线粒体自噬与人类疾病

线粒体在生物体的新陈代谢中起着非常重要的作用，不仅为代谢活动提供能量，还可以产生具有信号传递和基因调节作用的活性氧．线粒体发生功能障碍或损坏都可能造成严重的后果，甚至导致细胞死亡．受损线粒体通常通过线粒体自噬降解，研究发现线粒体自噬紊乱与多种疾病发生有关．本文阐述了线粒体自噬的调节机制和介导途径，详细论述了近年来线粒体自噬在神经退行性疾病、心脏病及肿瘤中的作用，总结指出线粒体自噬的两面性，即一方面正常范围内的线粒体自噬可以维持人体细胞的正常生理机能，另一方面，线粒体自噬水平过高和过低都会引发疾病．

Mitophagy in Human Disease

Mitochondria plays an important role in metabolism of living organism, and it provides energy for metabolic activity and produces reactive oxygen species with signal transduction and gene regulation capacity. For this reason, dysfunction or damage of mitochondria could cause serious consequences，even lead to cell death. Damaged mitochondria can be degradated by mitophagy. Studies indicated that dysfunction of mitophagy is closely related to the pathogenesis of many diseases. This paper is to elaborate the regulation mechanism and mediated pathways of mitophagy, and dwell upon the research progresses of the role of mitophagy in neurodegenerative diseases, heart failure and tumor, and to summarize the two sides of mitophagy on one hand the mitophagy within the normal range can maintain the normal physiological function, on the other hand, too high or too low level of mitophagy will cause disease.