亨廷顿病模型进展及展望

Animal models of Huntington disease: Research advances and perspectives

摘要:

亨廷顿病（Huntington disease，HD）是一种典型的神经退行性疾病。该病是由单基因HTT突变导致的遗传性疾病，当CAG重复序列>36时发病，HD患者出现变异蛋白的累积和神经元死亡病理特征，伴随着行为、认知、精神方面的障碍。其清晰的病因使得HD成为研究神经退行性疾病的首选模式疾病。本文将对现有HD动物模型取得的成果进行简单综述，以期为此类神经退行性疾病的研究提供思路。

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.