线粒体自噬及功能障碍与帕金森病

Mitochondrial autophagy and dysfunction in Parkinson’s disease

帕金森病(Parinson’s disease,PD)是全球第二大中枢神经系统退行性疾病,其病理特征主要是黑质中a-突触核蛋白(a-synuclein,a-syn)的聚集,其早期征兆是线粒体功能紊乱。PD相关蛋白PINK1、Parkin、DJ-1蛋白和a-syn等参与线粒体自噬及质量控制过程,PD相关蛋白突变时导致线粒体自噬及其功能异常,从而出现受损线粒体不能被选择性清除和线粒体功能障碍的现象,最终可能造成多巴胺能神经元的死亡。因此PD相关蛋白的失控与PD的发生密切相关,本文将对线粒体自噬及功能障碍与帕金森病的关系做一综述。

Parkinson’s disease(PD)is the second most common central nervous system degenerative disease worldwide.One pathological feature is a-synuclein accumulation in the substantia nigra,and an early sign is mitochondrial dysfunction.The PD-related proteins PINK1,Parkin,DJ-1,and a-synuclein are involved in the process of mitochondrial autophagy and quality control.The mutation of PD-related proteins leads to abnormal autophagy and mitochondrial function.A failure of selective clearance of damaged mitochondria and mitochondrial dysfunction may eventually lead to dopaminergic neuron death.Therefore,the loss of control of PD-related proteins is closely related to PD occurrence.This article reviews the associations between mitochondrial autophagy,dysfunction,and Parkinson’s disease.