亨廷顿舞蹈症实验动物模型研究进展  

Research progress on experimental animal models of Huntington’s disease

作　　者：付铄 张雯 宋俊科 杜冠华 FU Shuo;ZHANG Wen;SONG Junke;DU Guanhua(Beijing Key Laboratory of Drug Target and Screening Research,Institute of Materia Medica,ChineseAcademy of Medical Sciences and Peking Union Medical College,Beijing 100050,China)

机构地区：[1]中国医学科学院药物研究所,北京协和医学院,药物靶点研究与新药筛选北京市重点实验室,北京100050

出　　处：《中国实验动物学报》2024年第8期1065-1076,共12页Acta Laboratorium Animalis Scientia Sinica

基　　金：国家重点研发计划(2020YFC2008302);中国医学科学院医学与健康科技创新工程重大协同创新项目(2022-I2M-1-015)。

摘　　要：亨廷顿舞蹈症(Huntington’s disease,HD)是一类常染色体显性遗传的神经退行性疾病,患者主要症状为舞蹈状的不自主运动、精神行为异常和认知障碍,严重影响患者的生活,消耗大量社会医疗资源。为了更好地理解其病理机制并探索治疗方法,已有多种HD实验动物模型被成功构建。本文概述了从秀丽隐杆线虫、果蝇、斑马鱼到小鼠、大鼠和小型猪等多种动物模型的建立与应用情况,并对不同动物模型的特点和优势进行了分析。通过综述不同动物模型及其相关评价指标,本文强调了综合运用多种动物模型的重要性,以期深入理解疾病的机制并制定有效的治疗策略。Huntington’s disease(HD)is an autosomal dominant neurodegenerative disease,with the main symptoms including chorea-like involuntary movements,psychiatric behavioral abnormalities,and cognitive impairment,which severely affect the lives of patients and consume extensive social and medical resources.Various experimental animal models of HD have been successfully established,to further our understanding of the pathological mechanisms and to explore treatment method of HD.This review outlines the establishment and application of various animal models,ranging from Caenorhabditis elegans,Drosophila melanogaster and zebrafish to mice,rats and miniature pigs,and analyzes the characteristics and advantages of the different models.By reviewing the different animal models and their relevant evaluation indicators,this article emphasizes the importance of utilizing a combination of multiple animal models to promote a deeper understanding of the disease mechanisms and develop effective treatment strategies.

关 键 词：亨廷顿舞蹈症 动物模型 模型特点 评价指标 

分 类 号：Q95-33[生物学—动物学]