Moore Darren J, West Andrew B, Dawson Valina L, Dawson Ted M
Institute for Cell Engineering, Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
Annu Rev Neurosci. 2005;28:57-87. doi: 10.1146/annurev.neuro.28.061604.135718.
Parkinson's disease (PD) is a progressive neurodegenerative movement disorder that results primarily from the death of dopaminergic neurons in the substantia nigra. Although the etiology of PD is incompletely understood, the recent discovery of genes associated with rare monogenic forms of the disease, together with earlier studies and new experimental animal models, has provided important and novel insight into the molecular pathways involved in disease pathogenesis. Increasing evidence indicates that deficits in mitochondrial function, oxidative and nitrosative stress, the accumulation of aberrant or misfolded proteins, and ubiquitin-proteasome system dysfunction may represent the principal molecular pathways or events that commonly underlie the pathogenesis of sporadic and familial forms of PD .
帕金森病(PD)是一种进行性神经退行性运动障碍,主要由黑质中多巴胺能神经元的死亡所致。尽管PD的病因尚未完全明确,但最近发现的与该疾病罕见单基因形式相关的基因,连同早期研究和新的实验动物模型,为疾病发病机制中涉及的分子途径提供了重要且新颖的见解。越来越多的证据表明,线粒体功能缺陷、氧化和亚硝化应激、异常或错误折叠蛋白质的积累以及泛素-蛋白酶体系统功能障碍可能是散发性和家族性PD发病机制共同的主要分子途径或事件。