Abstract:Huntington’ s disease ( HD) is an inherited autosomal dominant and progressive neurodegenerative disease with locomotor, cognitive, and psychiatric functional deficits. Currently, there is no cure for HD, and the clinical benefit is greatly limited for some treatments available for controlling HD symptoms. In 1993, it was discovered that HD is caused by a single Huntingtin gene (HTT) mutation with 36 CAG repeats and or more, which translates to a mutated polyglutamine, Resulting in neuronal excitotoxicity and abnormal mitochondrial energy metabolism, and eventually neurodegeneration in the cerebral cortex and basal nuclei. To better understand the pathogenesis mechanisms of HD and explore therapeutic interventions, it is important to create HD animal models. In this review, we will introduce HD animal models based on neuropathology, and explore the molecular biology of HD such as neural excitotoxicity and mitochondrial toxicity.