Current opinion in neurology, 8(6), 469-473 (1995-12-01)
Compelling evidence suggests that a defect in energy metabolism may play a role in the pathogenesis of various degenerative disorders including Parkinson's disease and Huntington's disease. The behavioural and neuropathological consequences in primates of chronic systemic administration of mitochondrial toxins
Huntington's disease (HD) is a progressive neurodegenerative disorder with a spectrum of cognitive, behavioral, and motor abnormalities. The mitochondrial toxin 3-nitropropionic acid (3-NP) effectively induces specific behavioral changes, primarily manifested as prepulse inhibition (PPI) deficit of acoustic startle stimuli, and
Progress in neurobiology, 72(2), 87-110 (2004-04-06)
Huntington's disease (HD) is an inherited neurodegenerative disease, in which there is progressive motor and cognitive deterioration, and for which the pathogenesis of neuronal death remains controversial. Mitochondrial toxins like 3-nitropropionic acid (3-NP) and malonate, functioning as the inhibitors of
Journal of neurochemistry, 95(6), 1521-1540 (2005-11-23)
Huntington's disease (HD) is a neurodegenerative disorder caused by a mutation in the gene encoding Huntingtin. The mechanisms underlying the preferential degeneration of the striatum, the most striking neuropathological change in HD, are unknown. Of those probably involved, mitochondrial defects
Neuroscience and biobehavioral reviews, 21(3), 289-293 (1997-05-01)
Huntington's disease (HD) is a progressive neurodegenerative disorder associated with severe degeneration of basal ganglia neurons, especially the intrinsic neurons of the striatum, and characterized by progressive dementia and involuntary abnormal choreiform movements. Despite our increasing knowledge of the pathophysiology
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