Li Ming-Xi, Mu De-Zhi
Department of Pediatrics, West China Second Hospital, Sichuan University/Key Laboratory of Birth Defects and Related Diseases of Women and Children, Ministry of Education/Key Laboratory of Development and Related Diseases of Women and Children, Chengdu 610041, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2017 Jun;19(6):724-729. doi: 10.7499/j.issn.1008-8830.2017.06.021.
Mitophagy is a process during which the cell selectively removes the mitochondria via the mechanism of autophagy. It is crucial to the functional completeness of the whole mitochondrial network and determines cell survival and death. On the one hand, the damaged mitochondria releases pro-apoptotic factors which induce cell apoptosis; on the other hand, the damaged mitochondria eliminates itself via autophagy, which helps to maintain cell viability. Mitophagy is of vital importance for the development and function of the nervous system. Neural cells rely on autophagy to control protein quality and eliminate the damaged mitochondria, and under normal circumstances, mitophagy can protect the neural cells. Mutations in genes related to mitophagy may cause the development and progression of neurodegenerative diseases. An understanding of the role of mitophagy in nervous system diseases may provide new theoretical bases for clinical treatment. This article reviews the research advances in the relationship between mitophagy and different types of nervous system diseases.
线粒体自噬是细胞通过自噬机制选择性清除线粒体的过程。它对于整个线粒体网络的功能完整性至关重要，并决定细胞的存活与死亡。一方面，受损的线粒体释放促凋亡因子，诱导细胞凋亡；另一方面，受损的线粒体通过自噬自我清除，这有助于维持细胞活力。线粒体自噬对神经系统的发育和功能至关重要。神经细胞依靠自噬来控制蛋白质质量并清除受损的线粒体，在正常情况下，线粒体自噬可以保护神经细胞。与线粒体自噬相关的基因突变可能导致神经退行性疾病的发生和发展。了解线粒体自噬在神经系统疾病中的作用可能为临床治疗提供新的理论依据。本文综述了线粒体自噬与不同类型神经系统疾病之间关系的研究进展。
