Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder. In 1993 the mutation that causes HD was identified as an unstable expansion of CAG repeats in the IT15 gene. Since then one of the most important advances in HD research has been the generation of various mouse models that enable the exploration of early pathological, molecular and cellular abnormalities produced by the mutation. In addition, these models have made it possible to test different pharmacological approaches to delay the onset or slow the progression of HD. In this article, insights gained from mouse models towards the understanding of HD and the design of new therapeutic strategies are discussed.

中文翻译：

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亨廷顿舞蹈症的小鼠模型。

亨廷顿舞蹈病（HD）是常染色体显性遗传神经退行性疾病。在1993年，导致HD的突变被鉴定为IT15基因中CAG重复序列的不稳定扩增。从那时起，HD研究最重要的进展之一就是生成了多种小鼠模型，这些模型能够探索由突变产生的早期病理，分子和细胞异常。此外，这些模型使测试不同的药理方法以延缓HD的发作或减慢其进展成为可能。在本文中，将讨论从小鼠模型获得的对HD的理解和新治疗策略设计的见解。