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2008•Zhongguo shengwu huaxue yu fenzi shengwu xuebaoRequires access

Cu,Zn-superoxide Dismutase Associated with Amyotrophic Lateral Sclerosis

Chuang Qiu

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Abstract

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.

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What this paper is about

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.

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Available abstract

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.

Key concepts: SOD1, Amyotrophic lateral sclerosis, Superoxide dismutase, Dismutase, Chaperone (clinical), Chemistry, In vivo, Biochemistry

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