Huntington disease

JPG Vonsattel, M DiFiglia - Journal of neuropathology and …, 1998 - search.proquest.com
Huntington disease (HD) is an autosomal dominant neurodegenerative disorder with midlife
onset characterized by psychiatric, cognitive, and motor symptoms. Death occurs between …

Oxidative stress in Huntington's disease

SE Browne, RJ Ferrante, MF Beal - Brain pathology, 1999 - Wiley Online Library
It has been five years since the elucidation of the genetic mutation underlying the
pathogenesis of Huntington's disease (HD)(97), however the precise mechanism of the …

Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175

LB Menalled, AE Kudwa, S Miller, J Fitzpatrick… - PloS one, 2012 - journals.plos.org
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder
characterized by motor, cognitive and psychiatric manifestations. Since the mutation …

Regional and cellular gene expression changes in human Huntington's disease brain

A Hodges, AD Strand, AK Aragaki… - Human molecular …, 2006 - academic.oup.com
Huntington's disease (HD) pathology is well understood at a histological level but a
comprehensive molecular analysis of the effect of the disease in the human brain has not …

Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity

FC Nucifora Jr, M Sasaki, MF Peters, H Huang… - Science, 2001 - science.org
Expanded polyglutamine repeats have been proposed to cause neuronal degeneration in
Huntington's disease (HD) and related disorders, through abnormal interactions with other …

Genetics and neuropathology of Huntington's disease

A Reiner, I Dragatsis, P Dietrich - International review of neurobiology, 2011 - Elsevier
Huntington's disease (HD) is an autosomal dominant progressive neurodegenerative
disorder that prominently affects the basal ganglia, leading to affective, cognitive, behavioral …

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease

CL Wellington, LM Ellerby, CA Gutekunst… - Journal of …, 2002 - Soc Neuroscience
Huntington's disease (HD) results from polyglutamine expansion in huntingtin (htt), a protein
with several consensus caspase cleavage sites. Despite the identification of htt fragments in …

Structural and functional evolution of the basal ganglia in vertebrates

A Reiner, L Medina, CL Veenman - Brain research reviews, 1998 - Elsevier
While a basal ganglia with striatal and pallidal subdivisions is1 clearly present in many
extant anamniote species, this basal ganglia is cell sparse and receives only a relatively …

Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways

J Velier, M Kim, C Schwarz, TW Kim, E Sapp… - Experimental …, 1998 - Elsevier
Huntingtin is a cytoplasmic protein that is found in neurons and somatic cells. In patients with
Huntington's disease (HD), the NH2-terminal region of huntingtin has an expanded …

Huntingtin localization in brains of normal and Huntington's disease patients

E Sapp, C Schwarz, K Chase, PG Bhide… - Annals of Neurology …, 1997 - Wiley Online Library
The immunohistochemical localization of huntingtin was examined in the Huntington's
disease (HD) brain with an antibody that recognizes the wild‐type and mutant proteins …