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蛋白质错误折叠与神经退行性疾病
引用本文:王晔,顾振纶,秦正红.蛋白质错误折叠与神经退行性疾病[J].中国临床神经科学,2005,13(4):422-426.
作者姓名:王晔  顾振纶  秦正红
作者单位:苏州大学医学院基础医学系药理教研室,215123
摘    要:许多神经退行性疾病的发生是由蛋白质错误折叠引起的,这类疾病被称为蛋白质错误折叠病。蛋白质突变、泛素-蛋白酶和自噬功能的失常与蛋白质错误折叠的发生,异常蓄积和聚集有关。本文综述了蛋白质错误折叠和聚集的机制和神经退行性疾病的有关研究进展。

关 键 词:蛋白质错误折叠  分子伴侣  泛素-蛋白酶系统  神经退行性疾病  聚集体
文章编号:1008-0678(2005)04-0422-05
修稿时间:2005年4月8日

Protein Misfolding and Neurodegenerative Diseases
WANG Ye,GU Zhen-Lun,QIN Zheng-Hong.Protein Misfolding and Neurodegenerative Diseases[J].Chinese Journal of Clinical Neurosciences,2005,13(4):422-426.
Authors:WANG Ye  GU Zhen-Lun  QIN Zheng-Hong
Institution:WANG Ye,GU Zhen-Lun,QIN Zheng-Hong Dept.of Pharmacology,School of Medicine,Soochow University,Suzhou 215123,China
Abstract:Several neurodegenerative diseases are believed to be caused by protein misfolding.Thus these diseases are also called protein misfolding diseases.Gene mutation,dysfunction of ubiquitin-proteasome system(UPS) and autophagy play important roles in protein misfolding,abnormal accumulation and aggregation of misfolded proteins.We reviewed the recent progress in mechanisms of protein misfolding and aggregation and the role of protein misfolding in the pathogenesis of neurodegenerative diseases.
Keywords:protein misfolding  molecular chaperone  ubiquitin-proteasome system  neurodegenerative disease  aggregates  
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