线粒体功能障碍、α-突触核蛋白与帕金森病

Mitochondrial dysfunction,α-synuclein and Parkinson's disease

帕金森病(Parkinson’s disease,PD)是以中脑黑质多巴胺能神经元缺失,黑质残存神经元内出现路易小体为主要病理改变的神经退行性疾病。越来越多的证据显示线粒体复合体Ⅰ功能障碍是散发性PD的核心发病机制,α-突触核蛋白(α-synuclein,α-Syn)异常聚集是PD病理学级联反应的关键事件。近年来,α-Syn与线粒体功能障碍的关系尤其引人注意,但至今对它们在PD中的作用机制并不完全清楚,二者间的相互作用尚不明确。本文综述了α-Syn与线粒体在PD发病中的关键作用以及二者在PD发病中的相互作用。对α-Syn及线粒体在PD发病中发挥作用的机制深入了解将对研发新的PD治疗方法具有重要意义。

Parkinson＇s disease（ PD） is a common neurodegenerative disorder. The disease is characterized by the loss of dopamine neurons in the substantia nigra and the presence of intraneuronal Lewy bodies. Many studies suggest that mitochondrial dysfunction is the central pathogenesis of PD. The abnormal aggregation of α-synuclein is the key factor in the cascade of PD pathological events. Recently,the interaction between α-synuclein and mitochondrial dysfunction has attracted much attention,but the exact mechanism underlying PD pathogenesis is still unclear. In this review,we discuss the key roles of mitochondria and α-synuclein in the pathogenesis of PD and the interaction between them. Understanding the functional consequences of α-synuclein＇s interactions with mitochondria is likely to provide important insights into disease pathophysiology,and may also reveal therapeutic strategies.