Huntington DiseaseHyperkinesisAgedMiddle AgedCorpus StriatumFemaleA morphometric method was employed to investigate the relationship of rigidity and hyperkinesia to the degree of striatal and nigral nerve cell loss in one patient with the rigid variant of Huntington's disease and four patients with ...
In the present experiments we evaluate the functional viability of one particular cell line, the hNT cells developed by Layton Bioscience, to replace lost neurons and alleviate asymmetrical motor deficits in a unilateral excitotoxic lesion model of Huntington's disease. Because the grafts involved ...
Huntingtons disease (HD) is a fatal neurodegenerative disease characterized by abnormal motor coordination, cognitive decline and psychiatric disorders. This disease is caused by an expanded CAG trinucleotide repeat in the gene encoding the protein huntingtin. Reduced levels of brain-derived neurotrophic ...
Less tried is an approach involving direct PERK activation without compromising long-term recovery of eIF2伪 function by dephosphorylation. Here we show major improvement in cellular (ST) and mouse (R6/2) Huntington's disease (HD) models using a potent small molecule PERK activator that we ...
This mutation creates an expanded polyglutamine tract in the Huntingtin protein (HTT) and results in a mutant form of HTT (mHTT) that disrupts multiple cellular functions (The Huntington’s Disease Collaborative Research Group, 1993). Pathologically, HD is characterized by profound neuronal death in...
Graphical analysis of the predicted age of motor diagnosis showed an expected strong relationship with the trinucleotide expansion that causes Huntington’s disease. Graphical analysis of the deviance-type residual revealed there were individuals who converted to a diagnosis despite having relatively low ...
The circadian disruption seen in patients of Huntington's disease (HD) is recapitulated in the R6/2 mouse model. As the disease progresses, the activity of R6/2 mice increases dramatically during the rest (light) period and decreases during the active (dark) period, eventually leading to a ...
Survival of Huntington's disease patients in Serbia: longer survival in female patients. Eur J Epidemiol. 2007;22:523-6.Pekmezovic T, Svetel M, Maric J, Dujmovic-Basuroski I, Dragasevic N, Keckarevic M, Romac S, Kostic VS. Sur- vival of Huntington's disease patients in Serbia: ...
diseasesurvivalanalysisBackground and aims The precise knowledge of the natural history of Huntington's disease (HD) and the impact of variables that may modify the rate of survival are essential for counselling of patients and design of future therapeutic trials. Failure to use survival analyses ...
The precise knowledge of the natural history of Huntington's disease (HD) and the impact of variables that may modify the rate of survival are essential for counselling of patients and design of future therapeutic trials. Failure to use survival analyses methodology in previous studies may have ...