中文摘要：

线粒体自噬（mitophagy）是指细胞通过自噬的机制选择性地清除线粒体的过程，对于整个线粒体网络的功能完整性十分关键，决定细胞的生存和死亡。一方面损伤的线粒体释放促凋亡因子，诱导细胞进入凋亡；另一方面，损伤的线粒体通过自噬自我清除，维持细胞的存活。线粒体自噬对于神经系统的发育和功能都十分重要。神经细胞依赖自噬控制蛋白质量，移除损伤的线粒体，正常情况下线粒体自噬起到保护神经细胞的作用。线粒体自噬相关基因的突变可能导致神经退行性疾病以及一些小儿神经系统疾病的发生和发展。了解神经系统疾病中线粒体自噬的作用，可望为临床治疗提供新的理论依据。该文就近年来线粒体自噬与各类神经系统疾病的关系作一综述。

英文摘要：

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.