帕金森病相关基因DJ-1与氧化应激

摘要

Parkinson’s disease is characterized by the substantia nigra pars compacta dopaminergic neuron-specific death and the presence of eosinophilic inclusion bodies-Lewy bodies which’s main component isα- synuclein in residual neuronal. Recent studies show that oxidative stress is a leading cause of brain dopaminergic neuron-specific death. DJ-1(Park7)gene is an autosomal recessive Parkinson’s disease related genes. DJ-1 protein has a strong antioxidant effect,mainly acts as identity of active oxygen scavenger,chaperones,signaling pathways regulating molecules to against the oxidative stress,thereby protecting dopamine neurons from damage. Both deletion and mutation of DJ-1 gene will cause the reduction of its anti-oxidative stress,resulting in neuronal death,and this situation may be rescued by over-expression of DJ-1 protein. Because of the important role of DJ-1 in the development and occurrence of Parkinson’s disease,the paper will expand on its review of new research,and discuss primarily from the relationship between DJ-1 and oxidative stress.%帕金森病主要以中脑黑质致密部多巴胺能神经元特异性死亡以及残余神经元内呈现以α-突触共核蛋白（α-synuclein）为主要成分的嗜酸性包涵体－路易小体（Lewy body，LD）为主要特征。近年来研究表明氧化应激是引起中脑多巴胺能神经元特异性死亡的主要原因。DJ-1（Park7）基因是与帕金森病相关的一种常染色体隐性基因。DJ-1蛋白具有强抗氧化作用，主要以活性氧清除剂、分子伴侣、信号通路调节分子等身份发挥抗氧化应激功能，从而保护多巴胺能神经元免受损伤。DJ-1的基因缺失或突变均会造成其抗氧化应激功能降低，导致神经元死亡，且此状况可由过表达DJ-1蛋白缓解。鉴于DJ-1在帕金森病发生发展中的重要作用，该文将就其最新研究展开综述，并主要侧重其与氧化应激之间的关系进行深入地探讨。