Current understanding on the pathogenesis of polyglutamine diseases

作     者：何晓辉 林芳 秦正红 

作者机构：苏州大学医学部药理学系衰老与神经疾病实验室苏州215123 

出 版 物：《Neuroscience Bulletin》 (神经科学通报（英文版）)

年 卷 期：2010年第26卷第3期

页      面：247-256页

核心收录：

学科分类：1002[医学-临床医学] 100204[医学-神经病学] 10[医学] 

基　　金：supported by the grants from the National Natural Science Foundation of China(No.30600197) the Specialized Research Fund for the Doctoral Program of Higher Education of China(No.20050285017) 

主　　题：polyglutamine the central nervous system neurodegenerative diseases late-onset disorders ubiquitin autophagy 

摘      要：Polyglutamine （polyQ） diseases are a family of neurodegenerative disorders including Huntington’s disease, spinobulbar muscular atrophy,dentatorubral-pallidoluysian atrophy and several spinocerebellar *** diseases are caused by abnormal expansion of CAG repeats in certain *** expanded CAG repeats are then translated into a series of abnormally expanded polyQ *** polyQ tracts may induce misfolding of the disease-causing *** present review mainly focuses on the common characteristics of the pathogenesis of these polyQ diseases,including conformational transition of proteins and its influence on the function of these proteins,the correlation between decreased ability of proteoly-sis and late-onset polyQ diseases,and the relationship between wide expression of disease-causing proteins and selective neuronal death.