肌萎缩性侧索硬化症相关的Cu,Zn-SOD

超氧化物歧化酶（superoxide dismutase，SOD）被称为生物体内自由基的清洁剂，其主要形式Cu,Zn-SOD称SOD1. SOD1突变体可引起致死性运动神经元疾病肌萎缩性侧索硬化症(ALS).但是,SOD1的毒性机理尚未完全清楚.本文概述了SOD1、Cu分子伴侣（copper chaperone for SOD1，CCS）的分子结构和CCS活化SOD1的机理，重点分析了突变体SOD1构象变化的原因及其在ALS中的可能致病机制的最新研究进展.

Cu,Zn-superoxide Dismutase Associated with Amyotrophic Lateral Sclerosis

Superoxide dismutases(SOD)are known as biological cleaning agents of free radicals in vivo,and Cu,Zn-SOD also called SOD1,is one of the main forms.SOD1 mutations have been linked fatal human motor neuron diseases,such as amyotrophic lateral sclerosis,ALS.However,the precise roles of SOD1 in ALS occurenace and progression remianed unknown.This review will focus on the mechanism of SOD1's activation based on the structural conformation analysis of SOD1 and the copper chaperone for SOD1(CCS).Meanwhile,the latest research progresses of the possible pathogenic mechanisms in ALS will also be discussed.