Mitochondrial proteins: from biogenesis to functional networks
线粒体蛋白：从生物发生到功能网络
摘要：
Mitochondria are essential for the viability of eukaryotic cells as they perform crucial functions in bioenergetics, metabolism and signalling and have been associated with numerous diseases. Recent functional and proteomic studies have revealed the remarkable complexity of mitochondrial protein organization. Protein machineries with diverse functions such as protein translocation, respiration, metabolite transport, protein quality control and the control of membrane architecture interact with each other in dynamic networks. In this Review , we discuss the emerging role of the mitochondrial protein import machinery as a key organizer of these mitochondrial protein networks. The preprotein translocases that reside on the mitochondrial membranes not only function during organelle biogenesis to deliver newly synthesized proteins to their final mitochondrial destination but also cooperate with numerous other mitochondrial protein complexes that perform a wide range of functions. Moreover, these protein networks form membrane contact sites, for example, with the endoplasmic reticulum, that are key for integration of mitochondria with cellular function, and defects in protein import can lead to diseases.
线粒体对于真核细胞的生存是必不可少的，因为它们在生物能量学，代谢和信号传导中发挥关键作用并且与许多疾病相关。最近的功能和蛋白质组学研究揭示了线粒体蛋白质组织的显着复杂性。具有多种功能的蛋白质机器如蛋白质易位，呼吸，代谢物转运，蛋白质质量控制和膜结构控制在动态网络中相互作用。在本综述中，我们讨论了线粒体蛋白质进口机制作为这些线粒体蛋白质网络的关键组织者的新兴作用。位于线粒体膜上的前蛋白转位不仅在细胞器生物发生过程中起作用，以将新合成的蛋白质递送至其最终的线粒体目的地，而且还与许多其他具有广泛功能的线粒体蛋白质复合物配合。此外，这些蛋白质网络形成膜接触位点，例如，与内质网，其是线粒体与细胞功能整合的关键，并且蛋白质输入的缺陷可导致疾病。