Tatsuta Takashi, Langer Thomas
Institute for Genetics and Centre for Molecular Medicine, University of Cologne, Cologne, Germany.
EMBO J. 2008 Jan 23;27(2):306-14. doi: 10.1038/sj.emboj.7601972.
Dysfunction of mitochondria has severe cellular consequences and is linked to ageing and neurodegeneration in human. Several surveillance strategies have evolved that limit mitochondrial damage and ensure cellular integrity. Intraorganellar proteases conduct protein quality control and exert regulatory functions, membrane fusion and fission allow mitochondrial content mixing within a cell, and the autophagic degradation of severely damaged mitochondria protects against apoptosis. Here, we will summarize the current knowledge on these surveillance strategies and their role in human disease.
线粒体功能障碍会产生严重的细胞后果，并与人类衰老和神经退行性变相关。已经进化出了几种监测策略来限制线粒体损伤并确保细胞完整性。线粒体内蛋白酶进行蛋白质质量控制并发挥调节功能，膜融合和裂变使线粒体内容物在细胞内混合，而对严重受损线粒体的自噬降解可防止细胞凋亡。在这里，我们将总结关于这些监测策略及其在人类疾病中的作用的当前知识。
