Mitochondrial quality control (MQC) mechanisms are required to maintain a functional proteome, which enables mitochondria to perform a myriad of important cellular functions from oxidative phosphorylation to numerous other metabolic pathways. Mitochondrial protein homeostasis begins with the import of over 1000 nuclear-encoded mitochondrial proteins and the synthesis of 13 mitochondrial DNA-encoded proteins. A network of chaperones and proteases helps to fold new proteins and degrade unnecessary, damaged, or misfolded proteins, whereas more extensive damage can be removed by mitochondrial-derived vesicles (MDVs) or mitochondrial autophagy (mitophagy). Here, focusing on mechanisms in mammalian cells, we review the importance of mitochondrial protein import as a sentinel of mitochondrial function that activates multiple MQC mechanisms when impaired.

中文翻译：

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线粒体质量控制途径感知线粒体蛋白输入

线粒体质量控制（MQC）机制是维持功能性蛋白质组所必需的，这使得线粒体能够执行从氧化磷酸化到许多其他代谢途径的无数重要细胞功能。线粒体蛋白质稳态始于 1000 多种核编码线粒体蛋白质的输入和 13 种线粒体 DNA 编码蛋白质的合成。分子伴侣和蛋白酶网络有助于折叠新蛋白质并降解不必要的、受损的或错误折叠的蛋白质，而更广泛的损伤可以通过线粒体衍生囊泡 (MDV) 或线粒体自噬 (mitophagy) 消除。在这里，我们重点关注哺乳动物细胞中的机制，回顾了线粒体蛋白输入作为线粒体功能哨兵的重要性，线粒体功能受损时会激活多种 MQC 机制。