Meng Hao, Yan Wan-Yu, Lei Yu-Hong, Wan Zheng, Hou Ye-Ye, Sun Lian-Kun, Zhou Jue-Pu
Department of Pathophysiology, College of Basic Medical Sciences, Jilin University, Changchun, China.
Department of Neurosurgery, The First Hospital of Jilin University, Changchun, China.
Front Aging Neurosci. 2019 Nov 12;11:313. doi: 10.3389/fnagi.2019.00313. eCollection 2019.
Neurodegenerative diseases are disorders that are characterized by a progressive decline of motor and/or cognitive functions caused by the selective degeneration and loss of neurons within the central nervous system. The most common neurodegenerative diseases are Alzheimer's disease (AD), Parkinson's disease (PD), and Huntington's disease (HD). Neurons have high energy demands, and dysregulation of mitochondrial quality and function is an important cause of neuronal degeneration. Mitochondrial quality control plays an important role in maintaining mitochondrial integrity and ensuring normal mitochondrial function; thus, defects in mitochondrial quality control are also significant causes of neurodegenerative diseases. The mitochondrial deacetylase SIRT3 has been found to have a large effect on mitochondrial function. Recent studies have also shown that SIRT3 has a role in mitochondrial quality control, including in the refolding or degradation of misfolded/unfolded proteins, mitochondrial dynamics, mitophagy, and mitochondrial biogenesis, all of which are affected in neurodegenerative diseases.
神经退行性疾病是一类以中枢神经系统内神经元选择性变性和丧失导致运动和/或认知功能进行性衰退为特征的疾病。最常见的神经退行性疾病是阿尔茨海默病（AD）、帕金森病（PD）和亨廷顿病（HD）。神经元对能量需求很高，线粒体质量和功能失调是神经元变性的重要原因。线粒体质量控制在维持线粒体完整性和确保线粒体正常功能方面发挥着重要作用；因此，线粒体质量控制缺陷也是神经退行性疾病的重要病因。线粒体去乙酰化酶SIRT3已被发现对线粒体功能有很大影响。最近的研究还表明，SIRT3在线粒体质量控制中发挥作用，包括在错误折叠/未折叠蛋白质的重折叠或降解、线粒体动力学、线粒体自噬和线粒体生物发生中，而这些在神经退行性疾病中都会受到影响。
