Ontology highlight
ABSTRACT:
SUBMITTER: Miller BR
PROVIDER: S-EPMC3184508 | biostudies-literature | 2010 Sep
REPOSITORIES: biostudies-literature
Miller Benjamin Ray BR Bezprozvanny Ilya I
Future neurology 20100901 5
Huntington's disease (HD) is a noncurable and progressive autosomal-dominant neurodegenerative disorder that results from a polyglutamine expansion in the amino-terminal region of the huntingtin protein. The generation of rodent HD models has revealed that cellular dysfunction, rather than cell death alone, occurs early in the disease progression, appearing even before overt symptom onset. Much evidence has now established that dysfunction of the corticostriatal circuit is key to HD symptomology ...[more]