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

ABSTRACT: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder characterized by motor, cognitive and psychiatric manifestations. Since the mutation responsible for the disease was identified as an unstable expansion of CAG repeats in the gene encoding the huntingtin protein in 1993, numerous mouse models of HD have been generated to study disease pathogenesis and evaluate potential therapeutic approaches. Of these, knock-in models best mimic the human condition from a genetic perspective since they express the mutation in the appropriate genetic and protein context. Behaviorally, however, while some abnormal phenotypes have been detected in knock-in mouse models, a model with an earlier and more robust phenotype than the existing models is required. We describe here for the

SUBMITTER: Menalled LB 

PROVIDER: S-EPMC3527464 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature