Abstract:Huntington disease (HD) is a typical hereditary neurodegenerative disease caused by single HTT gene mutation, and a CAG repeat of >36 in the HTT gene may lead to HD. HD patients are characterized by the accumulation of variant proteins and neuronal death, with behavioral, cognitive, and mental disorders. Clear etiology makes HD an ideal model for the analysis of neurodegenerative diseases. This article briefly reviews the achievements in animal models of HD, in order to provide ideas for the research on such neurodegenerative diseases.