Norat Pedro, Soldozy Sauson, Sokolowski Jennifer D, Gorick Catherine M, Kumar Jeyan S, Chae Youngrok, Yağmurlu Kaan, Prada Francesco, Walker Melanie, Levitt Michael R, Price Richard J, Tvrdik Petr, Kalani M Yashar S
Department of Neurological Surgery, University of Virginia Health System, Charlottesville, VA, USA.
Department of Biomedical Engineering, University of Virginia School of Medicine, Charlottesville, VA, USA.
NPJ Regen Med. 2020 Nov 23;5(1):22. doi: 10.1038/s41536-020-00107-x.
Mitochondria are fundamental for metabolic homeostasis in all multicellular eukaryotes. In the nervous system, mitochondria-generated adenosine triphosphate (ATP) is required to establish appropriate electrochemical gradients and reliable synaptic transmission. Notably, several mitochondrial defects have been identified in central nervous system disorders. Membrane leakage and electrolyte imbalances, pro-apoptotic pathway activation, and mitophagy are among the mechanisms implicated in the pathogenesis of neurodegenerative diseases, such as Alzheimer's, Parkinson's, and Huntington's disease, as well as ischemic stroke. In this review, we summarize mitochondrial pathways that contribute to disease progression. Further, we discuss pathological states that damaged mitochondria impose on normal nervous system processes and explore new therapeutic approaches to mitochondrial diseases.
线粒体对于所有多细胞真核生物的代谢稳态至关重要。在神经系统中，线粒体产生的三磷酸腺苷（ATP）是建立适当的电化学梯度和可靠的突触传递所必需的。值得注意的是，在中枢神经系统疾病中已发现多种线粒体缺陷。膜泄漏和电解质失衡、促凋亡途径激活以及线粒体自噬是与神经退行性疾病（如阿尔茨海默病、帕金森病和亨廷顿病）以及缺血性中风发病机制相关的机制。在本综述中，我们总结了促成疾病进展的线粒体途径。此外，我们讨论了受损线粒体对正常神经系统过程造成的病理状态，并探索线粒体疾病的新治疗方法。
