自噬-溶酶体途径和泛素-蛋白酶体系统在肌萎缩侧索硬化发病中的作用  

Role of Macroautophagy and Ubiquitin-proteasome System in Amyotrophic Lateral Sclerosis

作　　者：宋林[1] 张晓洁[1] 乐卫东[1] 

机构地区：[1]上海交通大学附属瑞金医院神经内科,200025

出　　处：《中国临床神经科学》2011年第6期657-660,共4页Chinese Journal of Clinical Neurosciences

摘　　要：肌萎缩侧索硬化(ALS)是常见的成人运动神经元疾病。迄今为止,ALS的病因及发病机制尚未完全明确,但是越来越多的证据表明蛋白的错误折叠和清除障碍参与了包括ALS在内的多种神经变性疾病的发生与进展。自噬-溶酶体途径(ALP)和泛素-蛋白酶体系统(UPS)是机体修复或消除异常蛋白质的两种最主要的途径,在神经变性疾病中发挥着重要作用。两者都参与细胞的分化、发育和凋亡,贯穿于细胞生长发育和病理生理过程,任何一种系统受损都会导致细胞功能障碍甚至死亡。本文主要对自噬和UPS在ALS发病中的作用予以综述。Amyotrophic lateral sclerosis（ALS） is the most common adult-onset motor neuron disease.Although the etiology and pathomechanism underlying ALS remain obscure so far,there is increasing evidence that misfolded or impaired cellular proteins is involved in the pathogenesis of various neurodegenerative diseases,including ALS.Autophagy-lysosome pathway（ALP） and ubiquitin-proteasome system（UPS） are the two major systems for degradation of aberrant proteins,which play an important part in neurodegenerative diseases.Both of them take part in the differentiation,maturation and apoptosis of cells.Neither of them injured can cause the deterioration of various cellular functions,even lead to neuronal cell death.This review mainly discussed the role of macroautophagy and ubiquitin-proteasome system in ALS.

关 键 词：肌萎缩侧索硬化 自噬 泛素-蛋白酶体系统 蛋白沉积 

分 类 号：R744.8[医药卫生—神经病学与精神病学]