Vall d'Hebron Research Institute-CIBERNED, Barcelona, Spain.
Vall d'Hebron Research Institute-CIBERNED, Barcelona, Spain Catalan Institution for Research and Advanced Studies (ICREA), Barcelona, Spain Department of Biochemistry and Molecular Biology, Autonomous University of Barcelona, Barcelona, Spain.
Neuroscientist. 2016 Jun;22(3):266-77. doi: 10.1177/1073858415574600. Epub 2015 Mar 11.
Parkinson's disease is a common, adult-onset neurodegenerative disorder whose pathogenesis is still under intense investigation. Substantial evidence from postmortem human brain tissue, genetic- and toxin-induced animal and cellular models indicates that mitochondrial dysfunction plays a central role in the pathophysiology of the disease. This review discusses our current understanding of Parkinson's disease-related mitochondrial dysfunction, including bioenergetic defects, mitochondrial DNA alterations, altered mitochondrial dynamics, activation of mitochondrial-dependent programmed cell death, and perturbations in mitochondrial tethering to the endoplasmic reticulum. Whether a primary or secondary event, mitochondrial dysfunction holds promise as a potential therapeutic target to halt the progression of neurodegeneration in Parkinson's disease.
帕金森病是一种常见的成人发病的神经退行性疾病,其发病机制仍在深入研究中。大量来自尸检人脑组织、基因和毒素诱导的动物和细胞模型的证据表明,线粒体功能障碍在疾病的病理生理学中起着核心作用。这篇综述讨论了我们目前对帕金森病相关线粒体功能障碍的理解,包括生物能量缺陷、线粒体 DNA 改变、线粒体动力学改变、线粒体依赖性程序性细胞死亡的激活以及线粒体与内质网的连接受到干扰。无论线粒体功能障碍是原发性还是继发性事件,作为一种潜在的治疗靶点,它都有望阻止帕金森病神经退行性变的进展。