYSES OF PEACE CORPS PROGRAMS IN SELECTED HOST COUNTRIES (THE PHILIPPINES, MALAYA, THAILAND, PERU, BOLIVIA, AFGHANISTAN, SOMALIA, NIGERIA, TANGANYIKA, SIERRA LEONE, TUNISIA, MOROCCO, AND JAMAICA) WRITTEN BY AN AUTHORITY ON EACH COUNTRY. COVERAGE OF DOMESTIC OPERATIONS OF THE PEACE CORPS IS STRICTLY LIMITED TO WHAT IS…

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

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.

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...

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...

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.

A History of the Little Rock District U. S. Army Corps of Engineers

Science.gov (United States)

1971-01-01

1970 there were 300 such establishments with overnight accommodations for 8,339 people. In 1940 there were only seven restaurants, cafes , and public...Barry County is included in the franchise release re- quests." 54 t District’s Purple Martin program first received national pub- licity at...benefit of their own citizens. The Engineers also expected communities to benefit from franchises to local concessionaires. The plan did not work

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

77 FR 32986 - Notice of Inventory Completion: Marine Corps Base Camp Pendleton, U.S. Marine Corps, San Diego...

Science.gov (United States)

2012-06-04

... Inventory Completion: Marine Corps Base Camp Pendleton, U.S. Marine Corps, San Diego County, CA AGENCY..., institution, or Federal agency that has control of the Native American human remains. The National Park... and present archeological theory place the Luiseno tribes within this geographic area of San Diego...

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.

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

Foreign Language Training in the United States Peace Corps.

Science.gov (United States)

Kulakow, Allan

This document reports on the foreign language training offered in the Peace Corps. Following a brief introductory statement, a list of languages taught by the Peace Corps in the years 1961-67 is provided, as well as a brief description of Peace Corps language training methods. Guidelines for language coordinators are outlined, and the approach to…

77 FR 23669 - Public Hearing and Notice of Availability for the Draft Environmental Impact Statement for the...

Science.gov (United States)

2012-04-20

... (JCPA) submitted an application to the U.S. Army Corps of Engineers (Corps), Mobile District... 2288, Mobile, Alabama 36628-0001, via email at [email protected] or by phone at (251) 690... application to the U.S. Army Corps of Engineers (Corps), Mobile District, Mississippi Department of...

78 FR 59409 - In the Matter of AcuNetx, Inc., Alliance Pharmaceutical Corp., BBV Vietnam SE.A. Acquisition Corp...

Science.gov (United States)

2013-09-26

... Pharmaceutical Corp., BBV Vietnam SE.A. Acquisition Corp., Cash Technologies, Inc., Conspiracy Entertainment... that there is a lack of current and accurate information concerning the securities of Cash Technologies... concerning the securities of Conspiracy Entertainment Holdings, Inc. because it has not filed any periodic...

Leader development transformation in the Army Nurse Corps.

Science.gov (United States)

Funari, Tamara S; Ford, Kathleen; Schoneboom, Bruce A

2011-01-01

The Army Nurse (AN) Corps is undergoing a historic transformation. Under the leadership of its Chief, MG Patricia Horoho, the Corps developed and implemented the AN Campaign Plan to insure that the Corps has the right capability and capacity to meet the current and future needs of the US Army. This article describes the work conducted by the AN Corps Leadership Imperative Action Team (Leader IAT) to develop full-spectrum leaders for the future. The mission of the Leader IAT is derived from both the AN Campaign plan as well as the operational objectives defined in the AN balanced scorecard. As a result of the analysis conducted during preparation of the AN Campaign Plan, several key gaps were identified regarding the Army Nurse Corps' ability to match leadership talents with the diverse demands of current missions, as well as its adaptability and flexibility to be prepared for unknown future missions. This article also introduces the Leadership Capability Map and other initiatives implemented to ensure the development of full-spectrum leaders who will be effective in the future military healthcare environment.

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.

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.

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

Service - Tradition - Change: A History of the Fort Worth District, U.S. Army Corps of Engineers 1975-1999

Science.gov (United States)

2000-01-01

remembered, "and the environmentalists would get up and play guitar and do folk songs, ’The Corps of Engineers March,’ and things like that." At that...requirements was very important."s ’$ officers. According to Rogers, in 1998 "we received our highest scores on questions related to making the customer...a part of the team and involving them in our work for them." These scores reflected "the " E results of good customer care. ven so, Rogers was

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

An Analysis of the Populations of the Air Force's Medical and Professional Officer Corps.

Science.gov (United States)

Keating, Edward G; Massey, Hugh G; Mele, Judith D; Mundell, Benjamin F

2012-01-01

Since the advent of the all-volunteer force, one of the foremost personnel challenges of the U.S. Air Force has been recruiting and retaining an adequate number of medical and professional officers in the Air Force's seven medical and professional officer corps: the Biomedical Sciences Corps (BSC), the Chaplain Corps, the Dental Corps, the Judge Advocate General (JAG) Corps (attorneys), the Medical Corps (physicians), the Medical Service Corps (MSC), and the Nurse Corps. For each of these corps, there are highly similar jobs in the private sector, so attracting and retaining these corps' officers is a constant challenge. This article analyzes all seven Air Force medical and professional officer corps and their relative statuses with regard to end strengths, accession levels, promotion flow, and attrition since the late 1970s. The authors find that recent accession and retention trends have been most adverse in the Air Force's Nurse Corps, while the MSC and the JAG Corps appear to have the most stable populations.

Prairies Water Management on Corps Lands

Science.gov (United States)

2009-02-01

infiltration, autogenic mechanisms can lead to the recovery of essential soil processes. The Role of Organic Matter in Soil Formation. In a prairie...management in EP-1130-2-540 (USACE 2005), and does not have a fire management training program in place (USACE 2008). Some Corps resource managers...are trained and partner with other entities to conduct prescribed burns on Corps grasslands. However, prescribed burning as a management strategy is

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.

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...

Why does the Job Corps increase gender earnings inequality?

OpenAIRE

Strittmatter, Anthony

2016-01-01

Several studies considering the Job Corps find more positive earnings effects for males than for females. This effect heterogeneity in favor of males contrasts with the results of the majority of other training program evaluations. Applying the translated quantile approach of Bitler, Hoynes, and Domina (2014), I show that an important mechanism behind the surprising findings for the Job Corps operates through existing gender earnings inequality rather than Job Corps trainability differences b...

77 FR 3836 - Supplemental Environmental Impact Statement, Mingo County, WV

Science.gov (United States)

2012-01-25

... Individual Permit (IP) application submitted by Consol of Kentucky, Inc. (CONSOL), for the discharge of fill...; email: [email protected] . Ginger Mullins, Chief, Regulatory Branch, U.S. Army Corps of Engineers, 502 Eighth Street, Huntington, WV 25701-2070; telephone: (304) 399- 5610; email: [email protected

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

Corps sans organes et anamnèse

DEFF Research Database (Denmark)

Wilson, Alexander

2011-01-01

Je trace certains liens entre le corps sans organes de Deleuze et Guattari et les principes de l’organologie générale que décrit Bernard Stiegler.......Je trace certains liens entre le corps sans organes de Deleuze et Guattari et les principes de l’organologie générale que décrit Bernard Stiegler....

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,...

Steward of Headwaters: U.S. Army Corps of Engineers, St. Paul District, 1975-2000

Science.gov (United States)

2012-01-01

to their shells, thereby preventing them from migrating or burrowing .73 The St. Paul District initiated a monitoring process of the zebra mussel at...P ro je ct s 4 104 Strong Move, 13 February 1997, File LaFarge Ho Chunk, Box 6410, SPDAR; Ron Wilber to Larry Garvin, HoChunk Researcher, 10...July 1987, File Congressional Correspondence, Box 6412, SPDAR. 39 Larry and Diane Uhlir to Mr. Gregg Struss, Resource Manager, Gull Lake Recreation

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

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,

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

76 FR 20992 - Sun Chemical Corp.; Filing of Color Additive Petition

Science.gov (United States)

2011-04-14

...] Sun Chemical Corp.; Filing of Color Additive Petition AGENCY: Food and Drug Administration, HHS. ACTION: Notice. SUMMARY: The Food and Drug Administration (FDA) is announcing that Sun Chemical Corp. has... given that a color additive petition (CAP 1C0290) has been filed by Sun Chemical Corp., 5020 Spring...

Marine Corps Private Cloud Computing Environment Strategy

Science.gov (United States)

2012-05-15

leveraging economies of scale through the MCEITS PCCE, the Marine Corps will measure consumed IT resources more effectively, increase or decrease...flexible broad network access, resource pooling, elastic provisioning and measured services. By leveraging economies of scale the Marine Corps will be able...IaaS SaaS / IaaS 1 1 LCE I ACE Dets I I I I ------------------~ GIG / CJ Internet Security Boundary MCEN I DISN r :------------------ MCEN

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.

78 FR 32250 - CDM Smith and Dynamac Corp; Transfer of Data

Science.gov (United States)

2013-05-29

... ENVIRONMENTAL PROTECTION AGENCY [EPA-HQ-OPP-2013-0036; FRL-9387-5] CDM Smith and Dynamac Corp... the submitter, will be transferred to CDM Smith and its subcontractor, Dynamac Corp, in accordance with 40 CFR 2.307(h)(3) and 2.308(i)(2). CDM Smith and its subcontractor, Dynamac Corp, have been...

AmeriCorps VISTA: Getting Things Done for America

Science.gov (United States)

Corporation for National and Community Service, 2017

2017-01-01

AmeriCorps VISTA (Volunteers in Service to America) engages more than 8,000 individuals annually to support community efforts to overcome poverty. AmeriCorps VISTA members serve full time for a year at nonprofit organizations or local government agencies to build the capacity of these organizations to carry out programs that alleviate poverty.…

Report of Marine Corps Internal Controls over Military Equipment Funds

National Research Council Canada - National Science Library

Granetto, Paul J; Marsh, Patricia A; Sauls, Barbara A; Carey, Alice F; Negash, Lidet K; Pray, Davita N; Tsay, Shirlenne S; Kleiman-Redden, Ellen

2007-01-01

.... The Marine Corps Systems Command mission is to serve as the Marine Corps Commandant's principal agent for acquisition and sustainment of systems and equipment used to accomplish warfighting missions...

[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.

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

A Comparison of Local Search Methods for the Multicriteria Police Districting Problem on Graph

Directory of Open Access Journals (Sweden)

F. Liberatore

2016-01-01

Full Text Available In the current economic climate, law enforcement agencies are facing resource shortages. The effective and efficient use of scarce resources is therefore of the utmost importance to provide a high standard public safety service. Optimization models specifically tailored to the necessity of police agencies can help to ameliorate their use. The Multicriteria Police Districting Problem (MC-PDP on a graph concerns the definition of sound patrolling sectors in a police district. The objective of this problem is to partition a graph into convex and continuous subsets, while ensuring efficiency and workload balance among the subsets. The model was originally formulated in collaboration with the Spanish National Police Corps. We propose for its solution three local search algorithms: a Simple Hill Climbing, a Steepest Descent Hill Climbing, and a Tabu Search. To improve their diversification capabilities, all the algorithms implement a multistart procedure, initialized by randomized greedy solutions. The algorithms are empirically tested on a case study on the Central District of Madrid. Our experiments show that the solutions identified by the novel Tabu Search outperform the other algorithms. Finally, research guidelines for future developments on the MC-PDP are given.

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)

The Marine Corps Challenges in Creating a Diverse Force

Science.gov (United States)

2013-03-22

demographics, young adult’s perception of the Marines, etc.  The majority of Marine Corps Officer Selection Offices ( OSO ) has a majority of Caucasian...officers in senior the billets at the OSO . This makes potential candidates view of the Marine Corps unappealing because they feel as if leadership

A History of the Little Rock District U.S. Army Corps of Engineers 1881-1979

Science.gov (United States)

1979-01-01

establishments with overnight accommodations for 8,339 people. In 1940 there were only seven restaurants, cafes , and public dining rooms in the vicinity...that only one out of five docks located in Barry County is included in the franchise release re- quests." 54 • District’s Purple Martin program...benefit of their own citizens. The Engineers also expected communities to benefit from franchises to local concessionaires. The plan did not work out

20 CFR 638.502 - Job Corps basic education program.

Science.gov (United States)

2010-04-01

... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false Job Corps basic education program. 638.502... education program. The Job Corps Director shall prescribe or provide for basic education curricula to be... coordination with regional offices, shall review and approve the basic education program at each center. Center...

River Engineers on the Middle Mississippi: A History of the St. Louis District, U.S. Army Corps of Engineers

Science.gov (United States)

1978-01-01

34, ... r... Ut! A I _~ 4"’’’ ..... J Ih::".r~ ........ fl".. ... / ;)""’U’ ~’’’~U’[1 ŕ,,,. ,, iJ~~’;’m~’---z J( ufo , CiVP>4 or Crfg.,. ... ~.t IX.J...unusual projects undertaken by the District were the Alien Enemy Internment Camp at Weingarten, Missouri, and the LCT Project at the District service...base. The Weingarten camp was de- signed to hold aliens who were considered a potential threat to national security. Obviously it required the same

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....

Peace Corps Aquaculture Training Manual. Training Manual T0057.

Science.gov (United States)

Peace Corps, Washington, DC. Information Collection and Exchange Div.

This Peace Corps training manual was developed from two existing manuals to provide a comprehensive training program in fish production for Peace Corps volunteers. The manual encompasses the essential elements of the University of Oklahoma program that has been training volunteers in aquaculture for 25 years. The 22 chapters of the manual are…

AmeriCorps: Getting Things Done for America. Fact Sheet

Science.gov (United States)

Corporation for National and Community Service, 2017

2017-01-01

AmeriCorps engages more than 80,000 men and women in intensive service each year at more than 21,000 locations including nonprofits, schools, public agencies, and community and faith-based groups across the country. AmeriCorps members help communities tackle pressing problems while mobilizing millions of volunteers for the organizations they…

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...

Cultivating Political Powerhouses: TFA Corps Members Experiences That Shape Local Political Engagement

Science.gov (United States)

Jacobsen, Rebecca; White, Rachel; Reckhow, Sarah

2016-01-01

In recent years, Teach for America (TFA) has invested in developing corps members as leaders. Although TFA asks corps members for a two-year commitment, TFA celebrates the achievements of alumni who have gone on to careers in politics, public policy, and advocacy. Thus, many community leaders see the arrival of TFA corps members as having a…

77 FR 2567 - Proposed Collection of Information for the Job Corps Process Study; New Collection

Science.gov (United States)

2012-01-18

...: Job Corps center management and staff; Job Corps students; Job Corps partner organizations' staff; and... Human Resources Managers 8 Academic Instruction Managers 24 Career Technical Training (CTT) Managers 24... the Job Corps Process Study; New Collection AGENCY: Employment and Training Administration (ETA...

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.

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

Science.gov (United States)

2010-03-12

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

78 FR 67198 - Agency Information Collection Activities; Submission for OMB Review; Comment Request; Job Corps...

Science.gov (United States)

2013-11-08

... for OMB Review; Comment Request; Job Corps Application Data ACTION: Notice. SUMMARY: The Department of... collection request (ICR) titled, ``Job Corps Application Data,'' to the Office of Management and Budget (OMB... Job Corps application data collected on three forms (ETA-652, Job Corps Data Sheet; ETA-655, Statement...

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...

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.

Responsiveness and Reliability: A History of the Philadelphia District and the Marine Design Center, U.S. Army Corps of Engineers, 1972-2008

Science.gov (United States)

2012-01-01

levels and the impacts of salinity variation on the fish and wildlife resources.” According to the Corps, the report fulfilled the congressional...57, 65-66. 29 Elliot Goldberg , “Sites Sought for Dumping River Spoils,” The Gloucester County Times (Woodbury, N.J.), 22 February 1980, copy in...construction contract for this project in June 2003, and work commenced soon after. Specifics of the dam removal involved construction of a tempo - rary

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.

20 CFR 670.505 - What types of training must Job Corps centers provide?

Science.gov (United States)

2010-04-01

... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false What types of training must Job Corps centers... Operations § 670.505 What types of training must Job Corps centers provide? (a) Job Corps centers must... necessary for those jobs, and as appropriate, recommend changes in the center's vocational training program...

Corps et esprit : l’identité humaine selon Spinoza

Directory of Open Access Journals (Sweden)

Lamine Hamlaoui

2005-09-01

Full Text Available Contrairement à Descartes, Spinoza refuse à l’esprit humain et par conséquent à l’homme le statut de substance : l’homme est défini comme l’union de deux modes, un corps et une âme. On ne peut donc plus comme chez Descartes distinguer une identité substantielle, conférée au corps par l’âme, et une identité modale, déterminée par le rapport du corps humain aux autres corps. Ces deux identités sont fondues dans une identité essentielle. L’objet de cet article est de mettre en évidence le statut problématique de cette identité dans l’Éthique. L’esprit humain y est en effet déduit et défini comme l’idée du corps humain, c’est-à-dire le concept que Dieu forme du corps humain. Mais tantôt Spinoza identifie cette idée du corps humain à l’essence de l’esprit humain, tantôt il établit une distinction entre les deux. De même, tantôt en vertu du parallélisme des attributs il identifie l’idée du corps humain à l’idée de l’esprit humain, tantôt il distingue les deux. D’où des tensions qui travaillent le système de l’intérieur.Contrary to Descartes, Spinoza refuses to human mind and therefore to man the status of substance : man is defined as the union of two modes, body and mind. We can’t distinguish, as in Descartes, a substantial identity, conferred to the body by the mind, and a modal identity, determined by the relation between human body and other bodies. Both identities form an essential identity. This paper explains the problematical character of this identity in Spinoza’s Ethic. Human mind is indeed deduced and defined as the idea of human body, namely the concept of human body formed by God. But sometimes Spinoza identifies the idea of human body with the essence of human body, sometimes he makes a distinction between both. In the same way, sometimes he identifies the idea of human body with the idea of human mind, in accordance with parallelism of attributes, sometimes he

Problems and Counselling Needs of Corps Members in Nigeria (Pp ...

African Journals Online (AJOL)

Nekky Umera

1973-05-22

May 22, 1973 ... major problems and counseling needs of Corps Members were psychological in nature. ... and must be resolved sufficiently at a given stage if the individual is to cope successfully with ... (ii) Is there any difference between the problems of male and female ... youth corps member of different age group?

A Text Analysis of the Marine Corps Fitness Report

Science.gov (United States)

2017-06-01

43 3. Support Vector Machine .............................................................44 4. Boosting ...demonstrated below: Original text “#1 captain in the battalion. MRO is one of the most talented and gifted minds we have in the Marine Corps. He is a...officer, place in billets where the Corps needs are best and brightest. Finally, there is no doubt in my mind , this officer’s will and should be

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...

76 FR 6839 - ActiveCore Technologies, Inc., Battery Technologies, Inc., China Media1 Corp., Dura Products...

Science.gov (United States)

2011-02-08

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] ActiveCore Technologies, Inc., Battery Technologies, Inc., China Media1 Corp., Dura Products International, Inc. (n/k/a Dexx Corp.), Global Mainframe Corp., GrandeTel Technologies, Inc., Magna Entertainment Corp. (n/k/a Reorganized Magna Entertainment...

Corps rebelles

OpenAIRE

Salle, Muriel

2010-01-01

Corps rebelles. Les tatouages de la collection Lacassagne (1874-1924). À la fin du XIXe siècle, le Docteur Alexandre Lacassagne est médecin militaire en poste en Algérie, auprès des bataillons d’Afrique, ces compagnies disciplinaires où sont envoyés les soldats indisciplinés, aux confins de l’empire colonial français. Dans cette relégation à « régime spécial » où les conditions d’existence sont infernales, le jeune médecin entreprend de relever les marques corporelles dont s’ornent les soldat...

Solar installer training: Home Builders Institute Job Corps

Energy Technology Data Exchange (ETDEWEB)

Hansen, K.; Mann, R. [San Diego Job Corps Center, Imperial Beach, CA (United States). Home Builders Inst.

1996-10-01

The instructors describe the solar installation training program operated since 1979 by the Home Builders Institute, the Educational Arm of the National Association of Home Builders for the US Department of Labor, Job Corps in San Diego, CA. The authors are the original instructors and have developed the program since its inception by a co-operative effort between the Solar Energy Industries Association, NAHB and US DOL. Case studies of a few of the 605 students who have gone to work over the years after the training are included. It is one of the most successful programs under the elaborate Student Performance Monitoring Information System used by all Job Corps programs. Job Corps is a federally funded residential job training program for low income persons 16--24 years of age. Discussion details the curriculum and methods used in the program including classroom, shop and community service projects. Solar technologies including all types of hot water heating, swimming pool and spa as well as photovoltaics are included.

The Marine Corps Schools: Driving Institutional Change Towards the Second World War

Science.gov (United States)

2014-05-22

Graduate Degree Programs Robert F. Baumann, Ph.D. The opinions and conclusions expressed herein are those of the student author, and do not...Brigadier General Smedley Butler believed an independent Marine Corps with little or no ties to the Navy was the future of the Corps. To...Colonel USMC. “The Sea School.” The Marine Corps Gazette 10 no. 2 (September 1925): 103-109. Dunlap, Robert H., Colonel USMC. “Lessons For Marines

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

Future Retention of the Marine Corps Top Performing Aviators

Science.gov (United States)

2016-06-10

balance, and Aviator Continuation Pay. 15. SUBJECT TERMS USMC Aviator Retention, USMC Aviation Manpower, Marine Corps Talent Management , Marine Corps...Wing Aircraft: Includes AH-1, UH-1, and CH-53 platforms. Talent Management : The process through which employers anticipate and meet their needs for...a bias due to his experience as an executive officer responsible for the talent management of company grade WTI aviators. All attempts have been

Application of a System-Based Inventory Model to Marine Corps Repairable Parts

National Research Council Canada - National Science Library

Barnett, Craig

2001-01-01

... isolation of the inventories and because of mathematical flaws in the Marine Corps' sparing methodology The Marine Corps is seeking to centralize the management of secondary repairables and is considering...

The Peace Corps and Higher Education: Finally the Envisioned Partnership?

Science.gov (United States)

Quigley, Kevin F. F.

2013-01-01

A number of structural and contextual changes underway suggests that now that the Peace Corps has begun its second half-century, it may be the opportune time for a broader and deeper strategic partnership with higher education along the lines that the Peace Corps founders' envisioned. That partnership would involve higher education playing an…

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.

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.

Study of a Federal Nuclear Operations Corps

International Nuclear Information System (INIS)

1981-12-01

The staffing requirements of the nuclear electric utility industry will create very large demands in the next 3 years for nuclear trained personnel in operation and maintenance. The commercial nuclear industry recognizes these needs, the importance of having qualified trained personnel, and is taking appropriate action to satisfy these requirements. The education and training capabilities both inside and outside of the utility industry have the capacity to meet these demands if they are properly managed. In particular, the Institute of Nuclear Power Operations Human Resource Management System provides a framework for an organized industry-wide program. The Nuclear Regulatory Commission's Office of Inspection and Enforcement is in effect a Federal Corps focused on nuclear operations. Its Reactor Training Center trains the professionals required to inspect nuclear operations and enforce regulations. Therefore, the Department of Energy (DOE) believes that the objectives of a Federal Nuclear Operations Corps can be met by existing private and Federal efforts and that there is no need to establish another corps or training academy. This is consistent with the Administration's policy of minimizing the Federal role in areas where the private sector is capable

Elevation - LiDAR Survey - Rainy River, MN and Canada (Border Water)

Data.gov (United States)

Army Corps of Engineers, Department of the Army, Department of Defense — In November of 2006, Sanborn was contracted by the U.S. Army Corps of Engineers, St. Louis District on behalf of the St. Paul District to execute a LIDAR (Light...

77 FR 28373 - Liberty Energy (Midstates) Corp.; Notice of Baseline Filing

Science.gov (United States)

2012-05-14

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. PR12-24-000] Liberty Energy (Midstates) Corp.; Notice of Baseline Filing Take notice that on April 30, 2012, Liberty Energy (Midstates) Corp. submitted a baseline filing of their Statement of Operating Conditions for services provided...

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.

Competitive Sourcing in the Marine Corps: Friend or Foe?

Science.gov (United States)

2006-02-07

Report, March 2004 (Washington, D.C.), 82. 8. Mr. P. R. Stenner , “Military – Civilian Conversions,” 21 October 2004, https://www.manpower.usmc.mil...14 December 2005). 9. Stenner , 2. 10. United States Marine Corps, MARADMIN 058/05: Military- Civilian FY06 Conversions, 9 February 2005...Lieutenant General, USMC. DC I&L. “Excellence in Warfighting Support,” Marine Corps Gazette, August 2004, 30-31. Stenner , P. R. “Military – Civilian

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...

75 FR 74088 - Agency Information Collection Activities; Submission for OMB Review; Comment Request; Job Corps...

Science.gov (United States)

2010-11-30

... for OMB Review; Comment Request; Job Corps Enrollee Allotment Determination ACTION: Notice. SUMMARY... Administration (ETA) sponsored information collection request (ICR) titled, ``Job Corps Enrollee Allotment... number) or by e-mail at [email protected] . SUPPLEMENTARY INFORMATION: Job Corps enrollees may elect...

Optimizing Marine Corps Personnel Assignments Using an Integer Programming Model

Science.gov (United States)

2012-12-01

Corps. vi THIS PAGE INTENTIONALLY LEFT BLANK vii TABLE OF CONTENTS I. INTRODUCTION ...throughout our careers. xvi THIS PAGE INTENTIONALLY LEFT BLANK 1 I. INTRODUCTION The Marine Corps Manpower and Reserve Affairs (M&RA) office has the...2012 BAH Rates-with Dependents. Defense Travel Mangement Office. (2011, December). 2012 BAH Rates-without Dependents. M ileage C ost 1 Per D iem

20 CFR 670.975 - How is the performance of the Job Corps program assessed?

Science.gov (United States)

2010-04-01

... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false How is the performance of the Job Corps..., DEPARTMENT OF LABOR THE JOB CORPS UNDER TITLE I OF THE WORKFORCE INVESTMENT ACT Administrative and Management Provisions § 670.975 How is the performance of the Job Corps program assessed? The performance of the Job...

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.

75 FR 72841 - Agency Information Collection Activities; Submission for OMB Review; Comment Request; Job Corps...

Science.gov (United States)

2010-11-26

... for OMB Review; Comment Request; Job Corps Health Questionnaire ACTION: Notice. SUMMARY: The...) sponsored information collection request (ICR) titled, ``Job Corps Health Questionnaire,'' to the Office of... an applicant to the Job Corps. The information is obtained from the applicant during an interview...

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...

Can AmeriCorps Build Communities?

Science.gov (United States)

Thomson, Ann Marie; Perry, James L.

1998-01-01

An examination of AmeriCorps in five communities focused on its impact on the capacity of local community-based organizations to achieve community goals. Most partner organizations were unable to build sufficient commitment for self-governance; few programs were designed to encourage collective goals. Getting things done in local communities may…

Barossa Night: cohesion in the British Army officer corps.

Science.gov (United States)

Bury, Patrick

2017-06-01

Contrasting the classical explanation of military group cohesion as sustained by interpersonal bonds, recent scholars have highlighted the importance of ritualized communication, training and drills in explaining effective military performance in professional armies. While this has offered a welcome addition to the cohesion literature and a novel micro-sociological method of examining cohesion, its primary evidential base has been combat groups. Indeed, despite their prominent role in directing operations over the past decade, the British Army's officer corps has received relatively little attention from sociologists during this period. No attempt has been made to explain cohesion in the officer corps. Using a similar method to recent cohesion scholars, this paper seeks to address this imbalance by undertaking a micro-sociology of one ritual in particular: 'Barossa Night' in the Royal Irish Regiment. Firstly, it draws on the work of Durkheim to examine how cohesion amongst the officer corps is created and sustained through a dense array of practises during formal social rituals. It provides evidence that the use of rituals highlights that social solidarity is central to understanding officer cohesion. Secondly, following Hockey's work on how private soldiers negotiate order, the paper shows how this solidarity in the officer corps is based on a degree of negotiated order and the need to release organizational tensions inherent in a strictly hierarchical rank structure. It highlights how the awarding of gallantry medals can threaten this negotiated order and fuel deviancy. In examining this behaviour, the paper shows that even amongst an officer class traditionally viewed as the elite upholders of organizational discipline, the negotiation of rank and hierarchy can be fluid. How deviant behaviour is later accepted and normalized by senior officers indicates that negotiated order is as important to understanding cohesion in the British Army's officer corps as it is

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.

75 FR 74711 - Planet Energy (Pennsylvania) Corp.; Supplemental Notice That Initial Market-Based Rate Filing...

Science.gov (United States)

2010-12-01

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ER11-2167-000] Planet Energy (Pennsylvania) Corp.; Supplemental Notice That Initial Market-Based Rate Filing Includes Request for Blanket... proceeding, of Planet Energy (Pennsylvania) Corp.'s application for market-based rate authority, with an...

75 FR 74712 - Planet Energy (Maryland) Corp.; Supplemental Notice That Initial Market-Based Rate Filing...

Science.gov (United States)

2010-12-01

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ER11-2168-000] Planet Energy (Maryland) Corp.; Supplemental Notice That Initial Market-Based Rate Filing Includes Request for Blanket... proceeding, of Planet Energy (Maryland) Corp.'s application for market-based rate authority, with an...

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

Analysis of Marine Corps renewable energy planning to meet installation energy security requirements

OpenAIRE

Chisom, Christopher M.; Templenton, Jack C., II

2013-01-01

Approved for public release; distribution is unlimited. The purpose of this thesis is to analyze Marine Corps installation energy consumption and the pursuit of increased renewable energy generation goals across Marine Corps installations. The main objective of this report is to determine the cost of interruption and the net present value (NPV) of renewable energy generation needed to meet the Marine Corps energy security objectives. First, we determine installation-specific energy consump...

Le corps dans l’espace architectural. Le Corbusier, Claude Parent et Henri Gaudin

Directory of Open Access Journals (Sweden)

Julie Cattant

2016-12-01

Full Text Available Le premier geste de l’espace architectural est d’accueillir nos corps humains. En leur offrant un espacement à habiter, il leur permet de s’abriter, de se mouvoir et de se rencontrer. L’homme et l’architecture s’affectent l’un l’autre. Nombreux sont les architectes qui interrogent cette coexistence car, renouveler la pensée de l’espace nécessite de reconsidérer le corps humain. L’étude des relations entre le corps et l’espace chez trois architectes français sera l’occasion d’évoquer les écarts et les convergences entre l’homme et l’architecture. L’œuvre de Le Corbusier, protagoniste du mouvement moderne, maintient ainsi une distance entre un corps machinisé et un espace idéal. Marquée par un mode de pensée dualiste, l’architecture de Le Corbusier annonce toutefois son propre dépassement, dépassement que Claude Parent et Henri Gaudin opèreront par la suite à leur manière, le premier en introduisant l’oblique et la déstabilisation du corps, le second en transgressant la limite de l’espace et du corps pour envisager leur porosité réciproque. Finalement, c’est la manière dont l’homme appréhende l’espace avec son corps qui est questionnée par ces trois architectes, de la prégnance du visuel à son évincement.

Corps G-2 Staff Competencies: A Desert Storm Case Study

Science.gov (United States)

2017-06-09

Department of the Army, Army Doctrine Reference Publication (ADRP) 2-0, Intelligence (Washington, DC: Government Printing Office, August 2012), 3-2...Washington, DC: Government Printing Office, August 2012), 5-9. Intelligence Operations The second key corps G-2 intelligence meta-competency...Publication (ADRP) 2-0, Intelligence (Washington, DC: Government Printing Office, 2016), 4-2 to 4-9. Intelligence Analysis The final corps G-2

Maryland Cleaning & Abatement Services Corp. Information Sheet

Science.gov (United States)

Maryland Cleaning & Abatement Services Corp. (the Company) is located in Baltimore, Maryland. The settlement involves renovation activities conducted at property constructed prior to 1978, located in Baltimore, Maryland.

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...

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

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

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

The Corps Engineer Battalion in Contingency Operations

National Research Council Canada - National Science Library

Raymer, James

2001-01-01

.... The central research question asks: Is the proposed echelons above division engineer battalion design a better one for active and reserve component corps engineer forces to respond in a contingency...

77 FR 35059 - Agency Information Collection Activities; Submission for OMB Review; Comment Request; Job Corps...

Science.gov (United States)

2012-06-12

... for OMB Review; Comment Request; Job Corps Process Study ACTION: Notice. SUMMARY: The Department of... collection request (ICR) proposal titled, ``Job Corps Process Study,'' to the Office of Management and Budget... conduct site visits to Job Corps centers and a survey of center directors for a process study of the Job...

75 FR 71744 - Fifth Street Finance Corp., et al.; Notice of Application

Science.gov (United States)

2010-11-24

... Street Finance Corp., et al.; Notice of Application November 18, 2010. AGENCY: Securities and Exchange... Street Finance Corp. (``Fifth Street''), Fifth Street Management LLC, Fifth Street Mezzanine Partners IV... regulated as a business development company (``BDC'') under the Act. Fifth Street is a specialty finance...

The U.S. Forest Service Job Corps 28 Civilian Conservation Centers

Science.gov (United States)

Larry J. Dawson; Alicia D. Bennett

2011-01-01

In 1964, President Lyndon B. Johnson laid out his plan for the Great Society. His vision for America was one in which all segments of society could participate equally. To this end, the Economic Opportunity Act, which created Job Corps, was passed. The Job Corps program provides economically disadvantaged youth, ages 16-24, with the academic, vocational, and social...

75 FR 50010 - Atchison Casting Corp. (n/k/a Bradken-Atchison/St. Joseph, Inc.), CityFed Financial Corp., Divine...

Science.gov (United States)

2010-08-16

...., Hampton Consulting Corp., and Jake's Pizza International, Inc.; Order of Suspension of Trading August 12... there is a lack of current and accurate information concerning the securities of Jake's Pizza...

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 ...

Effectiveness of the Marine Corps’ Junior Enlisted Performance Evaluation System: An Evaluation of Proficiency and Conduct Marks

Science.gov (United States)

2017-03-01

courses, college credits received from traditional and vocational educational institutes , and through the College Level Examination Program (CLEP). 4...21 A. LABOR ECONOMIC THEORY : INTERNAL LABOR MARKETS...Corps IRAM Individual Records Administrative Manual MCI Marine Corps Institute MCSF Marine Corps Security Forces MCU Marine Corps University

Applying Technology to Marine Corps Distance Learning

National Research Council Canada - National Science Library

Broihier, Michael

1997-01-01

The purpose of this thesis is to investigate the application of technology to distance learning with the intention of recommending to the Marine Corps a feasible migration path away from its current...

Constraints placed on Marine Corps ammunition requirements by the PPBS

OpenAIRE

Burlingham, Donald Michael.

1988-01-01

Approved for public release; distribution is unlimited The purpose of this report is to determine whether the products of the Planning, Programming and Budgeting System (PPBS) are worthwhile, they must be measured against some form of output. The prepositioned War Reserve (PWR) of the Marine Corps is a measure of sustainability: a desired output of the PPBS. This thesis investigated the PPBS, the Marine Corps programming methodology and ammunition requirement generation to determine whethe...

75 FR 74711 - Planet Energy (USA) Corp.; Supplemental Notice That Initial Market-Based Rate Filing Includes...

Science.gov (United States)

2010-12-01

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ER11-2166-000] Planet Energy (USA) Corp.; Supplemental Notice That Initial Market-Based Rate Filing Includes Request for Blanket... proceeding, of Planet Energy (USA) Corp.'s application for market-based rate authority, with an accompanying...

75 FR 76004 - Planet Energy (New York) Corp.; Supplemental Notice That Initial Market-Based Rate Filing...

Science.gov (United States)

2010-12-07

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ER11-2179-000] Planet Energy (New York) Corp.; Supplemental Notice That Initial Market-Based Rate Filing Includes Request for... proceeding, of Planet Energy (New York) Corp.'s application for market-based rate authority, with an...

20 CFR 638.812 - State and local taxation of Job Corps deliverers.

Science.gov (United States)

2010-04-01

... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false State and local taxation of Job Corps deliverers. 638.812 Section 638.812 Employees' Benefits EMPLOYMENT AND TRAINING ADMINISTRATION, DEPARTMENT OF... § 638.812 State and local taxation of Job Corps deliverers. The Act provides that transactions conducted...

Marine Corps Pay Incentives

Science.gov (United States)

Marines from 2000 to 2017. The thesis includes a literature review on economic theory related to pay incentives in the Department of Defense, a...The purpose of this thesis to provide the Marine Corps with a comprehensive report on pay incentive programs and special pay that were available to...summarization of pay incentive categories, a data analysis on take-up rates and average annual amounts at the end of each fiscal year, and a program review

Le(s "corps politique(s" dans L ’Esprit des lois de Montesquieu

Directory of Open Access Journals (Sweden)

Denis de Casabianca

2007-01-01

Full Text Available Cet article cherche à déterminer les usages de l’expression « corps politique » dans L’Esprit des lois, pour préciser le regard que Montesquieu porte sur la réalité politique, et comment il entend poser son questionnement par rapport à la tradition contractualiste et jusnaturaliste. Dans les rapports qui existent entre les différents « corps politiques », se joue l’unité du « corps politique ». La monarchie modérée ne saurait exister sans une bonne composition des puissances.

77 FR 5398 - Safety Zone; Atlantic Intracoastal Waterway, Vicinity of Marine Corps Base, Camp Lejeune, NC

Science.gov (United States)

2012-02-03

...-AA00 Safety Zone; Atlantic Intracoastal Waterway, Vicinity of Marine Corps Base, Camp Lejeune, NC... zone on the Atlantic Intracoastal Waterway (AICW) adjacent to Marine Corps Base (MCB) Camp Lejeune..., Vicinity of Marine Corps Base, Camp Lejeune, NC in the Federal Register (77 FR 1431). We received no...

28 CFR 92.3 - How and when should I apply to participate in the Police Corps?

Science.gov (United States)

2010-07-01

... participate in the Police Corps? 92.3 Section 92.3 Judicial Administration DEPARTMENT OF JUSTICE (CONTINUED) OFFICE OF COMMUNITY ORIENTED POLICING SERVICES (COPS) Police Corps Eligibility and Selection Criteria § 92.3 How and when should I apply to participate in the Police Corps? (a) The application and...

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.

78 FR 76377 - Makism3D Corp.; Order of Suspension of Trading

Science.gov (United States)

2013-12-17

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] Makism3D Corp.; Order of Suspension of Trading... protection of investors require a suspension of trading in the securities of Makism3D Corp. (``Makism3D... opinion that the public interest and the protection of investors require a suspension of trading in the...

Peace corps partnered health services implementation research in global health: opportunity for impact.

Science.gov (United States)

Dykens, Andrew; Hedrick, Chris; Ndiaye, Youssoupha; Linn, Annē

2014-09-01

There is abundant evidence of the affordable, life-saving interventions effective at the local primary health care level in low- and middle-income countries (LMICs). However, the understanding of how to deliver those interventions in diverse settings is limited. Primary healthcare services implementation research is needed to elucidate the contextual factors that can influence the outcomes of interventions, especially at the local level. US universities commonly collaborate with LMIC universities, communities, and health system partners for health services research but common barriers exist. Current challenges include the capacity to establish an ongoing presence in local settings in order to facilitate close collaboration and communication. The Peace Corps is an established development organization currently aligned with local health services in many LMICs and is well-positioned to facilitate research partnerships. This article explores the potential of a community-Peace Corps-academic partnership approach to conduct local primary healthcare services implementation research. The Peace Corps is well positioned to offer insights into local contextual factors because volunteers work closely with local leaders, have extensive trust within local communities, and have an ongoing, constant, well-integrated presence. However, the Peace Corps does not routinely conduct primary healthcare services implementation research. Universities, within the United States and locally, could benefit from the established resources and trust of the Peace Corps to conduct health services implementation research to advance access to local health services and further the knowledge of real world application of local health services in a diversity of settings. The proposed partnership would consist of (1) a local community advisory board and local health system leaders, (2) Peace Corps volunteers, and (3) a US-LMIC academic institutional collaboration. Within the proposed partnership approach

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.

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.

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.

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.)

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.

Le Don De Corps En Cote D\\'ivoire | Broalet | African Journal of ...

African Journals Online (AJOL)

Introduction Le don de corps qui permet de se procurer le matériel anatomique de dissection que constitue le cadavre humain n\\'existe pas en Afrique noire et notamment en Cote d\\' Ivoire. Objectif Recueillir des informations sur le don de corps à Abidjan. Méthode Les auteurs rapportent les informations recueillies au cours ...

Sun, Sand and Water: A History of the Jacksonville District U.S. Army Corps of Engineers 1821-1975

Science.gov (United States)

1981-01-01

centuries-old ecological balance which had been developed in nature was not understood, considered, or known by those early pioneers who envisioned tap...could only see that "water will run down hill," brought ecological disasters to south Florida; the Jacksonville District Engineers, studying the...guided by the experienced man on the job. Starrett Brothers also shifted to prefabrication to aid construction. They set up plans for building

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...

Maintaining the Marine Corps’ Tank Dominant Combat Overmatch in an Uncertain Future

Science.gov (United States)

2012-03-12

Company, 1999), 6. 2 2011 Tank Conference Report. (Dated 02 Feb 2011) 3 Kenneth E. Estes. Marines Under Armor : The Marine Corps and the Armored...Kenneth W. Marines Under Armor : The Marine Corps and the Armored Fighting Vehicle, 1916-2000. Annapolis, MD: Naval Institute Press, 2000. Ford

76 FR 13438 - AccessTel, Inc., American Asset Management Corp., DME Interactive Holdings, Inc., DocuPort, Inc...

Science.gov (United States)

2011-03-11

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] AccessTel, Inc., American Asset Management Corp., DME Interactive Holdings, Inc., DocuPort, Inc., and iCarbon Corp., Order of Suspension of... Asset Management Corp. because it has not filed any periodic reports since the period ended March 31...

77 FR 59984 - Comment Request for Information Collection for Placement Verification and Follow-Up of Job Corps...

Science.gov (United States)

2012-10-01

... to Lawrence Lyford, Office of Job Corps, Room N-4507, Employment and Training Administration, U.S... multiple barriers to employment faced by youth throughout the United States. Job Corps is authorized by.... Information about satisfaction with the services provided by Job Corps. Type of Review: Extension without...

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 Marginal Majority: Women War Correspondents in the Salvadoran Press Corps Association (SPCA).

Science.gov (United States)

Pedelty, Mark

1997-01-01

Finds that men in the Salvadoran Press Corps Association used Hemingwayesque myths and ritual performances which dominated corps culture to escape the discipline of corporate press institutions, while women correspondents experienced the rituals as an obstacle to individual and collective attempts to negotiate or reform the field. Analyses gender…

75 FR 45111 - Electric Quarterly Reports; Strategic Energy Management Corp.; Solaro Energy Marketing...

Science.gov (United States)

2010-08-02

.... ER00-167-000; Docket No. ER03- 752-000] Electric Quarterly Reports; Strategic Energy Management Corp.; Solaro Energy Marketing Corporation; Notice of Revocation of Market- Based Rate Tariff July 23, 2010. On... FERC ] 61,334 (2003). In the June 25 Order, the Commission directed Strategic Energy Management Corp...

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.

Enhancement of Marine Corps' Recruiting and Retention Programs

National Research Council Canada - National Science Library

Davis, Shalisa W

2008-01-01

Though the Marine Corps has historically met its recruitment and retention goals, recruiting has become more challenging and cultural indicators show that recruiting will become even more difficult in the future...

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

More LNG ship orders for GD (General Dynamics Corp. )

Energy Technology Data Exchange (ETDEWEB)

1977-09-01

General Dynamics Corp. has been awarded a contract for two LNG tankers to transport LNG from Algeria to Lake Charles, La., with the U.S. Maritime Administration funding 25.5% of the $155 million cost of each vessel. The two ships are being built for Lachmar Inc., of Delaware, a partnership composed of Morgas Inc., Pantheon Inc., and Pelmar Inc., subsidiaries respectively of Moore-McCormack Bulk Transport Inc., General Dynamics Corp., and Panhandle Eastern Pipe Line Co. Upon completion in Dec. 1979 and Mar. 1980, the ships will be operated by Gastrans Inc. of Delaware, which is also a subsidiary of Moore-McCormack.

Engineers at the Golden Gate: A History of the San Francisco District U.S. Army Corps of Engineers 1866-1980

Science.gov (United States)

1980-01-01

long-distance, endurance horse ride. A rather unique activity is the special observance of Native American Day, held to pay tribute to the Porno ...by San Francisco District in conjunction with the Mendocino Porno Council, a Native-American organization, to promote American Indian heritage and

76 FR 67175 - Riverbank Hydro No. 2 LLC, Lock Hydro Friends Fund XXXVI, Arkansas Electric Cooperative Corp...

Science.gov (United States)

2011-10-31

...; 14149-000] Riverbank Hydro No. 2 LLC, Lock Hydro Friends Fund XXXVI, Arkansas Electric Cooperative Corp... Lock Hydro Friends Fund XXXVI (Lock Hydro) and on April 11, 2011, Arkansas Electric Cooperative Corp... & Dam No. 3, as directed by the Corps. Applicant Contact: Mr. Wayne F. Krouse, Hydro Green Energy, 5090...

Using Peace Corps volunteers in community eye health

OpenAIRE

Barnes, Shawn

2009-01-01

Initiating a community eye health programme in an unfamiliar culture and language can be a daunting task. This report focuses on an underutilised resource for community eye health: American Peace Corps volunteers.

75 FR 4107 - Comment Request for Information Collection for Job Corps Application Data

Science.gov (United States)

2010-01-26

... youth throughout the United States. Job Corps is authorized by Title I, Subtitle C, of the Workforce... information for a homeless/runaway/foster child in one category. Job Corps proposes a slight modification to this form to capture the information for a homeless/runaway/foster child in three separate categories...

20 CFR 670.980 - What are the indicators of performance for Job Corps?

Science.gov (United States)

2010-04-01

... Provisions § 670.980 What are the indicators of performance for Job Corps? (a) At a minimum, the performance... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false What are the indicators of performance for Job Corps? 670.980 Section 670.980 Employees' Benefits EMPLOYMENT AND TRAINING ADMINISTRATION...

Analysis of the Retention and Affiliation Factors Affecting the Active and Reserve Naval Nurse Corps

National Research Council Canada - National Science Library

Messmer, Scott J; Pizanti, Kimberly A

2007-01-01

...) an empirical analysis to analyze characteristics of those who are retained in the active Naval Nurse Corps and those who affiliate with the reserve Naval Nurse Corps using multivariate logit regressions...

Corps de la Paix Madagascar Livre du Formateur. Langue: Sakalava (Peace Corps Madagascar Teacher's Manual. Language: Sakalava).

Science.gov (United States)

Tshiangale, Mupemba Wa

This teacher's manual for Sakalava, a dialect of Malagasy, is designed for the specific language instruction needs of Peace Corps personnel in Madagascar. It is written primarily in French and Sakalava, with some titles in English. It consists of 29 topical lessons, each geared to a specific domain and competency and containing information on…

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.

32 CFR 631.17 - Marine Corps policy.

Science.gov (United States)

2010-07-01

... 32 National Defense 4 2010-07-01 2010-07-01 true Marine Corps policy. 631.17 Section 631.17... CRIMINAL INVESTIGATIONS ARMED FORCES DISCIPLINARY CONTROL BOARDS AND OFF-INSTALLATION LIAISON AND OPERATIONS Off-Installation Operations (Military Patrols and Investigative Activities) and Policy § 631.17...

Web application to access U.S. Army Corps of Engineers Civil Works and Restoration Projects information for the Rio Grande Basin, southern Colorado, New Mexico, and Texas

Science.gov (United States)

Archuleta, Christy-Ann M.; Eames, Deanna R.

2009-01-01

The Rio Grande Civil Works and Restoration Projects Web Application, developed by the U.S. Geological Survey in cooperation with the U.S. Army Corps of Engineers (USACE) Albuquerque District, is designed to provide publicly available information through the Internet about civil works and restoration projects in the Rio Grande Basin. Since 1942, USACE Albuquerque District responsibilities have included building facilities for the U.S. Army and U.S. Air Force, providing flood protection, supplying water for power and public recreation, participating in fire remediation, protecting and restoring wetlands and other natural resources, and supporting other government agencies with engineering, contracting, and project management services. In the process of conducting this vast array of engineering work, the need arose for easily tracking the locations of and providing information about projects to stakeholders and the public. This fact sheet introduces a Web application developed to enable users to visualize locations and search for information about USACE (and some other Federal, State, and local) projects in the Rio Grande Basin in southern Colorado, New Mexico, and Texas.

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

An Analysis of United States Marine Corps Enlisted Entry-Level Training Using Supply Chain and Operations Management

Science.gov (United States)

2010-12-01

An Analysis of United States Marine Corps Enlisted Entry-Level Training Using Supply Chain and Operations Management ______________________________________ By...Report 4. TITLE AND SUBTITLE: An Analysis of United States Marine Corps Enlisted Entry-Level Training Using Supply Chain and Operations Management 6...Level Training; United States Marine Corps; Operations Management ; Supply Chain Management; Process Analysis 16. PRICE CODE 17. SECURITY

The role of the US Army Veterinary Corps in military family pet health.

Science.gov (United States)

Vincent-Johnson, Nancy A

2013-01-01

Even though privately-owned pet care is a lower priority mission than military working dog care, food inspection,and the public health mission, it is still very important,and the one that many Veterinary Corps officers, civil-ian veterinarians, and technicians enjoy the most. The vast majority of veterinarians and technicians went into veterinary medicine because of a love for animals. It is fulfilling to offer guidance to a client with a new puppy or kitten, see a sick pet improve after treatment, and interact with dozens of animals and clients in a day. The services provided by the Army Veterinary Corps in car-ing for pets has expanded over the years and the standard of care has improved as well. It is truly a privilege to serve those who dedicate themselves to the protection of our Nation. The Army Veterinary Corps is indeed proud to provide care to the pets of Warfighters of the Army,Navy, Marine Corps, Air Force, and Coast Guard; their family members; and our military retirees.

20 CFR 670.535 - Are Job Corps centers required to establish behavior management systems?

Science.gov (United States)

2010-04-01

... behavior management systems? 670.535 Section 670.535 Employees' Benefits EMPLOYMENT AND TRAINING... systems? (a) Yes, each Job Corps center must establish and maintain its own student incentives system to encourage and reward students' accomplishments. (b) The Job Corps center must establish and maintain a...

Council Districts

Data.gov (United States)

Town of Cary, North Carolina — View the location of the Town of Cary’s four Town Council districts.Please note that one district, District A, is split into two geo-spatial areas. One area is in...

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...

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.

Unity is strength: staff college and the British officer corps.

Science.gov (United States)

King, Anthony

2009-03-01

Utilizing Bourdieu's concept of the habitus, Keith Macdonald has recently examined the elite social origins of the British officer corps. His analysis is valid as far at it goes but it ignores the professional practices of British officers. This article examines Britain's Joint Services Command and Staff College to assess the unification of the three services around common forms of military practice. It argues that while the new staff college has been effective in disseminating new forms of professional expertise among British officers, various practices have been institutionalized which actively undermine the unity of the officer corps.

Additive Manufacturing in the Marine Corps

Science.gov (United States)

2015-06-01

TERMS additive manufacturing, 3D printing, improving logistics 15. NUMBER OF PAGES 123 16. PRICE CODE 17. SECURITY CLASSIFICATION OF REPORT...One such method is ultrasonic agglomeration. The process produces compact snack items that are nutrient-dense and shelf-stable. Currently Army...possess outdated technology. Additionally, prices for the printers are dropping significantly.202 Therefore, the Marine Corps will not only have outdated

75 FR 68607 - BP Canada Energy Marketing Corp. Apache Corporation; Notice for Temporary Waivers

Science.gov (United States)

2010-11-08

... Energy Marketing Corp. Apache Corporation; Notice for Temporary Waivers November 1, 2010. Take notice that on October 29, 2010, BP Canada Energy Marketing Corp. and Apache Corporation filed with the... assistance with any FERC Online service, please e-mail [email protected] , or call (866) 208-3676...

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.

75 FR 17161 - Job Corps: Preliminary Finding of No Significant Impact (FONSI) for the Installation of a Small...

Science.gov (United States)

2010-04-05

... DEPARTMENT OF LABOR Office of the Secretary Job Corps: Preliminary Finding of No Significant Impact (FONSI) for the Installation of a Small Wind Turbine at the Pine Ridge Job Corps Center Located at... the Pine Ridge Job Corps Center, 15710 Highway 385, Chadron, NE 69337. SUMMARY: Pursuant to the...

Interim Feasibility Report and Draft Environmental Impact Statement, Grays Harbor, Chehalis and Hoquiam Rivers, Washington, Channel Improvements for Navigation.

Science.gov (United States)

1982-06-01

community depends on the continued descent of organic materials from the overlying waters for nourishment. The Dungeness crab ( Cancer magister) is an...immediately north and south of Grays Harbor. EIS-34 o Crabs. Dungeness crabs ( Cancer magister) are abundant in Grays Harbor, though most are smaller...Cooper Cultural Resources Mama Seattle District, Evaluation Corps of Engineers Endangered Species Brunner Seattle District, Evaluation Corps of Engineers

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.

An Evaluation of Marine Corps Educational Credentials.

Science.gov (United States)

1983-12-01

Recruits with the same 103 Table 17 Percent Attrition Among Male Nonprior Service Accessions Who Entered the Marine Corps between FY 1978-83 by aLevel of...dictor: It Works, But Why, (Anaheim, CA.: paper presented at the 91st Annual Convention of the American Psychological Association, August 1983

77 FR 35946 - Notice of Availability for the Final Environmental Impact Statement/Environmental Impact Report...

Science.gov (United States)

2012-06-15

.... Army Corps of Engineers, Los Angeles District Regulatory Division (Corps), in coordination with the Los... 103 of the Marine Protection, Research, and Sanctuaries Act, to implement regulated activities in and...

76 FR 48751 - Lanxess Corp.; Filing of Food Additive Petition (Animal Use); Calcium Formate

Science.gov (United States)

2011-08-09

.... FDA-2011-F-0549] Lanxess Corp.; Filing of Food Additive Petition (Animal Use); Calcium Formate AGENCY... Administration (FDA) is announcing that Lanxess Corp. has filed a petition proposing that the food additive..., PA 15275-1112. The petition proposes to amend the food additive regulations in part 573 Food...

20 CFR 670.400 - Who is eligible to participate in the Job Corps program?

Science.gov (United States)

2010-04-01

... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false Who is eligible to participate in the Job Corps program? 670.400 Section 670.400 Employees' Benefits EMPLOYMENT AND TRAINING ADMINISTRATION, DEPARTMENT OF LABOR THE JOB CORPS UNDER TITLE I OF THE WORKFORCE INVESTMENT ACT Recruitment, Eligibility...

20 CFR 670.490 - How long may a student be enrolled in Job Corps?

Science.gov (United States)

2010-04-01

... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false How long may a student be enrolled in Job Corps? 670.490 Section 670.490 Employees' Benefits EMPLOYMENT AND TRAINING ADMINISTRATION, DEPARTMENT OF LABOR THE JOB CORPS UNDER TITLE I OF THE WORKFORCE INVESTMENT ACT Recruitment, Eligibility, Screening...

To the Issue on the History of the Cadet Corps in Tiflis

Directory of Open Access Journals (Sweden)

Mamuka Gogitidze

2016-06-01

Full Text Available The article discusses the issue of organizing in Tiflis the cadet corps. The Tiflis cadet corps was founded in 1882 year on the basis of the military school that existed in Tbilisi since 1875 year. In 1827, the officers and officials who served in the Caucasus, presented a petition to Nicholas I on the establishment in the city of Tiflis cadet corps in 150 children. The authors note that a serious problem to a certain extent, affect the quality of teaching cadets, the housing problem. Housing in Tiflis at that time very little was built. It was very difficult to find an apartment in the city on the take-out money for these purposes. The only solution to this problem was to increase salaries to pay for housing. This was done by Layminga recommendations.

75 FR 49005 - In the Matter of Geotec, Inc., InnoPet Brands Corp., Marbledge Group, Inc. (n/k/a AR Growth...

Science.gov (United States)

2010-08-12

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] In the Matter of Geotec, Inc., InnoPet Brands Corp., Marbledge Group, Inc. (n/k/a AR Growth Finance Corp.), Phlo Corp., Pliant Systems, Inc... Brands Corp. because it has not filed any periodic reports since the period ended March 31, 1998. It...

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.

The Canadian Corps in the Great War: A Learning Organization in Action

Science.gov (United States)

2016-04-04

resembled Garvin’s learning cycle, but it did so unevenly throughout the Corps. For instance, the 1st Division’s attacks benefited from improved artillery...Passchendaele, convinced that the cost of winning the battle outweighed the potential benefit . Currie’s unique position as a semi-independent Corps... Wine in New Bottles: A Comparison of British and Canadian Preparations for the Battle of Arras." In Vimy Ridge: A Canadian Reassessment, edited by

75 FR 74082 - Agency Information Collection Activities; Submission for OMB Review; Comment Request; Job Corps...

Science.gov (United States)

2010-11-30

... for OMB Review; Comment Request; Job Corps Placement and Assistance Record ACTION: Notice. SUMMARY... Administration (ETA) sponsored information collection request (ICR) titled, ``Job Corps Placement and Assistance... obtain information about student training for placement of students in jobs, further education or...

5 CFR 831.304 - Service with the Cadet Nurse Corps during World War II.

Science.gov (United States)

2010-01-01

... 5 Administrative Personnel 2 2010-01-01 2010-01-01 false Service with the Cadet Nurse Corps during World War II. 831.304 Section 831.304 Administrative Personnel OFFICE OF PERSONNEL MANAGEMENT (CONTINUED... Nurse Corps during World War II. (a) Definitions and special usages. In this section— (1) Basic pay is...

75 FR 3948 - Big Sky Energy Corp., Biomedical Waste Systems, Inc., Biometrics Security Technology, Inc...

Science.gov (United States)

2010-01-25

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] Big Sky Energy Corp., Biomedical Waste Systems, Inc., Biometrics Security Technology, Inc., Biosys, Inc., Bolder Technologies Corp., Boyds Wheels, Inc... securities of Biometrics Security Technology, Inc. because it has not filed any periodic reports since...

Corps, apparences vestimentaires et identités en France à la Renaissance

Directory of Open Access Journals (Sweden)

Isabelle Paresys

2012-02-01

Full Text Available Un corps vêtu spécifique émerge à la Renaissance en France et particulièrement à la Cour. Le vêtement impose sa marque sur le corps, accentue le dimorphisme sexuel des apparences, et donne à celles-ci rectitude ou distorsion, transformant le corps en une surface décorative sur laquelle peut s’exprimer la magnificence des souverains et celle de leur cour. Ce corps vêtu résulte de l’influence de modes européennes et de nouvelles normes de civilité qui construisent la présentation de soi. Elles trouvent leur pleine expression à la cour où le corps de mode aristocratique affiche une éloquence distinctive et cosmopolite. Dans le même temps, les identités qu’inculque le vêtement au corps paraissent menacées par les transferts de pratiques vestimentaires entre les sexes, entre les groupes sociaux et entre les nations, entraînant un malaise envers ce brouillage des apparences qui prend une couleur particulière durant les guerres civiles quand se pose de manière sanglante la question des identités et de la conscience nationale.Body, Dress and Identities in Renaissance France. A specific type of clothed body emerged in 16th century France especially at Court. Clothing put its mark on the body, accentuating sexual dimorphism, imposing either Renaissance rectitude or distortions, transforming the body into a decorative surface for displaying magnificence. Its specificity resulted from the influence of various vestimentary and decorative fashions, starting with the new kinds of civility circulating in Renaissance Europe, which fashioned self-presentation. It found its fullest expression on the bodies of cosmopolitan aristocrats seeking a distinctive eloquence for their particular identity. At the same time, the identities which clothes imprinted on their wearer seemed threatened by transfers of vestimentary practices between sexes, sexual groups, and/or nations. The unease created by this mixture of appearances reflects that of

Conception et corps féminin selon Hildegarde de Bingen

OpenAIRE

Moulinier , Laurence

2005-01-01

International audience; Hildegarde a laissé une œuvre multiforme qui a retenu depuis longtemps l'attention des spécialistes des études féminines, voire féministes. C'est à bon droit en tout cas que sa médecine, et la part à la fois importante et originale qu'elle y fait au corps féminin, ont suscité maint commentaire. La femme et son corps, en tant qu'origine de la vie, sont en effet très présents en divers endroits de son œuvre, et si des femmes écrivent à Hildegarde pour qu'elle les libère ...

U.S. Army Corps of Engineers and U.S. Department of Energy partnering for cleanup of the 1100 Area, Hanford Site, Washington

International Nuclear Information System (INIS)

Johansen, M.; Liias, R.; Chong, R.

1994-01-01

The US Department of Energy's Hanford Site was listed on the National Priorities List (NPL) in July 1989 and was divided and listed as four Sites: the 1100 Area, the 100 Area, the 200 Area, and the 300 Area. Each Area was further divided into sub-units called Operable Units. This paper describes Remedial Investigation and Feasibility Study activities for the 1100 Area leading to the first Record of Decision at the Hanford Site. Key issues included: (1) Definition of future land use; risk assessments and resulting remedial actions depended heavily upon future land use definition because no significant exposure pathways currently exist for the Site, (2) Potential impacts of groundwater contamination to a nearby groundwater well field supplying potable water to Richland, (3) Coordination with an offsite potentially responsible party (PRP) from whose property the groundwater contamination emanated, and (4) The development and determination of precedent setting cleanup requirements and approaches for the entire Hanford Site. The US Army Corps of Engineers, Walla Walla District, performed work leading to the signing of the Record of Decision in September, 1993. The Corps continues to perform investigative, design, and remedial action work at areas of the Site including activities supporting the cleanup and ultimate release of two large portions of the Hanford Site known as the Arid Lands Ecology Reserve (ALE) and the North Slope. These two areas comprise more than half of the total area of the entire Hanford reservation

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.

75 FR 71102 - White Mountain Hydroelectric Corp.; Notice of Application for Amendment of License, and...

Science.gov (United States)

2010-11-22

... Hydroelectric Corp.; Notice of Application for Amendment of License, and Soliciting Comments, Motions To Intervene, and Protests November 12, 2010. Take notice that the following hydroelectric application has been... Hydroelectric Corp. e. Name of Project: Apthorp Hydroelectric Project. f. Location: The project is located on...

75 FR 27602 - In the Matter of BVR Technologies Ltd. (n/k/a Technoprises Ltd.), Crystal Graphite Corp., Devine...

Science.gov (United States)

2010-05-17

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] In the Matter of BVR Technologies Ltd. (n/k/a Technoprises Ltd.), Crystal Graphite Corp., Devine Entertainment Corp., GEE TEN Ventures, Inc., National Construction, Inc. (n/k/a E.G. Capital, Inc.), SHEP Technologies, Inc., and WHEREVER.Net Holding Corp.; Order...

Corps de la Paix Madagascar Livre du Stagiare. Langue: Malagasy Ofisialy (Peace Corps Madagascar Volunteer Manual. Language: Official Malagasy).

Science.gov (United States)

Tshiangale, Mupemba Wa

This manual for Malagasy is designed for the specific language instruction needs of Peace Corps personnel working in Madagascar. It is written primarily in English and Malagasy, with introductory sections in French. It consists of 29 topical lessons, each geared to a specific domain and competency and containing information on needed materials,…

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

76 FR 80346 - Notice of Availability for the Draft Environmental Impact Statement/Environmental Impact Report...

Science.gov (United States)

2011-12-23

.... Army Corps of Engineers, Los Angeles District Regulatory Division (Corps), in coordination with the Los... pursuant to Section 10 of the Rivers and Harbors Act, and Section 103 of the Marine Protection, Research...

Le corps souffrant de Rifqâ, sainte maronite du xixe siècle

Directory of Open Access Journals (Sweden)

Chantal Verdeil

2006-11-01

Full Text Available Canonisée en juillet 2001, la moniale maronite Rifqâ (1832-1914, aveugle et paralysée, a supporté avec patience d’atroces souffrances physiques. Elle est présentée aujourd’hui par l’Église maronite comme la “ patronne des souffrants ” et les récits de vie écrits pour promouvoir sa cause illustrent la conception qu’a l’Église de la souffrance, de la maladie, et par conséquent du corps. Si elle laisse aux médecins le soin du corps malade, elle s’efforce en revanche de donner un sens à la souffrance, une souffrance qui permet de libérer l’âme du corps et de participer à la Passion du Christ.

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

Significant pre-accession factors predicting success or failure during a Marine Corps officer’s initial service obligation

OpenAIRE

Johnson, Jacob A.

2015-01-01

Approved for public release; distribution is unlimited Increasing diversity and equal opportunity in the military is a congressional and executive priority. At the same time, improving recruiting practices is a priority of the commandant of the Marine Corps. In an effort to provide information to the Marine Corps that may improve recruiting practice and enable retention of a higher quality and more diverse officer corps, probit econometric models are estimated to identify significant facto...

75 FR 13805 - Aspen Group Resources Corp., Commercial Concepts, Inc., Desert Health Products, Inc., Equalnet...

Science.gov (United States)

2010-03-23

... Concepts, Inc., Desert Health Products, Inc., Equalnet Communications Corp., Geneva Steel Holdings Corp... securities of Commercial Concepts, Inc. because it has not filed any periodic reports since the period ended... accurate information concerning the securities of Desert Health Products, Inc. because it has not filed any...

28 CFR 92.2 - Am I eligible to apply to participate in the Police Corps?

Science.gov (United States)

2010-07-01

... the Police Corps? 92.2 Section 92.2 Judicial Administration DEPARTMENT OF JUSTICE (CONTINUED) OFFICE... the participant will complete an educational course of study leading to the award of a baccalaureate... in full or otherwise satisfied. (c) Educational assistance under the Police Corps Act for any course...

78 FR 63559 - Order Of Suspension of Trading; In the Matter of ARX Gold Corp.

Science.gov (United States)

2013-10-24

... of ARX Gold Corp. October 22, 2013. It appears to the Securities and Exchange Commission (``Commission'') that there is a lack of current and accurate information concerning the securities of ARX Gold Corp. (``ARX Gold''), quoted under the ticker symbol DUCP, because of questions regarding the...

20 CFR 670.510 - Are Job Corps center operators responsible for providing all vocational training?

Science.gov (United States)

2010-04-01

... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false Are Job Corps center operators responsible for providing all vocational training? 670.510 Section 670.510 Employees' Benefits EMPLOYMENT AND TRAINING ADMINISTRATION, DEPARTMENT OF LABOR THE JOB CORPS UNDER TITLE I OF THE WORKFORCE INVESTMENT ACT...

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.

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

A New Approach in the Simplification of a Multiple-Beam Forming Network Based on CORPS Using Compressive Arrays

Directory of Open Access Journals (Sweden)

Armando Arce

2012-01-01

Full Text Available This research paper deals with a innovative way to simplify the design of beam-forming networks (BFNs for multibeam steerable antenna arrays based on coherently radiating periodic structures (CORPS technology using the noniterative matrix pencil method (MPM. This design approach is based on the application of the MPM to linear arrays fed by CORPS-BFN configurations to further reduce the complexity of the beam-forming network. Two 2-beam design configurations of CORPS-BFN for a steerable linear array are analyzed and compared using this compressive method. Simulation results show the effectiveness and advantages of applying the MPM on BFNs based on CORPS exploiting the nonuniformity of the antenna elements. Furthermore, final results show that the integration of CORPS-BFN and MPM reduces the entire antenna system including the antenna array and the beam-forming network subsystem resulting in a substantial simplification in such systems.

Development of a Civil Engineer Corps Community Portal Prototype

National Research Council Canada - National Science Library

Rader, Neil

2002-01-01

The Civil Engineer Corps (CEC) is a relatively small Navy community consisting of approximately 1300 officers, Billet locations for the CEC range from Bahrain, Saudi Arabia to Keflavik, Iceland, CEC officers have a broad range...

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.

Building esprit de corps: learning to better navigate between "my" patient and "our" patient.

Science.gov (United States)

DeRenzo, Evan G; Schwartz, Jack

2010-01-01

Excellence in the care of hospital patients, particularly those in an intensive care unit, reflects esprit de corps among the care team. Esprit de corps depends on a delicate balance; each clinician must preserve a sense of personal responsibility for "my" patient and yet participate in the collaborative work essential to the care of "our" patient. A harmful imbalance occurs when a physician demands total control of the decision-making process, especially concerning end-of-life treatment options. Although emotional factors may push a physician to claim decision-making exclusivity, compounded by a legal framework that overemphasizes individual responsibility, esprit de corps can be preserved through timely communication among clinicians and a recognition that optimal care for "my" patient requires effective team practice.

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.

Interdiction de Genre: La Loi Qui Fait Taire le Corp

Directory of Open Access Journals (Sweden)

Renato Duro Dias

2015-12-01

Full Text Available Cette recherche discute les identités de genre avec la base dans l'analyse du travail La Ley no escrita (2012 d'Oihana Cordero. Le travail de l'artiste plasticien a comme l'objectif provoque la réflexion sur les lois et les normes juridiques-sociales qu'ils existent autour du caractère ficcional et le genre performatife, révélant de nouvelles lectures sur l'acte du corps. C'est un travail sculptural (KRAUSS, 1984, composé par une base blanche, une épée et un groupe de feuilles, dans lesquelles ils sont exprimés, par un contrat de genre, comme ils se prononcent, interseccionam et c'est des interdictions imposées aux possibilités de genre. Comme ceci, les règles et des règlements sont faits la preuve imposés par le binarisme du genre que la force soumet et ils arrêtent des identités pour le compte de la normalisation (normatisation des corps. Pour cette enquête les concepts de genre sont problematize, le corps subjugué, la normativité et de la performance dans le Butler (2012a, 2012b, 2013, Foucault (1997, 1998, 2014, Fraser (2002, 2006, 2009, Scott (2008, Cordero (2012 et Preciado (2011. Cordero (2012, dans son travail, nous présente les copies multiples d'un contrat de papier imprimé a passé par une épée. C'est une métaphore, une forme de représentation dogmatizé du corps par les actes visibles et invisibles qui compose et ils naturalisent les identités de genre dans une dualité obscurantiste homme/femme. Ce travail comme l'objet conceptuel (FREIRE 2006 produit la puissance exprimée pour en plus d'un document légal perforé, elle vise à provoquer les gens de contempler sur leurs pratiques et leurs corps, mais, par-dessus tout, réfléchir comme ces périmètres ont de transgression difficile. Lors de l'analyse de l'intersection possible des identités de genre et un objet artistique dans le domaine juridique est prévu pour gérer des éléments capables de révéler les technologies complexes corporels politiques

Professionalization of the Senior Chinese Officer Corps Trends and Implications

National Research Council Canada - National Science Library

Mulvenon, James

1997-01-01

... consequences for longer-term U.S. national security interests. Specifically, this report evaluates demographic and cohort changes in the officer corps of the Chinese People's Liberation Army (PLA...

77 FR 47690 - 30-Day Notice of Proposed Information Collection: Civilian Response Corps Database In-Processing...

Science.gov (United States)

2012-08-09

.... Title of Information Collection: Civilian Response Corps Database In-Processing Electronic Form. OMB... DEPARTMENT OF STATE [Public Notice 7976] 30-Day Notice of Proposed Information Collection: Civilian Response Corps Database In-Processing Electronic Form, OMB Control Number 1405-0168, Form DS-4096...

75 FR 61746 - New England Wire Technologies Corp; Supplemental Notice That Initial Market-Based Rate Filing...

Science.gov (United States)

2010-10-06

...-referenced proceeding of New England Wire Technologies Corp's application for market-based rate authority... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ER10-2754-000] New England Wire Technologies Corp; Supplemental Notice That Initial Market-Based Rate Filing Includes Request for...

Le corps, instrument de connaissance du monde: la connaissance des Anciens Toltèques

Directory of Open Access Journals (Sweden)

Mabel Franzone

2009-10-01

Full Text Available Cet article traite le corps comme instrument de la connaissance de l’univers. Le corps s’étend vers des contrées infinies, se prolonge, atteint d’autres règnes, cherche l’unité absolue de ce qu’on appelle l’intention. L’intention est le mot clé pour les Anciens Toltèques, réunissant sous ce mot le visible et l’invisible, la volonté de la Nature et celle de l’univers. Le corps n’est qu’un instrument et pour arriver à le parfaire devons chercher la totalité de nous-mêmes, réussir la communication de deux côtés, droit et gauche, aller à la quête d’autres expériences, inimaginables. Les conditions nécessaires sont d’avoir un corps puissant et d’emmagasiner de l’énergie. Tout l’enseignement des sorciers est fondé dans un vrai changement de conception du monde et de la perception de ce monde. L’objectif final est celui de pouvoir choisir sa propre mort ; la vraie, la seule, l’unique liberté de l’homme.

Marine Corps Leadership: Empowering or Limiting the Strategic Corporal?

National Research Council Canada - National Science Library

Pastel, Teague A

2008-01-01

...: instilling character, providing a commitment to lifelong learning, and leadership. Currently, the Marine Corps is carrying out the first two steps well, but is not doing as well with the third step, specifically with respect to command climate...

District heating in Switzerland

International Nuclear Information System (INIS)

Herzog, F.

1991-01-01

District heating has been used in Switzerland for more than 50 years. Its share of the heat market is less than 3% today. An analysis of the use of district heating in various European countries shows that a high share of district heating in the heat market is always dependent on ideal conditions for its use. Market prospects and possible future developments in the use of district heating in Switzerland are described in this paper. The main Swiss producers and distributors of district heating are members of the Association of District Heating Producers and Distributors. This association supports the installation of district heating facilities where ecological, energetical and economic aspects indicate that district heating would be a good solution. (author) 2 tabs., 6 refs

Global Combat Support System-Marine Corps Logistics Chain Management Increment 1 (GCSS-MC LCM Inc 1)

Science.gov (United States)

2016-03-01

Global Combat Support System – Marine Corps (GCSS-MC) is a portfolio of systems that supports logistics elements of command and control, joint...future Marine Corps logistics systems modernization . GCSS-MC/LCM Increment 1 Capability Release 1 (hereinafter Release 1.1) is the baseline

76 FR 36532 - Iberdrola Renewables, Inc., PacifiCorp, NextEra Energy Resources, LLC, Invenergy Wind North...

Science.gov (United States)

2011-06-22

... Renewables, Inc., PacifiCorp, NextEra Energy Resources, LLC, Invenergy Wind North America LLC, Horizon Wind...), Iberdrola Renewables, Inc., PacifiCorp, NextEra Energy Resources, LLC, Invenergy Wind North America LLC, and Horizon Wind Energy LLC (Complainants) filed a formal complaint against Bonneville Power Administration...

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.

78 FR 45010 - In the Matter of Camelot Entertainment Group, Inc., Cavico Corp., Global 8 Environmental...

Science.gov (United States)

2013-07-25

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] In the Matter of Camelot Entertainment Group, Inc., Cavico Corp., Global 8 Environmental Technologies, Inc., GTC Telecom Corp., ICF Corporation, and... Entertainment Group, Inc. because it has not filed any periodic reports since the period ended September 30...

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.

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

The US Army Corps of Engineers Roadmap for Life-Cycle Building Information Modeling (BIM)

Science.gov (United States)

2012-11-01

that have been incorporated into BIM technologies marketed by competing vendors (e.g., Industry Foundation Class [IFC], Construction Operations...ER D C SR -1 2- 2 The US Army Corps of Engineers Roadmap for Life-Cycle Building Information Modeling ( BIM ) En gi ne er R es ea rc h an...Information Modeling ( BIM ) US Army Corps of Engineers Directorate of Civil Works Engineering and Construction Branch Washington, DC 20314-1000 Final

Women in the United States Marine Corps CI/HUMINT Community

National Research Council Canada - National Science Library

Reese, Jackson L

2008-01-01

The purpose of the researchers Masters of Military Arts and Science thesis is to explore the possibility of including women in the Marine Corps CI/HUMINT community without diminishing the overall unit...

20 CFR 670.620 - Are Job Corps students eligible to receive cash allowances and performance bonuses?

Science.gov (United States)

2010-04-01

... Student Support § 670.620 Are Job Corps students eligible to receive cash allowances and performance... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false Are Job Corps students eligible to receive cash allowances and performance bonuses? 670.620 Section 670.620 Employees' Benefits EMPLOYMENT AND...

20 CFR 670.430 - What entities conduct outreach and admissions activities for the Job Corps program?

Science.gov (United States)

2010-04-01

... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false What entities conduct outreach and admissions activities for the Job Corps program? 670.430 Section 670.430 Employees' Benefits EMPLOYMENT AND TRAINING ADMINISTRATION, DEPARTMENT OF LABOR THE JOB CORPS UNDER TITLE I OF THE WORKFORCE INVESTMENT ACT Recruitment...

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.

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...

Evaluation of Cardiovascular Risk Scores Applied to NASA's Astronant Corps

Science.gov (United States)

Jain, I.; Charvat, J. M.; VanBaalen, M.; Lee, L.; Wear, M. L.

2014-01-01

In an effort to improve cardiovascular disease (CVD) risk prediction, this analysis evaluates and compares the applicability of multiple CVD risk scores to the NASA Astronaut Corps which is extremely healthy at selection.

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

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

Empowerment Zones and Enterprise Districts - MDC_CommunityDevelopmentDistrict

Data.gov (United States)

NSGIC Local Govt | GIS Inventory — Community Development Districts (CDDs) are special taxing districts or local units of special-purpose government. A CDD may charge separate non-ad valorem special...

76 FR 55953 - American Capital Partners Limited, Inc., American Educators Financial Corp. (n/k/a Asia Ventures...

Science.gov (United States)

2011-09-09

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] American Capital Partners Limited, Inc., American Educators Financial Corp. (n/k/a Asia Ventures Corp.), Austral Pacific Energy Ltd., Bidville, Inc... lack of current and accurate information concerning the securities of American Capital Partners Limited...

33 CFR 326.3 - Unauthorized activities.

Science.gov (United States)

2010-07-01

... requiring permits, district engineers should make the best use of all available resources. Corps employees... required, the district engineer should consider whether serious jeopardy to life, property, or important... detrimental impacts to the satisfaction of the district engineer. (ii) No permit application will be accepted...

The youth conservation corps and adolescents' self-concept

Science.gov (United States)

A. Laverne Dickerson

1977-01-01

Four dimensions of self-concept were measured at the beginning and the end of the Youth Conservation Corps's 1971 pilot program. The overall evaluation of the program had found no changes in self-concept, but factor analysis identified some significant differences. The campers' perceptions of their adequacy decreased, while their perceptions of their personal...

District nurse training

OpenAIRE

Elliott, Arnold; Freeling, Paul; Owen, John

1980-01-01

Training for district nursing is being reviewed. By 1981 district nurses will have a new administrative structure, a new curriculum, and a new examination. Training for nursing, like that for general practice, is to become mandatory. The history of the development of district nurse training is briefly described.

Steps Towards Determining the Right Number of Dental Recruits the Navy Should Access to Meet the Projected Targets for Navy Dental Corps Officers

Science.gov (United States)

2007-03-01

Periodontics , Oral Surgery, and Prosthodontics. 1. Overall Dental Corps The data included for the overall loss rate for the Dental Corps included...Endodontists. This may occur due to the pay disparity between military and civilian Oral Surgeons. The Dental Corps attempts to mitigate the loss ...gum disease . Periodontics is another specialty that Dental Corps officers can decide to practice. NNMC, Naval Postgraduate Dental School offers a

Army Corps of Engineers: Actions Needed to Improve Cost Sharing for Dam Safety Repairs

Science.gov (United States)

2015-12-01

agreements with the Corps, their history of being a sponsor, the financial impacts of cost sharing for dam safety repair projects, and the Corps...1240 (2007)) and Beaver Lake dam, AR (Pub. L. No. 102-377, 106 Stat. 1315, 1318 (1992), Pub. L. No. 102-580, § 209(f), 106 Stat. 4797, 4830 (1992...inaction in setting a clear policy for a provision under which sponsors face significant financial impacts has contributed to conditions under

75 FR 26822 - In the Matter of: Universal Property Development & Acquisition Corp.; Order of Suspension of Trading

Science.gov (United States)

2010-05-12

... Universal Property Development & Acquisition Corp. (``Universal Property'') because it has not filed any... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] In the Matter of: Universal Property Development & Acquisition Corp.; Order of Suspension of Trading May 10, 2010. It appears to the Securities and...

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.)

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...

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.

Information, Vol. 1, Number 4. Teacher Corps Dissemination Project Bulletin.

Science.gov (United States)

Rosenau, Fred S., Ed.

Guidelines are provided for disseminating information on teacher corps projects. Information is given on experienced disseminators such as existing networks that are available to help in planning. Suggestions are made on targeting information and marketing. (JD)

Bat Habitat Restoration and Management Opportunities on Corps of Engineers Projects

National Research Council Canada - National Science Library

2000-01-01

.... Although bats have not traditionally been addressed in Corps of Engineers planning and natural resources management efforts, the recent focus on ecosystem management and inclusion of nongame species...

32 CFR 765.14 - Unofficial use of the seal, emblem, names, or initials of the Marine Corps.

Science.gov (United States)

2010-07-01

... create the impression that the Marine Corps or the United States is in any way responsible for any financial or legal obligation of the user; (iii) Give the impression that the Marine Corps selectively... to the Director, Administration Resource Management (ARDE). Prior to granting approval for commercial...

The Marine Corps' infantry selection and assignment process: is it ready for gender neutrality?

OpenAIRE

Stooksbury, William Thorpe.

1994-01-01

Approved for public release; distribution is unlimited Opportunities for women in the military continue to expand. At some point in the future, the Marine Corps may be compelled to address the assimilation of women into combat arms occupational specialties. Existing high attrition rates in the Marine Corps' infantry specialties indicate a flawed selection and assignment process resulting in a poor fit of the person to the job. Because of gender differences, this problem will be magnified i...

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.

Financial Management: U.S. Army Corps of Engineers Financial Information Imported Into the Defense Departmental Reporting System - Audited Financial Statements

National Research Council Canada - National Science Library

Granetto, Paul J; Peek, Marvin L; Armstrong, Jack L; Wenzel, Paul C; Furey, Kathleen A; Zimmerman, Craig W

2004-01-01

... are: the Corps of Engineers Financial Management System, the Corps of Engineers Enterprise Management Information System, and the Defense Departmental Reporting System - Audited Financial Statements...

Infiltration Control Landfill Cover Demonstration at Marine Corps Base, Hawaii

National Research Council Canada - National Science Library

Karr, Leslie

1999-01-01

.... Demonstration caps were installed at Marine Corps Base Hawaii (MCBH) Kaneohe Bay in 1994. The study used an innovative but simple concept to manipulate the fate of rain water falling on waste sites with moderate to high precipitation...

Oxidative Stress and Huntington's Disease: The Good, The Bad, and The Ugly.

Science.gov (United States)

Kumar, Amit; Ratan, Rajiv R

2016-10-01

Redox homeostasis is crucial for proper cellular functions, including receptor tyrosine kinase signaling, protein folding, and xenobiotic detoxification. Under basal conditions, there is a balance between oxidants and antioxidants. This balance facilitates the ability of oxidants, such as reactive oxygen species, to play critical regulatory functions through a direct modification of a small number of amino acids (e.g. cysteine) on signaling proteins. These signaling functions leverage tight spatial, amplitude, and temporal control of oxidant concentrations. However, when oxidants overwhelm the antioxidant capacity, they lead to a harmful condition of oxidative stress. Oxidative stress has long been held to be one of the key players in disease progression for Huntington's disease (HD). In this review, we will critically review this evidence, drawing some intermediate conclusions, and ultimately provide a framework for thinking about the role of oxidative stress in the pathophysiology of HD.

Cognitive and behavioral changes in Huntington disease before diagnosis.

Science.gov (United States)

Paulsen, Jane S; Miller, Amanda C; Hayes, Terry; Shaw, Emily

2017-01-01

Phenotypic manifestations of Huntington disease (HD) can be detected at least 15 years prior to the time when a motor diagnosis is given. Advances in clinical care and future research will require consistent use of HD definitions and HD premanifest (prodromal) stages being used across clinics, sites, and countries. Cognitive and behavioral (psychiatric) changes in HD are summarized and implications for ongoing advancement in our knowledge of prodromal HD are suggested. The earliest detected cognitive changes are observed in the Symbol Digit Modalities Test, Stroop Interference, Stroop Color and Word Test-interference condition, and Trail Making Test. Cognitive changes in the middle and near motor diagnostic stages of prodromal HD involve nearly every cognitive test administered and the greatest changes over time (i.e., slopes) are found in those prodromal HD participants who are nearest to motor diagnosis. Psychiatric changes demonstrate significant worsening over time and remain elevated compared with healthy controls throughout the prodromal disease course. Psychiatric and behavior changes in prodromal HD are much lower than that obtained using cognitive assessment, although the psychiatric and behavioral changes represent symptoms most debilitating to independent capacity and wellness. Copyright © 2017 Elsevier B.V. All rights reserved.

Kazakh: Language Competencies for Peace Corps Volunteers in Kazakhstan.

Science.gov (United States)

Cirtautas, Ilse

The text is designed for classroom and self-study of Kazakh by Peace Corps volunteers training to serve in Kazakhstan. It consists of language and culture lessons on 13 topics: personal identification; classroom communication; conversation with a host counterpart or family; general communication; food; money; transportation; getting and giving…

Air-Land Battle Interdiction Model Corps Communications Module.

Science.gov (United States)

1984-06-01

communication network’s strengths and weaknesses. This method will closely emulate the ccmmunications obstacles that face V Corps in the Fulda Eowl area. (see...and the Dinamic Perfcince 0~ ~r niU~IT-onal -- DeIs, Y ’!7STmfof3 Ui’Versity 5i nt T ors 3r,7U1 8 4-.- *13. Weissinger-Baylcn, Grtage Can Decision

76 FR 67174 - Arkansas Electric Cooperative Corp., Riverbank Hydro No. 9 LLC, Solia 3 Hydroelectric LLC, Lock...

Science.gov (United States)

2011-10-31

...; 14166-000; 14180-000; 14193-000] Arkansas Electric Cooperative Corp., Riverbank Hydro No. 9 LLC, Solia 3... May 2, 2011, Arkansas Electric Cooperative Corp. (Arkansas Electric), Riverbank Hydro No. 9 LLC.... Krouse, Hydro Green Energy, 5090 Richmond Avenue 390, Houston, TX 77056. (877) 556-6566 x 709. FFP 2's...

20 CFR 670.480 - At what point is an applicant considered to be enrolled in Job Corps?

Science.gov (United States)

2010-04-01

... 20 Employees' Benefits 3 2010-04-01 2010-04-01 false At what point is an applicant considered to be enrolled in Job Corps? 670.480 Section 670.480 Employees' Benefits EMPLOYMENT AND TRAINING ADMINISTRATION, DEPARTMENT OF LABOR THE JOB CORPS UNDER TITLE I OF THE WORKFORCE INVESTMENT ACT Recruitment...

7 CFR 917.14 - District.

Science.gov (United States)

2010-01-01

... District. (g) Contra Costa District includes and consists of Contra Costa County. (h) Santa Clara District... Ventura County. (l) Stockton District includes and consists of San Joaquin County, Amador County...

Care of patients with Huntington's disease in South America: a survey

Directory of Open Access Journals (Sweden)

Ricardo Oliveira Horta Maciel

2013-06-01

Full Text Available Huntington's disease (HD is a rare neurodegenerative disease with a multitude of symptoms, which requires access to specialized multidisciplinary care for adequate management. The aim of this study was to survey the characteristics of care in various HD centers in South America (SA. Methods A questionnaire was sent to 24 centers involved in the care for HD patients in SA. Results Of the total 24 centers, 19 (79.2% are academic units. The majority of centers (62.5% are general movement disorders clinics. Multidisciplinary care is available in 19 (79.2% centers and in 20 (83.3% care is provided free of charge. Genetic testing and counseling are available in 25 and 66.6% of centers, respectively. The majority of centers (83.3% have no institutional support for end-stage care. Conclusions Although HD centers in SA are committed to providing multidisciplinary care, access to genetic counseling and end-stage care are lacking in most centers.

Is gardening a stimulating activity for people with advanced Huntington's disease?

Science.gov (United States)

Spring, Josephine A; Viera, Marc; Bowen, Ceri; Marsh, Nicola

2014-11-01

This study evaluated adapted gardening as an activity for people with advanced Huntington's disease (HD) and explored its therapeutic aspects. Visitors and staff completed a questionnaire and participated in structured interviews to capture further information, whereas a pictorial questionnaire was designed for residents with communication difficulties. Staff reported that gardening was a constructive, outdoor activity that promoted social interaction, physical activity including functional movement and posed cognitive challenges. Half the staff thought the activity was problem free and a third used the garden for therapy. Visitors used the garden to meet with residents socially. Despite their disabilities, HD clients enjoyed growing flourishing flowers and vegetables, labelling plants, being outside in the sun and the quiet of the garden. The garden is valued by all three groups. The study demonstrates the adapted method of gardening is a stimulating and enjoyable activity for people with advanced HD. © The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

Gardening with Huntington's disease clients--creating a programme of winter activities.

Science.gov (United States)

Spring, Josephine Anne; Baker, Mark; Dauya, Loreane; Ewemade, Ivie; Marsh, Nicola; Patel, Prina; Scott, Adrienne; Stoy, Nicholas; Turner, Hannah; Viera, Marc; Will, Diana

2011-01-01

A programme of garden-related indoor activities was developed to sustain a gardening group for people with mid to late stage Huntington's disease during the winter. The activities were devised by the horticulturist, working empirically, involving the services occupational therapist, physiotherapist, occupational therapy art technician, computer room, recreation and leisure staff. The programme was strongly supported by the nursing and care staff. Feedback on the effectiveness of the activities was sought from the clients, team members and unit staff. The clients' interest in gardening was sustained by a multidisciplinary programme of indoor growing and using plant products in creative activities, computing and group projects. The clients enjoyed all activities except one that they said lacked contact with plants. The inexpensive programme of activities enabled creativity and self-expression, stimulated social contact and helped with therapeutic goals of the clients. In addition, it engaged the multi-disciplinary team and the unit staff, was practical and enhanced the environment.

Guarani I and Work Book (For Peace Corps Volunteers).

Science.gov (United States)

Peace Corps (Paraguay).

This workbook is designed for the Guarani language training of Peace Corps volunteers in Paraguay, and the content focuses on daily communication needs in that context. The workbook contains nine thematic instructional units based on performance objectives. A brief introduction gives an overview of Guarani's origins, distribution, and phonology.…

AHP 40: AT HOME IN THE WORLD: GLOBALIZATION AND THE PEACE CORPS IN NEPAL

Directory of Open Access Journals (Sweden)

Sienna Craig

2016-02-01

Full Text Available It is something of a truism - if not a cliché - that cross-cultural encounters of the kind epitomized by the United States Peace Corps program can be life-changing experiences. Immersion into landscapes, languages, and cultures half a world away from where one is brought up cannot help but be transformative. Some flourish under the disorienting - or reorienting - spell of culture shock and emerge perhaps less certain about the world but more enamored of it. Others find such experiences tumultuous, sometimes making a hasty retreat back to familiar geographies or making it through these experiences but opting not to repeat them. In the case of James Fisher, his deep dive into the peoples and cultures of the Himalaya as part of Peace Corps 1 in Nepal (1962-1964 shaped not only his views of the world but also his professional trajectory in profound ways. Peace Corps was Fisher's gateway into a life of ethnographic exploration, teaching, and anthropological collaborations stemming half a century. ...

The Impact and Role of the Yi Civil Corps on Ethnic Work in Liangshan

Institute of Scientific and Technical Information of China (English)

MA Linying

2013-01-01

The formation and growth of Yi Civil Corps of the CPLA ( Chinese People ’ s Lib-eration Army ) Chengdu military region began and developed with the establishment of the Liangshan Yi Autonomous Prefecture .From the beginning to the end of its 34 year mission of military develop-ment the Yi Civil Corps made an irreplaceable con-tribution to the liberation of the people and the e-conomic development in Liangshan .However , due to various reasons , this unique historical episode which linked the military and civil people only re-mains in folk memory and was recorded only in some unpublished internal materials .So, the ma-jority of the public does not know about it .Based upon references taken from publications by Liang Wenying , Mao Junru , Li Shian and the Chinese Academy of Sciences , this article , for the first time, discusses the special military history of the CPLA by taking Yi Civil Corps as an example . After the CPLA liberated Liangshan in March , 1950 , in order to maintain local social or-der, speed up production and construction , and cultivate ethnic cadres , Liao Zhigao , the Party secretary of Sichuan province of the time , together with the Party Committee in Tibetan and Yi re-gions, established the Yi and Tibetan Civil Corps of the CPLA in November 1952.They did this by taking into account the reality and characteristics of ethnic works in the Tibetan and Yi regions , and af-ter reporting to the central military commission for approval .The establishment of Yi Civil Corps adds a new historical page for the glorious history of the Yi people .Just like the establishment of all levels of governments of the Yi people , it marks a new beginning in the lives of the Yi people .It declared a revival for the Yi people who had experienced great vicissitudes , the separation of their family clans, constant internal warfare , and who were generally in a poor and backward situation .The establishment of the Yi Civil Corps not only em-bodied the Chinese Communist

Legislative Districts, Four layers - One State Assembly district, one State Senate district, one US House of Rep district, and one US Senate district showing the locations in the County of Polk, WI., Published in 2007, 1:24000 (1in=2000ft) scale, Polk County Government.

Data.gov (United States)

NSGIC Local Govt | GIS Inventory — Legislative Districts dataset current as of 2007. Four layers - One State Assembly district, one State Senate district, one US House of Rep district, and one US...

76 FR 32332 - BASF Corp.; Filing of Food Additive Petition (Animal Use); Methyl Esters of Conjugated Linoleic...

Science.gov (United States)

2011-06-06

.... FDA-2011-F-0365] BASF Corp.; Filing of Food Additive Petition (Animal Use); Methyl Esters of... petition proposing that the food additive regulations be amended to provide for the safe use of methyl... is given that a food additive petition (FAP 2269) has been filed by BASF Corp. (BASF), 100 Campus Dr...

42 CFR 23.10 - Under what circumstances may a National Health Service Corps site's reimbursement obligation to...

Science.gov (United States)

2010-10-01

... Service Corps site's reimbursement obligation to the Federal Government be waived? 23.10 Section 23.10... National Health Service Corps site's reimbursement obligation to the Federal Government be waived? (a) The Secretary may waive in whole or in part the reimbursement requirements of section 334(a)(3) of the Act if he...

State and district policy influences on district-wide elementary and middle school physical education practices.

Science.gov (United States)

Chriqui, Jamie F; Eyler, Amy; Carnoske, Cheryl; Slater, Sandy

2013-01-01

To examine the influence of state laws and district policies on district-wide elementary school and middle school practices related to physical education (PE) time and the percentage of moderate-to-vigorous physical activity (MVPA) time during PE. Multivariate, cross-sectional analysis of state laws, district wellness and PE policies, and district PE practices for school year 2010-2011 controlling for district-level urbanicity, region, size, race/ethnicity of students, and socioeconomic status and clustered on state. One hundred ninety-five public school districts located in 42 states. District-level PE coordinators for the included districts who responded to an online survey. Minutes and days of PE per week and percent time spent in MVPA during PE time. District PE coordinators reported significantly less PE time than national standards-82.9 and 189.6 minutes at the elementary school and middle school levels, respectively. Physical education was provided an average of 2.5 and 3.7 days per week, respectively; and the percentage of MVPA time in PE was 64.4% and 65.7%, respectively. At the elementary school level, districts in either states with laws governing PE time or in a state and district with a law/policy reported significantly more days of PE (0.63 and 0.67 additional days, respectively), and districts in states with PE time laws reported 18 more minutes of PE per week. At the middle school level, state laws were associated with 0.73 more days of PE per week. Neither state laws nor district policies were positively associated with percent MVPA time in PE. State laws and district policies can influence district-level PE practices-particularly those governing the frequency and duration of PE-although opportunities exist to strengthen PE-related laws, policies, and practices.

33 CFR 385.5 - Guidance memoranda.

Science.gov (United States)

2010-07-01

... ability of the Corps of Engineers, the South Florida Water Management District, and other non-Federal... § 385.5 Guidance memoranda. (a) General. (1) Technical guidance for internal management of Corps of...) General format and content of Project Implementation Reports (§ 385.26(a)); (ii) Instructions for...

A Rebuttal to the 2010 Marine Corps Operating Concept

Science.gov (United States)

2013-05-23

Charles. “Combat in Nicaragua.” Marine Corps Gazette Vol. 92, No. 4 (April 2008): 71-76. Schlosser, Nicholas J. “The Marine Corps’ Small Wars Manual... Biddle , Stephen and Jeffrey A. Friedman. The 2006 Lebanon Campaign And The Future Of Warfare: Implications For Army and Defense Policy. U.S. Army War

Kreol: A Language Training Program for Peace Corps-Seychelles.

Science.gov (United States)

Mancienne, Roger

A course in French-derived Kreol is designed for language learning among Peace Corps volunteers assigned to the Seychelles. The materials consist of 36 lessons on topics of daily communication needs. Lessons contain a story, dialogue, and/or list of expressions, exercises, and vocabulary and grammar notes and translations in the margin. Some…

U.S. Naval Sea Cadet Corps 2009 Annual Report

Science.gov (United States)

2009-01-01

for the Naval Sea Cadet Corps. I salute your service to our Nation in developing future leaders. Bravo Zulu and keep charging! ttr’"-- U.S...Minutemen, NLCC, sponsored by Channel Isles Council, NLUS, CA. The NSCC Hall of Fame Award: Awarded to a founder or volunteer U.S. Naval Sea Cadet

Perceived influence of the National Youth Service Corps (NYSC ...

African Journals Online (AJOL)

A significant relationship existed between respondents' economic background and their perception of NYSC (X2 = 0.235, p<0.05). The study concluded that the influence of the NYSC scheme on the development of the youth corps members was perceived to be poor by majority of the respondents. While several calls for ...

78 FR 2287 - Daimler Buses North America, Inc., a Subsidiary of Daimler North America Corp, Including On-Site...

Science.gov (United States)

2013-01-10

... subsidiary of Daimler North America Corp., including on-site leased workers from Noramtec, First Choice... America, Inc., a Subsidiary of Daimler North America Corp, Including On-Site Leased Workers From Noramtec, First Choice Staffing, Staff Works, and Mr. Santo Lamarco From Wurth Revcar Fasteners, Inc., Oriskany...

75 FR 25887 - Order of Suspension of Trading; In the Matter of Alyn Corp., American HealthChoice, Inc...

Science.gov (United States)

2010-05-10

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] Order of Suspension of Trading; In the Matter of Alyn Corp., American HealthChoice, Inc., American Holding Investments, Inc., American Midland Corp... Amwest Environmental Group, Inc. May 6, 2010. It appears to the Securities and Exchange Commission that...

Analysis of Navy Supply Corps Lines of Operation

Science.gov (United States)

2017-12-01

Expeditionary Combat Support FM Financial Management FM Field Manual HCS Department of Defense Logistics Human Capital Strategy JCS Joint Chiefs of Staff...allocation, control, and execution of resources and funds for DOD organizations” ( Office of Supply Corps Personnel, 2011, p. 9) and that Financial ...Information Operations and Reports, 1215 Jefferson Davis Highway, Suite 1204, Arlington, VA 22202-4302, and to the Office of Management and Budget

INDUSTRIE ET ENVIRONNEMENT Les préoccupations environnementales de l’industrie des corps gras

Directory of Open Access Journals (Sweden)

Pages Xavier

2002-09-01

Full Text Available Les préoccupations environnementales de l’industrie des corps gras sont principalement rythmées par le renforcement des contraintes réglementaires et concernent tous les aspects environnementaux : effluents gazeux et aqueux, déchets, management, énergie. Ces aspects sont inégalement présents dans les différents sous-secteurs de l’industrie des corps gras ; quelques exemples sont présentés. La réduction des émissions de COV (composés organiques volatils et les nuisances olfactives sont une problématique concernant principalement l’activité de trituration, la lipochimie et la fonte de corps gras animaux. Des valeurs limites de consommation de solvant sont maintenant imposées réglementairement en extraction; la prise en compte de problèmes de nuisance olfactive donnent lieu à l’amélioration des procédés de traitement d’air (corps gras animaux ou à leur mise en place progressive (trituration de colza. La gestion des déchets reste une préoccupation essentielle pour l’ensemble de la profession et tout particulièrement pour les secteurs des corps gras animaux et la lipochimie, en raison de la remise en cause des filières d’élimination due au renforcement des contraintes réglementaires, mais aussi en raison des coûts de traitement. Ce poste ne doit pas être négligé par les entreprises : réduction à la source, recherche de filières pérennes, valorisation énergétique ou matière sont des éléments permettant également une réduction significative des coûts. La maîtrise de la consommation d’énergie est un élément important, voir stratégique, notamment dans le secteur de la trituration des graines oléagineuses. Des actions de sensibilisation permettent de poursuivre également l’effort auprès des PME, actuellement moins impliquées. En matière de traitement d’eau, des avancées notables sont observées depuis une décennie ; néanmoins certaines difficultés persistent, notamment dans les

California Political Districts

Data.gov (United States)

California Natural Resource Agency — This is a series of district layers pertaining to California'spolitical districts, that are derived from the California State Senateand State Assembly information....

District nursing in Dominica

NARCIS (Netherlands)

Kolkman, PME; Luteijn, AJ; Nasiiro, RS; Bruney, [No Value; Smith, RJA; Meyboom-de Jong, B

1998-01-01

District nurses constitute the basis of the primary health care services in Dominica. All encounters of three district nurses were registered using the international classification of primary care. Information on other aspects of district nursing was collected by participating observation and the

A Guide to Cultural and Environmental Interpretation in the U.S. Army Corps of Engineers.

Science.gov (United States)

1981-08-01

34 (Veverka 1978a, Blahna and Roggenbuck 1979). Although this belongingness or affilia- tion motive appears to be a strong one, these same researchers note that...with only one park or recreation area and to rarely see a Corps employee . In these cases, it may be necessary to use such per- sonal services as roving...more of the specifics of conducting guided tours, Corps interpreters are urged to consult: "Conducted Trips - A Training Bul- letin for Field Employees

Private Water Districts

Data.gov (United States)

California Natural Resource Agency — Private Water District boundaries are areas where private contracts provide water to the district in California. This database is designed as a regions polygon...

Metaphor and embodied cognition Metáfora e cognição corpórea

Directory of Open Access Journals (Sweden)

Raymond W. Gibbs Jr

2010-01-01

Full Text Available The present paper briefly describes recent advances in cognitive science on the embodied nature of human cognition with the aim to better situating contemporary work on embodied metaphor in language and thought. We do this by talking about key experimental findings in five areas main areas of research in cognitive science: perception, concepts, mental imagery, memory, and language processing (Gibbs 2006a We also describe some psycholinguistic studies on embodied metaphor understanding, and offer some details on one series of experiments in regard to people's embodied understanding of the DIFFICULTIES ARE WEIGHTS primary metaphor. Our conclusion draws connections between the research on embodied cognition and contemporary linguistic and psychological work on embodied metaphor.O presente trabalho descreve sucintamente avanços na ciência cognitiva a respeito da natureza corpórea da cognição humana com o objetivo de melhor situar o trabalho contemporâneo sobre metáforas corpóreas na linguagem e no pensamento. Fazemos isso por discutir achados de experimentos centrais em cinco áreas principais de pesquisa em ciência cognitiva: percepção, conceitos, imagem mental, memória e processamento da linguagem (Gibbs 2006a. Descrevemos também alguns estudos psicolinguísticos sobre a compreensão de metáforas corpóreas e detalhamos uma série de experimentos desenhados para investigar a compreensão corporificada de pessoas a respeito da metáfora primária DIFICULDADES SÃO PESOS. Nossa conclusão busca pontos de interesse comum entre a pesquisa sobre cognição corpórea e trabalhos na área da lingüística e psicologia sobre metáfora corpórea.

Improving the Marine Corps Reserve Infantry Battalion: Manning, Training, Integration, and Retention

National Research Council Canada - National Science Library

Parrish, Ross A

2008-01-01

.... Recognizing that all Marine Corps reserve infantry battalions have deployed at least once in support of the GWOT, further improvements in the manning, training, integration, and retention within...

State Water Districts

Data.gov (United States)

California Natural Resource Agency — State Water Project District boundaries are areas where state contracts provide water to the district in California. This database is designed as a regions polygon...

76 FR 45249 - Fairlawn Hydroelectric Company, LLC; Notice of Public Meeting

Science.gov (United States)

2011-07-28

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Project No. 12715-003] Fairlawn Hydroelectric Company, LLC; Notice of Public Meeting On August 5, 2011, Office of Energy Projects staff may participate in a public meeting hosted by the U.S. Army Corps of Engineers, Baltimore District (Corps) for the...

Evaluation of Biodiesel Fuels to Reduce Fossil Fuel Use in Corps of Engineers Floating Plant Operations

Science.gov (United States)

2016-07-01

ER D C/ CH L TR -1 6- 11 Dredging Operations and Environmental Research Program Evaluation of Biodiesel Fuels to Reduce Fossil Fuel Use... Fuels to Reduce Fossil Fuel Use in Corps of Engineers Floating Plant Operations Michael Tubman and Timothy Welp Coastal and Hydraulics Laboratory...sensitive emissions, increase use of renewable energy, and reduce the use of fossil fuels was conducted with funding from the U.S. Army Corps of

77 FR 67345 - Final Environmental Impact Statement/Environmental Impact Report (EIS/EIR) for the Clearwater...

Science.gov (United States)

2012-11-09

...The U.S. Army Corps of Engineers (Corps) in conjunction with the Sanitation Districts of Los Angeles County (Sanitation Districts) has completed a Final Environmental Impact Statement/Environmental Impact Report (EIS/EIR) for the Clearwater Program. The Clearwater Program is a comprehensive planning effort undertaken by the Sanitation Districts for the Joint Outfall System, a regional wastewater management system serving approximately 4.8 million people in 73 cities and unincorporated areas in Los Angeles County. A major component of the Clearwater Program is the evaluation of alternatives for construction of a new ocean outfall and rehabilitation of the existing ocean outfalls. Both activities would entail discharge of dredged and fill material in waters of the United States, work in navigable waters of the United States, and the transport of dredged material for ocean disposal. These activities would require authorization from the Corps pursuant to Section 404 of the Clean Water Act, Section 10 of the Rivers and Harbors Act, and Section 103 of the Marine Protection, Research, and Sanctuaries Act, respectively. The Draft EIS/EIR was circulated for a 57-day review period from February 13, 2012 through April 10, 2012. The Corps and the Sanitation Districts reviewed and provided responses to 19 agency comments and 33 public comments in preparing the Final EIS/EIR. The Final EIS/EIR, including a Draft 404(b)(1) alternatives analysis, is available for a 31-day review period from November 9, 2012 through December 10, 2012. The document is accessible via the World- Wide Web at www.ClearwaterProgram.org. Alternatively, printed copies are available at the following locations: Sanitation Districts of Los Angeles County, 1955 Workman Mill Road, Whittier, California; Carson Regional Library, 151 East Carson Street, Carson, California; Los Angeles Public Library, San Pedro Branch, 931 South Gaffey Street, San Pedro, California; and Los Angeles Public Library, Wilmington

Huntington's disease-like and ataxia syndromes: identification of a family with a de novo SCA17/TBP mutation

DEFF Research Database (Denmark)

Bech, Sara; Petersen, Thor; Nørremølle, Anne

2010-01-01

The autosomal dominant spinocerebellar ataxias, commonly referred to as SCAs, are clinically and genetically heterogeneous neurodegenerative disorders. Twenty-eight genetic subtypes have been identified, of which 7 are caused by expansion of a CAG trinucleotide repeat that encodes a polyglutamine....... The patient's mother and father both carried normal range repeats, 38/38 and 33/39 respectively. Analysis of the repeat structures revealed that the expansion had occurred upon expansion of the longer paternal allele. We conclude that, however rare, SCA17 must be considered as a cause of Huntington's disease...

Analysis of the Marine Corps Educational Tier System

Science.gov (United States)

2013-03-01

Joint Advertising , Market Research and Studies MCRC Marine Corps Recruiting Command MCT Marine Combat Training MLE Maximum Likelihood...Minorities in the Enlisted Force: Is the U.S. Military Representative of the Nation It Defends?, 23. 49 Ibid. 50 Davis Armour and Curtis L. Gilroy...among Hispanics, the rate dropped from 49 percent to 32 percent.52 As shown in Figure 10, a Joint Advertising , Market Research and Studies (JAMRS) poll

Características del habla, el lenguaje y la deglución en la enfermedad de Huntington

OpenAIRE

Camargo-Mendoza, Maryluz; Castillo-Triana, Nicolás; Fandiño-Cardona, Juan Miguel; Mateus-Moreno, Angélica; Moreno-Martínez, Mariana

2017-01-01

Resumen La enfermedad de Huntington (EH) ha sido descrita como una afección de causa genética producida por una mutación en la repetición de la secuencia de nucleótidos CAG (citosina-adenina-guanina). Según el estadio que curse la enfermedad, las personas pueden presentar dificultades en el habla, el lenguaje y la deglución. El propósito de este artículo es exponer con detalle dichas dificultades, así como su tratamiento fonoaudiológico. Se destaca que en el habla se encuentran característica...

75 FR 29365 - Job Corps: Final Finding of No Significant Impact (FONSI) for Small Wind Turbine Installation at...

Science.gov (United States)

2010-05-25

... (FONSI) for Small Wind Turbine Installation at the Pine Ridge Job Corps Center Located at 15710 Highway... Finding of No Significant Impact (FONSI) for Small Wind Turbine Installation at the Pine Ridge Job Corps....11(d), gives final notice of the proposed construction of a small wind turbine at the Pine Ridge Job...

Improving job performance of Neighborhood Youth Corps aides in an urban recreation program1

Science.gov (United States)

Pierce, Charles H.; Risley, Todd R.

1974-01-01

In most federal job training and employment programs, trainees' pay is not contingent on job performance, but upon physical presence. This study sought to increase the job performance of seven Neighborhood Youth Corps workers being paid an hourly wage for serving as aides in an urban recreation program. When thorough job descriptions and threatened termination of employment were insufficient to maintain adequate job performance, an attempt was made to make the hourly wage (required by the Neighborhood Youth Corps program) more contingent on job performance. When the number of hours credited the workers on their payroll sheets was proportional to their rating on a simple checklist of job performance, rather than to the number of hours they were present, their job performance was maintained at near-perfect levels. Although this simple semantic shift in emphasis—from “hours worked” to “hours worked”—was still interpreted as meeting the Neighborhood Youth Corps requirements for hourly pay, its behavioral effects were substantial. This simple procedure might be used in other training programs handicapped by hourly wage requirements. PMID:4436168

Constitutive upregulation of chaperone-mediated autophagy in Huntington's disease.

Science.gov (United States)

Koga, Hiroshi; Martinez-Vicente, Marta; Arias, Esperanza; Kaushik, Susmita; Sulzer, David; Cuervo, Ana Maria

2011-12-14

Autophagy contributes to the removal of prone-to-aggregate proteins, but in several instances these pathogenic proteins have been shown to interfere with autophagic activity. In the case of Huntington's disease (HD), a congenital neurodegenerative disorder resulting from mutation in the huntingtin protein, we have previously described that the mutant protein interferes with the ability of autophagic vacuoles to recognize cytosolic cargo. Growing evidence supports the existence of cross talk among autophagic pathways, suggesting the possibility of functional compensation when one of them is compromised. In this study, we have identified a compensatory upregulation of chaperone-mediated autophagy (CMA) in different cellular and mouse models of HD. Components of CMA, namely the lysosome-associated membrane protein type 2A (LAMP-2A) and lysosomal-hsc70, are markedly increased in HD models. The increase in LAMP-2A is achieved through both an increase in the stability of this protein at the lysosomal membrane and transcriptional upregulation of this splice variant of the lamp-2 gene. We propose that CMA activity increases in response to macroautophagic dysfunction in the early stages of HD, but that the efficiency of this compensatory mechanism may decrease with age and so contribute to cellular failure and the onset of pathological manifestations.

Huntington's disease in Greece: the experience of 14 years.

Science.gov (United States)

Panas, M; Karadima, G; Vassos, E; Kalfakis, N; Kladi, A; Christodoulou, K; Vassilopoulos, D

2011-12-01

A large scale genetic and epidemiological study of Huntington's disease (HD) was carried out in Greece from January 1995 to December 2008. Diagnostic testing was carried out in 461 symptomatic individuals, while 256 were tested for presymptomatic purposes. The diagnosis of HD with a CAG expansion ≥ 36 was confirmed in 278 symptomatic individuals. The prevalence of HD in Greece was estimated at approximately 2.5 to 5.4:100,000, while the mean minimum incidence was estimated at 2.2 to 4.4 per million per year. The molecular diagnosis of HD was confirmed in the majority of patients (84.4%) sent for confirmation. The false-positive cases 15.6% were characterized by the absence of a family history of HD and the presence of an atypical clinical picture. The uptake of predictive testing for HD was 8.6%. A prenatal test was requested in six pregnancies. The findings of our study do not differ significantly from those of similar studies from other European countries despite the relative genetic isolation of Greece. Of interest is the identification of clusters of HD in Greece. The presence or absence of a family history of HD should be interpreted cautiously, during the diagnostic process. © 2010 John Wiley & Sons A/S.

Reduction of IFNα and IL-10 in central nervous system and increase in peripheral IL-8 in transgenic porcine Huntington´s disease model

Czech Academy of Sciences Publication Activity Database

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

2015-01-01

Roč. 78, Suppl 2 (2015), s. 10-11 ISSN 1210-7859. [Conference on Animal Models for neurodegenerative Diseases /3./. 08.11.2015-10.11.2015, Liblice] R&D Projects: GA MŠk ED2.1.00/03.0124 Institutional support: RVO:67985904 Keywords : porcine Huntington ´s disease model * IFNα * IL-10 Subject RIV: EB - Genetics ; Molecular Biology

76 FR 11933 - 50th Anniversary of the Peace Corps

Science.gov (United States)

2011-03-03

... address changing and complex global needs in education, health and HIV/AIDS, business and information... America sees the world and the world sees us. Today, one of President Kennedy's most enduring legacies can... on. In our increasingly interconnected world, the mission of the Peace Corps is more relevant today...

John A. Lejeune, The Marine Corps’ Greatest Strategic Leader

Science.gov (United States)

2008-03-25

ranks, such as Brigadier General Smedley Butler, who would have been happy with a Commandant who focused on these traditional missions of the Corps...World War II,” in Command Decisions, ed. Kent Roberts Greenfield (Washington D.C.: Center of Military History United States Army, 1960), 14. 13

28 CFR 92.5 - What educational expenses does the Police Corps cover, and how will they be paid?

Science.gov (United States)

2010-07-01

... 28 Judicial Administration 2 2010-07-01 2010-07-01 false What educational expenses does the Police Corps cover, and how will they be paid? 92.5 Section 92.5 Judicial Administration DEPARTMENT OF JUSTICE... Criteria § 92.5 What educational expenses does the Police Corps cover, and how will they be paid? (a...

Zero Energy Districts

Energy Technology Data Exchange (ETDEWEB)

Polly, Benjamin J [National Renewable Energy Laboratory (NREL), Golden, CO (United States)

2018-05-04

This presentation shows how NREL is approaching Zero Energy Districts, including key opportunities, design strategies, and master planning concepts. The presentation also covers URBANopt, an advanced analytical platform for district that is being developed by NREL.

BIOPHYSICAL EVALUATION OF INDIVIDUAL COMPONENT LEVELS AND SELECTED CONFIGURATIONS OF THE UNITED STATES MARINE CORPS COLD-WEATHER CLOTHING ENSEMBLE

Science.gov (United States)

2018-01-02

Marine Corps Clothing and Equipment Team, Product Manager Infantry Combat Equipment, Marine Corps Systems Command Natick Soldier Research Design...10 Table 5. Total thermal resistance for recommended configurations .............................. 10 Table 6. Total evaporative resistance...for recommended configurations ....................... 11 Table 7. Total resistance values of headwear items

Care of patients with Huntington's disease in South America: a survey Cuidado de pacientes com doença de Huntington na América do Sul: um inquérito

Directory of Open Access Journals (Sweden)

Ricardo Oliveira Horta Maciel

2013-06-01

Full Text Available Huntington's disease (HD is a rare neurodegenerative disease with a multitude of symptoms, which requires access to specialized multidisciplinary care for adequate management. The aim of this study was to survey the characteristics of care in various HD centers in South America (SA. Methods A questionnaire was sent to 24 centers involved in the care for HD patients in SA. Results Of the total 24 centers, 19 (79.2% are academic units. The majority of centers (62.5% are general movement disorders clinics. Multidisciplinary care is available in 19 (79.2% centers and in 20 (83.3% care is provided free of charge. Genetic testing and counseling are available in 25 and 66.6% of centers, respectively. The majority of centers (83.3% have no institutional support for end-stage care. Conclusions Although HD centers in SA are committed to providing multidisciplinary care, access to genetic counseling and end-stage care are lacking in most centers. A doença de Huntington (DH é uma doença neurodegenerativa rara que requer tratamento multidisciplinar especializado para manejo adequado. O objetivo do presente trabalho foi pesquisar as características da assistência à saúde em centros de DH na América do Sul (AS. Métodos Um questionário foi enviado para 24 centros envolvidos no cuidado de pacientes com DH na AS. Resultados Dos 24 centros, 19 (79,2% são unidades acadêmicas. A maioria (62,5% são clínicas de distúrbios dos movimentos. Cuidado multidisciplinar é disponível em 19 (79,2% dos centros e em 20 (83,3%, o tratamento é gratuito. O teste e o aconselhamento genético estão disponíveis em 25 e 66,6% dos centros, respectivamente. Não há suporte institucional para cuidado terminal em 83,3% dos centros. Conclusões Apesar dos centros de DH na AS terem compromisso com o provimento de cuidados multidisciplinares, o acesso a aconselhamento genético e a tratamento na fase terminal são falhos na maioria dos centros.

Picture This: 4-H Press Corps Builds Life Skills

Science.gov (United States)

Clary, Christy D.

2018-01-01

A picture is worth a thousand words! Extension professionals are often looking for the picture that best captures an event and tells its story. Look beneath the surface, though, and a picture is worth much more. Developing a 4-H press corps results in a collection of useful photos but has the added benefit of providing 4-H members with an…

75 FR 9455 - U.S. Biomedical Corp., (f/k/a United Textiles & Toys, Inc.), U.S. Environmental Solutions, Inc...

Science.gov (United States)

2010-03-02

... SECURITIES AND EXCHANGE COMMISSION [File No. 500-1] U.S. Biomedical Corp., (f/k/a United Textiles & Toys, Inc.), U.S. Environmental Solutions, Inc. (n/k/a EnviroResolutions, Inc.), USA Bridge....S. Biomedical Corp. (f/k/a United Textiles & Toys, Inc.) because it has not filed any periodic...

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...

Early grey matter changes in structural covariance networks in Huntington's disease.

Science.gov (United States)

Coppen, Emma M; van der Grond, Jeroen; Hafkemeijer, Anne; Rombouts, Serge A R B; Roos, Raymund A C

2016-01-01

Progressive subcortical changes are known to occur in Huntington's disease (HD), a hereditary neurodegenerative disorder. Less is known about the occurrence and cohesion of whole brain grey matter changes in HD. We aimed to detect network integrity changes in grey matter structural covariance networks and examined relationships with clinical assessments. Structural magnetic resonance imaging data of premanifest HD ( n  = 30), HD patients (n = 30) and controls (n = 30) was used to identify ten structural covariance networks based on a novel technique using the co-variation of grey matter with independent component analysis in FSL. Group differences were studied controlling for age and gender. To explore whether our approach is effective in examining grey matter changes, regional voxel-based analysis was additionally performed. Premanifest HD and HD patients showed decreased network integrity in two networks compared to controls. One network included the caudate nucleus, precuneous and anterior cingulate cortex (in HD p  covariance might be a sensitive approach to reveal early grey matter changes, especially for premanifest HD.

78 FR 12799 - Advisory Committee for Innovation Corps; Notice of Meeting; Correction

Science.gov (United States)

2013-02-25

... announcing a meeting of the Advisory Committee for Innovation Corps. The location and time of the business meeting are updated to reflect the accurate plans. In accordance with the Federal Advisory Committee Act...

Future Services for District Heating Solutions in Residential Districts

Directory of Open Access Journals (Sweden)

Hannele Ahvenniemi

2014-06-01

Full Text Available The underlying assumption of this study is that in order to retain the competitiveness while reaching for the EU targets regarding low-energy construction, district heating companies need to develop new business and service models. How district heating companies could broaden their perspective and switch to a more service-oriented way of thinking is a key interest of our research. The used methods in our study are house builder interviews and a questionnaire. With the help of these methods we discussed the potential interest in heating related services acquiring a comprehensive understanding of the customer needs. The results indicate the importance of certain criteria when choosing the heating system in households: easiness, comfort and affordability seem to dominate the house builders’ preferences. Also environmental awareness seems to be for many an important factor when making a decision about the heating of the house. Altogether, based on the results of this study, we suggest that the prospects of district heating could benefit from highlighting certain aspects and strengths in the future. District heating companies need to increase flexibility, readiness to adopt new services, to invest in new marketing strategies and improving the communication skills.

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...

77 FR 12226 - Sadex Corp.; Filing of Food Additive Petition (Animal Use); Electron Beam and X-Ray Sources for...

Science.gov (United States)

2012-02-29

... DEPARTMENT OF HEALTH AND HUMAN SERVICES Food and Drug Administration 21 CFR Part 579 [Docket No. FDA-2012-F-0178] Sadex Corp.; Filing of Food Additive Petition (Animal Use); Electron Beam and X-Ray... Sadex Corp. has filed a petition proposing that the food additive regulations be amended to provide for...

Nankana West District of Ghana

African Journals Online (AJOL)

Agribotix GCS 077

Local governments in Ghana play very important roles with actors in the ... Metropolitan, Municipal and District Chief Executives (MMDCEs), the .... District Budget Officer, District Finance Officer, Presiding Member, members of the Works Sub-.

78 FR 36540 - Establishment of the Board of Visitors, Marine Corps University

Science.gov (United States)

2013-06-18

... University; and d. The operation and accreditation of the National Museum of the Marine Corps. The Board... membership about the Board's mission and functions. Written statements may be submitted at any time or in...

Crucible of fire: the Boer War and the birth of the Canadian Army Medical Corps.

Science.gov (United States)

McCulloch, I

1995-01-01

Although Canada's military physicians didn't come to prominence until WW I and WW II, the Canadian Army Medical Corps (CAMC), the forerunner of the Royal Canadian Army Medical Corps and the current Canadian Forces Medical Service, actually had its origins in the Boer War. During that turn-of-the-century conflict, field hospitals accompanied Canadian troops to South Africa. Ian McCulloch discusses that early type of medical service and the steps that led to the creation of the CAMC. Images p1495-a p1496-a PMID:7585380

Total recognition discriminability in Huntington's and Alzheimer's disease.

Science.gov (United States)

Graves, Lisa V; Holden, Heather M; Delano-Wood, Lisa; Bondi, Mark W; Woods, Steven Paul; Corey-Bloom, Jody; Salmon, David P; Delis, Dean C; Gilbert, Paul E

2017-03-01

Both the original and second editions of the California Verbal Learning Test (CVLT) provide an index of total recognition discriminability (TRD) but respectively utilize nonparametric and parametric formulas to compute the index. However, the degree to which population differences in TRD may vary across applications of these nonparametric and parametric formulas has not been explored. We evaluated individuals with Huntington's disease (HD), individuals with Alzheimer's disease (AD), healthy middle-aged adults, and healthy older adults who were administered the CVLT-II. Yes/no recognition memory indices were generated, including raw nonparametric TRD scores (as used in CVLT-I) and raw and standardized parametric TRD scores (as used in CVLT-II), as well as false positive (FP) rates. Overall, the patient groups had significantly lower TRD scores than their comparison groups. The application of nonparametric and parametric formulas resulted in comparable effect sizes for all group comparisons on raw TRD scores. Relative to the HD group, the AD group showed comparable standardized parametric TRD scores (despite lower raw nonparametric and parametric TRD scores), whereas the previous CVLT literature has shown that standardized TRD scores are lower in AD than in HD. Possible explanations for the similarity in standardized parametric TRD scores in the HD and AD groups in the present study are discussed, with an emphasis on the importance of evaluating TRD scores in the context of other indices such as FP rates in an effort to fully capture recognition memory function using the CVLT-II.

Plasma homovanillic acid and prolactin in Huntington's disease.

Science.gov (United States)

Markianos, Manolis; Panas, Marios; Kalfakis, Nikos; Vassilopoulos, Dimitrios

2009-05-01

Dopaminergic activity is expected to be altered in patients with Huntington's disease (HD) and be related to factors like duration and severity of illness or patients' specific symptomatology like dementia, depression, or psychotic features. We assessed plasma homovanillic acid (pHVA) and plasma prolactin (pPRL), two correlates of dopaminergic activity, in 116 subjects with CAG repeats expansion in the HD gene, 26 presymptomatic (18 females) and 90 with overt symptomatology (43 females). Patients were evaluated using the Unified HD Rating Scale and the Total Functional Capacity Scale. Presence of dementia, depression, and psychotic features were also assessed. The age range of the patients was 22-83 years, duration of illness from 0.5 to 27 years, and CAG repeat number from 34 to 66. A group of 60 age and sex matched healthy subjects served as control group. Plasma PRL in subjects at risk and in neuroleptic-free patients, evaluated separately for males and females, did not differ from controls. Plasma HVA levels did not differ from controls in the group of presymptomatic subjects, but were significantly higher in the patients group. This increase was positively associated mainly with severity of illness and functional capacity of the patients, and not with presence of depression or dementia. Plasma HVA levels may be proven to be a peripheral index of disease progression. Reducing dopaminergic activity may have not only symptomatic, but also neuroprotective effects in HD.

Characterizing Fractures Across the Astronaut Corps: Preliminary Findings from Population-Level Analysis

Science.gov (United States)

Rossi, Meredith M.; Charvat, Jacqueline; Sibonga, Jean; Sieker, Jeremy

2017-01-01

Despite evidence of bone loss during spaceflight and operational countermeasures to mitigate this loss, the subsequent risk of fracture among astronauts is not known. The physiologic process of diminished bone density and bone recovery during or following spaceflight is multifactorial. Such factors as age, sex, fracture history, and others may combine to increase fracture risk among astronauts. As part of the 2016 Bone Research and Clinical Advisory Panel (RCAP), the authors analyzed data collected on 338 NASA astronauts to describe the demographics, bone-relevant characteristics, and fracture history of the astronaut population. The majority of the population are male (n=286, 84.6%), have flown at least one mission (n=306, 90.5%), and were between the ages of 30 and 49 at first mission (n=296, 96.7% of those with at least one mission). Of the 338 astronauts, 241 (71.3%) experienced a fracture over the course of their lifetime. One hundred and five (43.5%) of these 241 astronauts only experienced a fracture prior to being selected into the Astronaut Corps, whereas 53 (22.0%) only experienced a fracture after selection as an astronaut. An additional 80 astronauts (33.2%) had both pre- and post-selection fractures. The remaining 3 astronauts had a fracture of unknown date, which could not be categorized as pre- or post-selection. Among the 133 astronauts with at least one post-selection fracture, males comprised 90.2% (n=120) compared to 84.5% of the entire Corps, and females accounted for 9.8% (n=13) compared to 15.4% of the Corps. Ninety-seven of the 133 astronauts with post-selection fractures (72.9%) had one fracture event, 22 (16.5%) had two fractures, and 14 (10.5%) had three or more fractures. Some astronauts with multiple fractures suffered these in a single event, such as an automobile accident. The 133 astronauts with a post-selection fracture accounted for a total of 188 fracture events. One hundred and four (78.2%) of astronauts with post

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.

Map showing selected surface-water data for the Huntington 30 x 60-minute quadrangle, Utah

Science.gov (United States)

Price, Don

1984-01-01

This is one of a series of maps that describe the geology and related natural resources of the Huntington 30 x 60-minute quadrangle, Utah. Streamflow records used to compile this map were collected by the U.S. Geological Survey in cooperation with the Utah Department of Natural Resources, Division of Water Rights, and the Utah Department of Transportation. The principal runoff-producing area shown on the map was delineated from a work map (scale 1:250,000) compiled to estimate water yields in Utah (Bagley and others, 1964). Sources of information about recorded floods resulting from cloudbursts included Woolley (1946) and Butler and Marsell (1972); sources of information about the chemical quality of streamflow included Mundorff (1972) and Mundorff and Thompson (1982).

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.

Aerial gamma ray and magnetic survey, Huntington quadrangle: Ohio, West Virginia and Kentucky. Final report

International Nuclear Information System (INIS)

1981-04-01

The Huntington quadrangle of Kentucky, Ohio, and West Virginia covers 7250 square miles of the easternmost Midwestern Physiographic Province. Paleozoic exposures dominate the surface. These Paleozoics deepen toward the east from approximately 500 feet to a maximum depth of 8000 feet. Precambrian basement is thought to underlie the entire area. No known uranium deposits exist in the area. One hundred anomalies were found using the standard statistical analysis. Some high uranium concentration anomalies that may overlie the stratigraphic equivalent of the Devonian-Mississippian New Albany or Chattanooga Shales may represent significant levels of naturally occurring uranium. Future studies should concentrate on this unit. Magnetic data are largely in concurrence with existing structural interpretations but suggest some complexities in the underlying Precambrian

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

Boundaries - US Army Corps of Engineers - Habitat Restoration and Enhancement Projects (HREPs)

Data.gov (United States)

Army Corps of Engineers, Department of the Army, Department of Defense — Congress authorized the Environmental Management Program (EMP) in the 1986 Water Resources Development Act to help address ecological needs on the Upper Mississippi...

Revelation of the IFN alpha, IL-10, IL-8 and IL-1 beta as promising biomarkers reflecting immuno-pathological mechanisms in porcine Huntington's disease model

Czech Academy of Sciences Publication Activity Database

Valeková, Ivona; Jarkovská, Karla; Kotrčová, Eva; Bucci, J.; Ellederová, Zdeňka; Juhás, Štefan; Motlík, Jan; Gadher, S. J.; Kovářová, Hana

2016-01-01

Roč. 293, č. 2 (2016), s. 71-81 ISSN 0165-5728 R&D Projects: GA MŠk ED2.1.00/03.0124; GA MŠk(CZ) LO1609 Institutional support: RVO:67985904 Keywords : porcine Huntington´s disease * immune response * cytokines * central nervous system * serum * biomarkers Subject RIV: FH - Neurology Impact factor: 2.720, year: 2016

Cuerpo de Paz Manual de Sistema de Programacion y Capacitacion (Peace Corps Programming and Training System Manual): T0063.

Science.gov (United States)

Peace Corps, Washington, DC.

This Spanish version of the Peace Corps Programming and Training System Manual is designed to help field staff members of the Peace Corps train volunteers. Its task descriptions, guidelines, examples, and definitions are intended to be practical and informative rather than restrictive. The manual is divided into six major sections: (1)…

Detection of early behavioral markers of Huntington's disease in R6/2 mice employing an automated social home cage

DEFF Research Database (Denmark)

Rudenko, Olga; Tkach, Vadim; Berezin, Vladimir

2009-01-01

developed behavior screening system, the IntelliCage, allows automated testing of mouse behavior in the home cage employing individual recognition of animals living in social groups. The present study validates the ability of the IntelliCage system to detect behavioral and cognitive dysfunction in R6/2 mice......Huntington's disease (HD) is an autosomal-dominant neurodegenerative disorder, for which no known cure or effective treatment exists. To facilitate the search for new potential treatments of HD, an automated system for analyzing the behavior of transgenic HD mice is urgently needed. A recently...

District Energy Windsor

Energy Technology Data Exchange (ETDEWEB)

NONE

2002-07-01

This paper presents a summary of how District Energy Windsor operates. It includes a system site map and reasons why it is advantageous to get connected to a district heating system. District Energy Windsor is a division of the Windsor Utilities Commission. It was developed in 1996 and was the first in North America to supply both heating and cooling requirements. It supplies nearly 2 million square feet of heating and cooling for Windsor's city centre. The district energy system produces hot water or chilled water at a central plant. Energy is then piped out to buildings in the area, including the Art Gallery of Windsor, the Royal Bank Business Centre, the Windsor Justice Facility, the Windsor Casino, and Northwind Windsor. The energy, which is transferred through heat exchangers, is used for space heating, domestic hot water heating, and air conditioning. The 8 reasons for getting connected are: (1) less management costs, (2) lower energy costs, (3) lower level of risk management, (4) stable energy rates, (5) better use of building space, (6) reliable service, (7) reduced expansion costs, and (8) a cleaner environment. District heating improves air quality through reduced carbon dioxide and nitrogen oxide emissions. In addition, fuel delivery and storage are eliminated. figs.

Dose-Dependent Lowering of Mutant Huntingtin Using Antisense Oligonucleotides in Huntington Disease Patients.

Science.gov (United States)

van Roon-Mom, Willeke M C; Roos, Raymund A C; de Bot, Susanne T

2018-04-01

On December 11 of 2017, Ionis Pharmaceuticals published a press release announcing dose-dependent reductions of mutant huntingtin protein in their HTTRx Phase 1/2a study in Huntington disease (HD) patients. The results from this Ionis trial have gained much attention from the patient community and the oligonucleotide therapeutics field, since it is the first trial targeting the cause of HD, namely the mutant huntingtin protein, using antisense oligonucleotides (ASOs). The press release also states that the primary endpoints of the study (safety and tolerability) were met, but does not contain data. This news follows the approval of another therapeutic ASO nusinersen (trade name Spinraza) for a neurological disease, spinal muscular atrophy, by the U.S. Food and Drug Administration and European Medicines Agency, in 2016 and 2017, respectively. Combined, this offers hope for the development of the HTTRx therapy for HD patients.

78 FR 72139 - Nevada Gold Corp.; Order of Suspension of Trading

Science.gov (United States)

2013-12-02

... current and accurate information concerning the securities of Nevada Gold Corp. (``Nevada Gold'') because of questions regarding the accuracy of assertions by Nevada Gold, and by others, to investors in..., and financial condition. Nevada Gold is a Delaware corporation based in Del Mar, California. The...

Les representations du corps chez les femmes pratiquant le fitness a ...

African Journals Online (AJOL)

L'image du corps semble bien avoir évolué et la corpulence est en passe d'être ... and control of their body (weight, health) in a context where dominant culture ... Body image seems to have evolved and corpulence is about to be devalued; ...

Additive Manufacturing Solutions in the United States Marine Corps

Science.gov (United States)

2017-12-01

public release. Distribution is unlimited. 12b. DISTRIBUTION CODE 13. ABSTRACT (maximum 200 words) This project conducts a cost benefit analysis to...continuous liquid interface production (CLIP). The findings from the cost benefit analysis show a cost advantage for additive manufacturing at the...Corps, cost benefit analysis, CLIP 15. NUMBER OF PAGES 119 16. PRICE CODE 17. SECURITY CLASSIFICATION OF REPORT Unclassified 18. SECURITY

Revisiting "Rodriguez v. Los Angeles Unified School District": A Case of Intra-District Inequities

Science.gov (United States)

Espinosa, Ruben W.

2010-01-01

The educational community and the courts continue to struggle with the challenges of intra-district resource inequality revealed by the California Supreme Court landmark case "Rodriguez v. Los Angeles Unified School District" (1992). Intra-district school resource inequality is one of the remaining bastions of major inequalities in the…

VII international district heating conference

International Nuclear Information System (INIS)

1988-01-01

The proceedings of the 7th International District Heating Conference contain the full texts of the 89 presented papers of which 11 fall under the INIS Subject Scope. The conference met in seven sessions and dealt with the following problem areas: design and optimization of systems of district heating, integration of the power system and the district heating systems, cooperation of nuclear and fossil burning sources in district heating systems, the use of specific nuclear power plants for heating purposes, questions of the control of systems of district heating, the development of components of heating networks, the reliability and design of heat supply pipes. (Z.M.)

Promoting Effective Parenting Practices and Preventing Child Behavior Problems in School among Ethnically Diverse Families from Underserved, Urban Communities

Science.gov (United States)

Brotman, Laurie Miller; Calzada, Esther; Huang, Keng-Yen; Kingston, Sharon; Dawson-McClure, Spring; Kamboukos, Dimitra; Rosenfelt, Amanda; Schwab, Amihai; Petkova, Eva

2011-01-01

This study examines the efficacy of "ParentCorps" among 4-year-old children (N = 171) enrolled in prekindergarten in schools in a large urban school district. "ParentCorps" includes a series of 13 group sessions for parents and children held at the school during early evening hours and facilitated by teachers and mental health…

Peace Corps Stateside Teacher Training for Volunteers in Liberia. Volume V: Cross-Cultural Training and Support Services. Final Report.

Science.gov (United States)

PSI Associates, Inc., Washington, DC.

The cross-cultural training module and support services for Peace Corps volunteers en route to Liberia make trainees more aware of and sensitive to cultural differences in human behavior and human interaction. In this part of the Peace Corps Stateside Teacher Training Model, the approach to training is both generic and specific, and both native…

Update: Exertional rhabdomyolysis, active component, U.S. Army, Navy, Air Force, and Marine Corps, 2011-2015.

Science.gov (United States)

Armed Forces Health Surveillance Branch

2016-03-01

Among active component members of the U.S. Army, Navy, Air Force, and Marine Corps in 2015, there were 456 incident episodes of rhabdomyolysis likely due to physical exertion or heat stress ("exertional rhabdomyolysis"). Annual rates of incident diagnoses of exertional rhabdomyolysis increased 17% between 2014 and 2015. In 2015, the highest incidence rates occurred in service members who were male; younger than 20 years of age; black, non-Hispanic; members of the Marine Corps and Army; recruit trainees; and in combat-specific occupations. Most cases of exertional rhabdomyolysis were diagnosed at installations that support basic combat/recruit training or major ground combat units of the Army or Marine Corps. Medical care providers should consider exertional rhabdomyolysis in the differential diagnosis when service members (particularly recruits) present with muscular pain and swelling, limited range of motion, or the excretion of dark urine (e.g., myoglobinuria) after strenuous physical activity, particularly in hot, humid weather.

District heating in Italy

International Nuclear Information System (INIS)

Sacchi, E.

1998-01-01

The legislative act establishing the electric monopoly virtually shut out the district heating associated with electricity cogeneration, while other laws, issued to counteract the effects of oil shocks, allowed municipal utilities to do so. Thus, district heating has experienced some development, though well below its possibilities. The article analyses the reasons for this lagging, reports district heating data and projects its forecasts against the Kyoto Protocol objectives [it

VCE-003.2, a novel cannabigerol derivative, enhances neuronal progenitor cell survival and alleviates symptomatology in murine models of Huntington's disease.

Science.gov (United States)

Díaz-Alonso, Javier; Paraíso-Luna, Juan; Navarrete, Carmen; Del Río, Carmen; Cantarero, Irene; Palomares, Belén; Aguareles, José; Fernández-Ruiz, Javier; Bellido, María Luz; Pollastro, Federica; Appendino, Giovanni; Calzado, Marco A; Galve-Roperh, Ismael; Muñoz, Eduardo

2016-07-19

Cannabinoids have shown to exert neuroprotective actions in animal models by acting at different targets including canonical cannabinoid receptors and PPARγ. We previously showed that VCE-003, a cannabigerol (CBG) quinone derivative, is a novel neuroprotective and anti-inflammatory cannabinoid acting through PPARγ. We have now generated a non-thiophilic VCE-003 derivative named VCE-003.2 that preserves the ability to activate PPARγ and analyzed its neuroprotective activity. This compound exerted a prosurvival action in progenitor cells during neuronal differentiation, which was prevented by a PPARγ antagonist, without affecting neural progenitor cell proliferation. In addition, VCE-003.2 attenuated quinolinic acid (QA)-induced cell death and caspase-3 activation and also reduced mutant huntingtin aggregates in striatal cells. The neuroprotective profile of VCE-003.2 was analyzed using in vivo models of striatal neurodegeneration induced by QA and 3-nitropropionic acid (3NP) administration. VCE-003.2 prevented medium spiny DARPP32(+) neuronal loss in these Huntington's-like disease mice models improving motor deficits, reactive astrogliosis and microglial activation. In the 3NP model VCE-003.2 inhibited the upregulation of proinflammatory markers and improved antioxidant defenses in the brain. These data lead us to consider VCE-003.2 to have high potential for the treatment of Huntington's disease (HD) and other neurodegenerative diseases with neuroinflammatory traits.

77 FR 74168 - Information Collection: Youth Conservation Corps Application and Medical History

Science.gov (United States)

2012-12-13

... DEPARTMENT OF AGRICULTURE Forest Service Information Collection: Youth Conservation Corps...-1706), the Forest Service, U.S. Department of Agriculture; the Fish and Wildlife Service, and National... of birth, age, mailing address, telephone numbers, email address, gender, educational background...

Agents of Change; A Close Look at the Peace Corps.

Science.gov (United States)

Hapgood, David; Bennett, Meridan

A critical evaluation of the Peace Corps covers a broad range of ideas and events, including the consequences of United States intervention in the affairs of others, the theory and practice of teaching in other cultures, the difficulties of food production and nutrition, and the problems of birth control and public health. There is also a…

7 CFR 958.27 - Districts.

Science.gov (United States)

2010-01-01

...; (2) changes in the relative position of existing districts with respect to onion production; (3) the... and Orders; Fruits, Vegetables, Nuts), DEPARTMENT OF AGRICULTURE ONIONS GROWN IN CERTAIN DESIGNATED... Districts. (a) For the purpose of selecting committee members, the following districts of the production...

Intra-District Disparities in Primary Education: A Case Study of Bankura District, West Bengal

Directory of Open Access Journals (Sweden)

Krishnendu Maji

2017-03-01

Full Text Available The level of education and its response to different educational opportunities vary from one spatial unit to another depending on various factors like social, economic, cultural, and institutional. It is understood that certain regions acquire relative advancements over others in terms of human resource development and human capital formation. The key purpose of this research is to examine the intra-district disparities in primary education in Bankura District one of the districts of West Bengal. It ranks 11thamong the 19 districts of West Bengal (Human Development Report, 2007.  Overall literacy rate of the district stands at 70.26% but the district scores low in terms of female literacy rates, which is 60.05%,whereas the male literacy rate is 80.05%, which is a huge gender literacy gap of 20%. There are also regional inequalities existing at block level. Kotulpur ranks first with a literacy rate of 78.01% while Saltora occupies the bottom position with literacy rate of just 61.45% (Census of India, 2011. The level of educational development is dependent on several factors—enrolment ratio, dropout and repetition rates, pupil-teacher ratio, habitations covered by educational institutions, space-student ratio, drinking water and sanitation facilities in school, etc. In this context, the present study aims at examining the issues of intra-district disparities in educational attainment with regard to various educational amenities of Bankura district, West Bengal. Ten attributes have been selected to examine the level of development in primary education. It is clear from the study that the level of development in eastern part of the district is relatively better in comparison to other regions. Economic backwardness and physical bottlenecks continue to be major issues in western blocks.

45 CFR 2520.30 - What capacity-building activities may AmeriCorps members perform?

Science.gov (United States)

2010-10-01

... AmeriCorps members perform should enhance the mission, strategy, skills, and culture, as well as systems... that are included in, or consistent with, your Corporation-approved grant application. (b) Examples of...

Congress and the Marine Corps: An Enduring Partnership

Science.gov (United States)

2017-12-22

dress uniforms; if the photographer happened to catch the guests’ children enjoying themselves with Marines, so much the bet - ter. It should be noted...say, well we’re going to tell you how this is going to be done, because we don’t think you understand what needs to be done, and so we’ll just tell...gives the Corps a window into what Congress as a whole is thinking . Along with established organizations and programs, there are three aspects of

Lieutenant Chief Warden Districts

Data.gov (United States)

Vermont Center for Geographic Information — This dataset is a representation overlay of Lieutenant Chief Warden Districts (areas of responsibility). The Vermont Lieutenant Chief Warden Districts layer is part...

Socioeconomic Status and Performance in the US Army and US Marine Corps

National Research Council Canada - National Science Library

Booth, Stefan

1998-01-01

The purpose or this study was to examine socioeconomic status (SES) of recruits in the Army and Marine Corps and to analyze the relationship between a recruit's SES background and his or her performance in the military over time...

State-Level Guidance and District-Level Policies and Practices for Food Marketing in US School Districts.

Science.gov (United States)

Merlo, Caitlin L; Michael, Shannon; Brener, Nancy D; Blanck, Heidi

2018-06-07

State agencies play a critical role in providing school districts with guidance and technical assistance on school nutrition issues, including food and beverage marketing practices. We examined associations between state-level guidance and the policies and practices in school districts regarding food and beverage marketing and promotion. State policy guidance was positively associated with districts prohibiting advertisements for junk food or fast food restaurants on school property. Technical assistance from states was negatively associated with 2 district practices to restrict marketing of unhealthy foods and beverages, but positively associated with 1 practice to promote healthy options. These findings may help inform the guidance that states provide to school districts and help identify which districts may need additional assistance to address marketing and promotion practices.

77 FR 63326 - Huron Wetland Management District, Madison Wetland Management District, and Sand Lake Wetland...

Science.gov (United States)

2012-10-16

..., consistent with sound principles of fish and wildlife management, conservation, legal mandates, and our... FXRS1265066CCP0] Huron Wetland Management District, Madison Wetland Management District, and Sand Lake Wetland Management District, SD; Final Comprehensive Conservation Plan and Finding of No Significant Impact for...

Embodied emotion impairment in Huntington's Disease.

Science.gov (United States)

Trinkler, Iris; Devignevielle, Sévérine; Achaibou, Amal; Ligneul, Romain V; Brugières, Pierre; Cleret de Langavant, Laurent; De Gelder, Beatrice; Scahill, Rachael; Schwartz, Sophie; Bachoud-Lévi, Anne-Catherine

2017-07-01

Theories of embodied cognition suggest that perceiving an emotion involves somatovisceral and motoric re-experiencing. Here we suggest taking such an embodied stance when looking at emotion processing deficits in patients with Huntington's Disease (HD), a neurodegenerative motor disorder. The literature on these patients' emotion recognition deficit has recently been enriched by some reports of impaired emotion expression. The goal of the study was to find out if expression deficits might be linked to a more motoric level of impairment. We used electromyography (EMG) to compare voluntary emotion expression from words to emotion imitation from static face images, and spontaneous emotion mimicry in 28 HD patients and 24 matched controls. For the latter two imitation conditions, an underlying emotion understanding is not imperative (even though performance might be helped by it). EMG measures were compared to emotion recognition and to the capacity to identify and describe emotions using alexithymia questionnaires. Alexithymia questionnaires tap into the more somato-visceral or interoceptive aspects of emotion perception. Furthermore, we correlated patients' expression and recognition scores to cerebral grey matter volume using voxel-based morphometry (VBM). EMG results replicated impaired voluntary emotion expression in HD. Critically, voluntary imitation and spontaneous mimicry were equally impaired and correlated with impaired recognition. By contrast, alexithymia scores were normal, suggesting that emotion representations on the level of internal experience might be spared. Recognition correlated with brain volume in the caudate as well as in areas previously associated with shared action representations, namely somatosensory, posterior parietal, posterior superior temporal sulcus (pSTS) and subcentral sulcus. Together, these findings indicate that in these patients emotion deficits might be tied to the "motoric level" of emotion expression. Such a double

Assessment of Charging Infrastructure for Plug-in Electric Vehicles at Marine Corps Base Camp Lejeune. Task 3

Energy Technology Data Exchange (ETDEWEB)

Schey, Stephen [Idaho National Lab. (INL), Idaho Falls, ID (United States); Francfort, Jim [Idaho National Lab. (INL), Idaho Falls, ID (United States)

2015-11-01

Battelle Energy Alliance, LLC, managing and operating contractor for the U.S. Department of Energy’s Idaho National Laboratory, is the lead laboratory for the U.S. Department of Energy’s advanced vehicle testing. Battelle Energy Alliance, LLC contracted with Intertek Testing Services, North America (Intertek) to conduct several U.S. Department of Defense-based studies to identify potential U.S. Department of Defense transportation systems that are strong candidates for introduction or expansion of plug-in electric vehicles (PEVs). Task 1 consisted of a survey of the non-tactical fleet of vehicles at Marine Corps Base Camp Lejeune to begin the review of vehicle mission assignments and types of vehicles in service. Task 2 selected vehicles for further monitoring and involved identifying daily operational characteristics of these select vehicles. Data logging of vehicle movements was initiated in order to characterize the vehicle’s mission. The Task 3 vehicle utilization report provided results of the data analysis and observations related to the replacement of current vehicles with PEVs. Finally, this report provides an assessment of charging infrastructure required to support the suggested PEV replacements. Intertek acknowledges the support of Idaho National Laboratory, Marine Corps headquarters, and Marine Corps Base Camp Lejeune Fleet management and personnel for participation in this study. Intertek is pleased to provide this report and is encouraged by enthusiasm and support from Marine Corps Base Camp Lejeune personnel.

VT Data - Overlay District 20170419, Colchester

Data.gov (United States)

Vermont Center for Geographic Information — The following Overlay Districts are included in the data:General Development Four Commercial DistrictGeneral Development Four Openspace DistrictShoreland...

Evidence for a modifier of onset age in Huntington disease linked to the HD gene in 4p16

Science.gov (United States)

Djoussé, Luc; Knowlton, Beth; Hayden, Michael R.; Almqvist, Elisabeth W.; Brinkman, Ryan R.; Ross, Christopher A.; Margolis, Russel L.; Rosenblatt, Adam; Durr, Alexandra; Dode, Catherine; Morrison, Patrick J.; Novelletto, Andrea; Frontali, Marina; Trent, Ronald J. A.; McCusker, Elizabeth; Gómez-Tortosa, Estrella; Mayo Cabrero, David; Jones, Randi; Zanko, Andrea; Nance, Martha; Abramson, Ruth K.; Suchowersky, Oksana; Paulsen, Jane S.; Harrison, Madaline B.; Yang, Qiong; Cupples, L. Adrienne; Mysore, Jayalakshmi; Gusella, James F.; MacDonald, Marcy E.

2007-01-01

Huntington disease (HD) is a neurodegenerative disorder caused by the abnormal expansion of CAG repeats in the HD gene on chromosome 4p16.3. A recent genome scan for genetic modifiers of age at onset of motor symptoms (AO) in HD suggests that one modifier may reside in the region close to the HD gene itself. We used data from 535 HD participants of the New England Huntington cohort and the HD MAPS cohort to assess whether AO was influenced by any of the three markers in the 4p16 region: MSX1 (Drosophila homeo box homologue 1, formerly known as homeo box 7, HOX7), Δ2642 (within the HD coding sequence), and BJ56 (D4S127). Suggestive evidence for an association was seen between MSX1 alleles and AO, after adjustment for normal CAG repeat, expanded repeat, and their product term (model P value 0.079). Of the variance of AO that was not accounted for by HD and normal CAG repeats, 0.8% could be attributed to the MSX1 genotype. Individuals with MSX1 genotype 3/3 tended to have younger AO. No association was found between Δ2642 (P=0.44) and BJ56 (P=0.73) and AO. This study supports previous studies suggesting that there may be a significant genetic modifier for AO in HD in the 4p16 region. Furthermore, the modifier may be present on both HD and normal chromosomes bearing the 3 allele of the MSX1 marker. PMID:15029481

Disruption of astrocyte-neuron cholesterol cross talk affects neuronal function in Huntington's disease.

Science.gov (United States)

Valenza, M; Marullo, M; Di Paolo, E; Cesana, E; Zuccato, C; Biella, G; Cattaneo, E

2015-04-01

In the adult brain, neurons require local cholesterol production, which is supplied by astrocytes through apoE-containing lipoproteins. In Huntington's disease (HD), such cholesterol biosynthesis in the brain is severely reduced. Here we show that this defect, occurring in astrocytes, is detrimental for HD neurons. Astrocytes bearing the huntingtin protein containing increasing CAG repeats secreted less apoE-lipoprotein-bound cholesterol in the medium. Conditioned media from HD astrocytes and lipoprotein-depleted conditioned media from wild-type (wt) astrocytes were equally detrimental in a neurite outgrowth assay and did not support synaptic activity in HD neurons, compared with conditions of cholesterol supplementation or conditioned media from wt astrocytes. Molecular perturbation of cholesterol biosynthesis and efflux in astrocytes caused similarly altered astrocyte-neuron cross talk, whereas enhancement of glial SREBP2 and ABCA1 function reversed the aspects of neuronal dysfunction in HD. These findings indicate that astrocyte-mediated cholesterol homeostasis could be a potential therapeutic target to ameliorate neuronal dysfunction in HD.

Simultaneous Estimation of Regression Functions for Marine Corps Technical Training Specialties.

Science.gov (United States)

Dunbar, Stephen B.; And Others

This paper considers the application of Bayesian techniques for simultaneous estimation to the specification of regression weights for selection tests used in various technical training courses in the Marine Corps. Results of a method for m-group regression developed by Molenaar and Lewis (1979) suggest that common weights for training courses…

Reinstating district nursing: A UK perspective.

Science.gov (United States)

Morris, Hannah

2017-09-01

As policy directives gather pace for service provision to be delivered in primary care, district nursing has not been recognised as a valuable asset to facilitate this agenda. Investment in district nursing and specialist district nursing education has fallen. This is concurrent with an ageing district nursing workforce, a lack of recruitment and growing caseloads, as district nursing adapts to meet the challenges of the complexities of contemporary healthcare in the community. The district nurse role is complex and multifaceted and includes working collaboratively and creatively to coordinate care. Redressing the shortages of specialist district nurse practitioners with increased numbers of health care support workers will not replace the skill, knowledge, experience required to meet the complex care needs of today's society. District nursing needs to be reinstated as the valuable asset it is, through renewed investment in the service, research development and in specialist practice education. To prevent extinction district nurses need to be able to demonstrate and articulate the complexities and dynamisms of the role to reinstate themselves to their commissioners as a valuable asset for contemporary practice that can meet current health and social care needs effectively. Crown Copyright © 2017. Published by Elsevier Ltd. All rights reserved.

Leadership Styles in United States Marine Corps Transport Helicopter Squadrons

Science.gov (United States)

1989-12-01

This thesis examined leadership styles in United States Marine Corps transport helicopter squadrons. Analyses were conducted to determine how... leadership styles related to subordinate extra effort, leader effectiveness, satisfaction with leader, unit cohesion, and unit morale. The importance of...military history to the development of military leaders was also examined. Leadership styles of officers were evaluated by the leader himself as well as

District Consolidation: Rivals Coming Together

Science.gov (United States)

Mart, Dan

2011-01-01

District consolidation is a highly emotional process. One key to success is sticking to the facts. In Iowa, school districts facing financial difficulties or enrollment concerns do not have to move directly to consolidation. In many cases, districts begin by developing sharing agreements. These sharing agreements may start with simple sharing of…

Uso de medicamentos, glicemia capilar e índice de massa corpórea em pacientes com diabetes mellitus

Directory of Open Access Journals (Sweden)

Márcio Flávio Moura de Araújo

2013-10-01

Full Text Available Objetivou-se relacionar o uso de medicamentos com a glicemia capilar e o índice de massa corpórea em pacientes com diabetes mellitus tipo 2. Estudo transversal realizado entre janeiro e julho de 2009, nos domicílios de 437 pacientes de doze centros de saúde de Fortaleza-CE. Utilizou-se um formulário para o registro das variáveis sociodemográficas e clinicas, glicemia capilar, índice de massa corpórea e uso de medicamentos. Dos pacientes com glicemia capilar normal, 93% não utilizavam antidepressivos / ansiolíticos (p = 0,02. Aproximadamente 99% dos que apresentavam a glicemia capilar elevada não utilizavam cálcio diariamente (p = 0,04. Entre aqueles com índice de massa corpórea normal, 45,5% tomavam diariamente anti-hipertensivos da classe inibidor da ECA (p = 0,03. O uso diário de drogas inibidoras da ECA, antidepressivos / ansiolíticos e cálcio apresentou associação com a glicemia capilar e o índice de massa corpórea, respectivamente. É importante que o enfermeiro avalie o peso corporal e a glicemia capilar dos pacientes com diabetes, em particular daqueles que fazem uso contínuo de medicamentos.

Effects of ParentCorps in Prekindergarten on Child Mental Health and Academic Performance

Science.gov (United States)

Brotman, Laurie Miller; Dawson-McClure, Spring; Kamboukos, Dimitra; Huang, Keng-Yen; Calzada, Esther J.; Goldfeld, Keith; Petkova, Eva

2017-01-01

IMPORTANCE Low-income minority children living in urban neighborhoods are at high risk for mental health problems and underachievement. ParentCorps, a family-centered, school-based intervention in prekindergarten, improves parenting and school readiness (ie, self-regulation and preacademic skills) in 2 randomized clinical trials. The longer-term effect on child mental health and academic performance is not known. OBJECTIVE To examine whether ParentCorps delivered as an enhancement to prekindergarten programs in high-poverty urban schools leads to fewer mental health problems and increased academic performance in the early elementary school years. DESIGN, SETTING, AND PARTICIPANTS This is a 3-year follow-up study of a cluster randomized clinical trial of ParentCorps in public schools with prekindergarten programs in New York City. Ten elementary schools serving a primarily low-income, black student population were randomized in 2005, and 4 consecutive cohorts of prekindergarten students were enrolled from September 12, 2005, through December 31, 2008. We report follow-up for the 3 cohorts enrolled after the initial year of implementation. Data analysis was performed from September 1, 2014, to December 31, 2015. INTERVENTIONS ParentCorps included professional development for prekindergarten and kindergarten teachers and a program for parents and prekindergarten students (13 two-hour group sessions delivered after school by teachers and mental health professionals). MAIN OUTCOMES AND MEASURES Annual teacher ratings of mental health problems and academic performance and standardized tests of academic achievement in kindergarten and second grade by testers masked to the intervention or control group randomization. RESULTS A total of 1050 children (4 years old; 518 boys [49.3%] and 532 girls [50.7%]) in 99 prekindergarten classrooms participated in the trial (88.1% of the prekindergarten population), with 792 students enrolled from 2006 to 2008. Most families in the

D-β-hydroxybutyrate is protective in mouse models of Huntington's disease.

Directory of Open Access Journals (Sweden)

Soyeon Lim

Full Text Available Abnormalities in mitochondrial function and epigenetic regulation are thought to be instrumental in Huntington's disease (HD, a fatal genetic disorder caused by an expanded polyglutamine track in the protein huntingtin. Given the lack of effective therapies for HD, we sought to assess the neuroprotective properties of the mitochondrial energizing ketone body, D-β-hydroxybutyrate (DβHB, in the 3-nitropropionic acid (3-NP toxic and the R6/2 genetic model of HD. In mice treated with 3-NP, a complex II inhibitor, infusion of DβHB attenuates motor deficits, striatal lesions, and microgliosis in this model of toxin induced-striatal neurodegeneration. In transgenic R6/2 mice, infusion of DβHB extends life span, attenuates motor deficits, and prevents striatal histone deacetylation. In PC12 cells with inducible expression of mutant huntingtin protein, we further demonstrate that DβHB prevents histone deacetylation via a mechanism independent of its mitochondrial effects and independent of histone deacetylase inhibition. These pre-clinical findings suggest that by simultaneously targeting the mitochondrial and the epigenetic abnormalities associated with mutant huntingtin, DβHB may be a valuable therapeutic agent for HD.

33 CFR 3.25-1 - Fifth district.

Science.gov (United States)

2010-07-01

... District § 3.25-1 Fifth district. (a) The District Office is in Portsmouth, Va. (b) The Fifth Coast Guard District is comprised of: North Carolina; Virginia; District of Columbia; Maryland; Delaware; that part of... boundary at the shoreline at the North Carolina-South Carolina border, point located at approximately 30°55...

Diffusion tensor imaging of brain white matter in Huntington gene mutation individuals

Directory of Open Access Journals (Sweden)

Roberta Arb Saba

Full Text Available ABSTRACT Objective To evaluate the role of the involvement of white matter tracts in huntingtin gene mutation patients as a potential biomarker of the progression of the disease. Methods We evaluated 34 participants (11 symptomatic huntingtin gene mutation, 12 presymptomatic huntingtin gene mutation, and 11 controls. We performed brain magnetic resonance imaging to assess white matter integrity using diffusion tensor imaging, with measurement of fractional anisotropy. Results We observed a significant decrease of fractional anisotropy in the cortical spinal tracts, corona radiate, corpus callosum, external capsule, thalamic radiations, superior and inferior longitudinal fasciculus, and inferior frontal-occipital fasciculus in the Huntington disease group compared to the control and presymptomatic groups. Reduction of fractional anisotropy is indicative of a degenerative process and axonal loss. There was no statistically significant difference between the presymptomatic and control groups. Conclusion White matter integrity is affected in huntingtin gene mutation symptomatic individuals, but other studies with larger samples are required to assess its usefulness in the progression of the neurodegenerative process.

National Register Historic Districts

Data.gov (United States)

Iowa State University GIS Support and Research Facility — The National Register Historic District layer is a shape file showing the boundaries of Historic Districts that are listed on the National Register of Historic Places.

The Need to Increase Marine Corps Special Operations Command

Science.gov (United States)

2009-01-01

Operations University presentation by Major Mark Raney for USSOCOM elective (lecture, MCU, Quantico VA, 17 Feb 20Q9). 5 Stew Magnuson, "Marine Special...2008 Posture Statement.doc (Accessed 23 December, 2008) 20 David Tucker and Christopher J. Lamb , United States Special Operations Forces (New York...invasion of Afghanistan encroached on the Marine Corps historical role of being "soldiers from the sea." 38 David Tucker and Christopher J. lamb , 182

DEPRESSION, ANXIETY, STRESS, AND THEIR ASSOCIATED FACTORS AMONG CORPS MEMBERS SERVING IN KEBBI STATE.

Science.gov (United States)

Balami, Ahmed D

2015-01-01

Depression, anxiety and stress, are not only health problems by themselves, but also associated with other negative health consequences. The national youth service is usually characterized by a number of new challenges and experiences which may require life style adjustments by the corps member. However, no previous study on psychological factors has been conducted among corps members. This study was conducted to determine the prevalence of depression, anxiety and, stress and their associated factors among corps members serving in Kebbi state. A cross-sectional study was conducted among 264 corps members from four local government areas of the state. Selection of the local government areas and the individual participants was by simple random sampling. Data was collected from May to June 2014 using a self-administered questionnaire. Data analysis used chi-square test to identify the relationship between categorical variables and multivariate logistic regression to identify the independent factors for depression, anxiety and stress each. The response rate was 97%. Most of the respondents were males (63.6%), single (85.5%), and above 20 years of age (71.6%). The overall prevalences of depression, anxiety and stress among the respondents were 36.4%, 54.5% and 18.2% respectively. The independent factors for depression were; being from the North central (OR = 5.99; 95% CI: 2.194-16.354) or South-south; and the perception of earning enough income (OR = 2.987; 95% CI: 1.062-8.400). For anxiety, male gender (OR = 0.411; 95% CI: 0.169-0.999); and being from the North central were significant risk factors (OR = 3.731; 95% CI: 1.450-9.599). Being above 26 years of age was an independent risk factor for stress (OR = 0.083; 95% CI: 0.018-0.381). Also, those who had ever schooled outside their towns of residence were less likely to be stressed compared to those who had never (OR = 0.30; 95% CI: 0.110-0.855). All other factors did not show any significant association with any of

The Partnership Pact: Fulfilling School Districts' Research Needs with University-District Partnerships

Science.gov (United States)

Ralston, Nicole; Weitzel, Bruce; Waggoner, Jacqueline; Naegele, Zulema; Smith, Rebecca

2016-01-01

There has been a recent shift in university-district partnership models from traditional transactional partnerships, which lack a shared purpose, to transformational partnerships that are mutually beneficial to both universities and school districts. These transformational research-practice partnerships have gained popularity in the United States…

PPARGC1A/PGC-1α, TFEB and enhanced proteostasis in Huntington disease: Defining regulatory linkages between energy production and protein–organelle quality control

OpenAIRE

La Spada, Albert R.

2012-01-01

Huntington disease (HD) results from CAG repeats that encode expanded polyglutamine tracts in the HTT/huntingtin protein. HD belongs to a large category of inherited and sporadic neurodegenerative disorders in which production of a misfolded protein initiates the pathogenic cascade. Previous studies have shown that misfolded proteins become resistant to cellular protein turnover pathways by eluding and disabling the ubiquitin-proteasome system (UPS) and autophagy-lysosome pathway. Based upon ...

75 FR 10808 - CIBA Vision Corp.; Withdrawal of Color Additive Petitions

Science.gov (United States)

2010-03-09

...) proposing that the color additive regulations be amended to provide for the safe use of Color Index (C.I... of Color Additives Exempt from Certification (21 CFR part 73) to provide for the safe use of C.I...) CIBA Vision Corp.; Withdrawal of Color Additive Petitions AGENCY: Food and Drug Administration, HHS...

From Four to Two: Transformation of the Army Ordnance Officer and Warrant Officer Corps

National Research Council Canada - National Science Library

Napier, Joyce

2003-01-01

.... This study will examine how senior leaders within the Army and specifically the Ordnance Corps must change the officer and warrant officer force structure education system and leader development...

2005 United States Army Corps of Engineers (USACE) Post-Hurricane Katrina Levee Surveys

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — These topographic data were collected for the U.S. Army Corps of Engineers by a helicopter-mounted LiDAR sensor over the New Orleans Hurricane Protection Levee...

Marine Corps Joint Officer Management Policy and O-7 Joint Service Officer Requirements

National Research Council Canada - National Science Library

Eck, Larry R

2007-01-01

.... The research was conducted at the request of Manpower and Reserve Affairs, Headquarters, U.S. Marine Corps to review current policies and their effectiveness in supporting the requirements in the FY 2005 National Defense Authorization Act...

District Fiscal Policy and Student Achievement

Directory of Open Access Journals (Sweden)

Gary G. Huang

2002-09-01

Full Text Available School restructuring raises questions about the role of school districts in improving student learning. Centralization by state governments and decentralization to individual schools as proposed in systemic reform leave districts' role unsettled. Empirical research on the district role in the context of ongoing reform is inadequate. This analysis of combined data from the NAEP and the Common Core of Data (CCD was intended to address the issue. We analyzed 1990, 1992, and 1996 NAEP 8th grade mathematics national assessment data in combination with CCD data of corresponding years to examine the extent to which student achievement was related to districts' control over instructional expenditure, adjusting for relevant key factors at both district and student levels. Upon sample modification, we used hierarchical linear modeling (HLM to estimate the relationships of student achievement to two district fiscal policy indictors, current expenditure per pupil (CEPP and districts' discretionary rates for instructional expenditure (DDR. Net of relevant district factors, DDR was found unrelated to districts' average 8th grade math performance. The null effect was consistent in the analysis of the combined NAEP-CCD data for 1990, 1992, and 1996. In contrast, CEPP was found related to higher math performance in a modest yet fairly consistent way. Future research may be productive to separately study individual states and integrate the findings onto the national level.

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.

Complexity Leadership Theory: A United States Marine Corps Historical Overlay

Science.gov (United States)

2017-05-25

DD-MM-YYYY) 23-04-2017 2. REPORT TYPE Master’s Thesis 3. DATES COVERED (From - To) JUN 2016 – MAY 2017 4. TITLE AND SUBTITLE Complexity...Monograph Title : Complexity Leadership Theory : A United States Marine Corps Historical Overlay Approved by...General Ridgway’s Success in Korea.” Master’s thesis , United States Army Command and General Staff College, 2010. Diana, Gabriel. “Vision, Education