《生命科学》 2010, 22(10): 1009-1012

Parkin、PINK1、DJ-1和线粒体功能障碍与帕金森病

杨　辉，左　伋，刘　雯*

(复旦大学上海医学院细胞与遗传医学系，上海200032)

摘　要：

摘　要：帕金森病(Parkinson抯 disese， PD)是一种常见的神经退行性疾病，但到目前为止发病机制尚不明确，环境和遗传等因素与其发病有密切关系。研究表明，蛋白质异常积聚(泛素/蛋白酶体途径)和线粒体氧化损伤(线粒体途径)，可能是导致PD患者发病的关键分子机制。Parkin、PINK1和DJ-1等基因突变与常染色体隐性的家族性PD有关，这些相关基因编码的蛋白对于维持线粒体形态和功能起着重要的作用。本文将主要从Parkin、PINK1、DJ-1和线粒体功能障碍与帕金森病的关系进行综述。
关键词：Parkin；PINK1；DJ-1；线粒体功能障碍；帕金森病
中图分类号：R742.5   文献标识码：A

    

 

Parkin， PINK1， DJ-1 and mitochondria dysfunction

YANG Hui， ZUO Ji， LIU Wen*

(Department of Cellular and Genetic Medicine， Shanghai Medical College， Fudan University， Shanghai 200032， China)

Abstract:

Abstract: Parkinson{$39}s disease (PD) is a common neurodegenerative disease， but so far its pathogenesis remains unclear， environmental and genetic factors are closely related to the disease. Recent studies have shown that the accumulation of abnormal proteins (ubiquitin/proteasome pathway) and mitochondrial oxidative damage (mitochondrial pathway) may lead to the impairments of dopaminergic neurons in PD patients. Mutations of Parkin， PINK1 (PTEN-induced kinase 1) and DJ-1 have been found in autosomal recessive PD. The three proteins encoded by Parkin， PINK1 and DJ-1 play an important role in improving the mitochondrial functions. This review will focus on Parkin， PINK1， DJ-1 and mitochondrial dysfunction as well as overview of progress made in recent years. 
Key words: Parkin; PINK1; DJ-1; Mitochondria dysfunction; Parkinson{$39}s disease