Institute of Health Science, Shanghai Institutes for Biological Sciences of Chinese Academy of Sciences and Shanghai Jiaotong University School of Medicine, Shanghai 200025, China; Department of Neurology and Institute of Neurology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200025, China; E-mail:
Neurosci Bull. 2006 Jul;22(4):232-4.
Parkinson's disease (PD) is a slowly progressive neurodegenerative disorder characterized clinically by bradykinesia, rigidity, tremor, gait dysfunction, and postural instability. Several genes have been identified for monogenic disorders that variably resemble Parkinson's disease. Here, we focus on PARK7, a gene relates to an autosomal recessive form of early-onset Parkinsonism and encodes a protein named DJ-1. Though the exact role of DJ-1 needs to be elucidated, it is generally thought to be functioned as a molecular chaperone and an oxidative sensor (or antioxidative factor). We will review the protective role of DJ-1 to prevent dopaminergic neurons in the substantia nigra pars compacta (SNpc) from degeneration and how its dysfunction would lead to neurodegeneration.
帕金森病(PD)是一种进行性神经退行性疾病,临床上表现为运动迟缓、肌肉强直、震颤、步态异常和姿势不稳。已经发现了几个与单基因疾病相关的基因,这些疾病在不同程度上与帕金森病相似。在这里,我们重点介绍 PARK7 基因,它与早发性帕金森病的常染色体隐性形式有关,编码一种名为 DJ-1 的蛋白质。虽然 DJ-1 的确切作用尚需阐明,但它通常被认为是一种分子伴侣和氧化传感器(或抗氧化因子)。我们将回顾 DJ-1 的保护作用,以防止黑质致密部(SNpc)中的多巴胺能神经元退化,以及其功能障碍如何导致神经退行性变。