Dual Role of Mitofilin in Mitochondrial Membrane Organization and Protein Biogenesis
Dual Role of Mitofilin in Mitochondrial Membrane Organization and Protein Biogenesis
复制标题
DOI：
10.1016/j.devcel.2011.08.026
复制
发表时间：
2011-10-18
影响因子：
11.8
通讯作者：
van der Laan, Martin
中科院分区：
文献类型：
--
作者：
von der Malsburg, Karina;Mueller, Judith M.;van der Laan, Martin
线粒体内膜由两个区域组成，即内边界膜和嵴膜，它们通过嵴连接处连接。据报道，Mitofilin/Fcj1 参与嵴连接的形成，然而，对其功能和可能的伙伴蛋白存在不同的看法。我们报告 Mitofilin 发挥双重作用。 Mitofilin 是大型内膜复合物的一部分，我们确定了五种伙伴蛋白作为线粒体内膜组织系统 (MINOS) 的组成部分，该系统是保持嵴膜与内边界膜连接所必需的。此外，mitofilin 与外膜偶联，并通过线粒体膜间空间组装途径促进蛋白质输入。我们的研究结果表明，mitofilin 是 MINOS 的核心成分，并作为线粒体结构和蛋白质生物发生的多功能调节剂。
The mitochondrial inner membrane consists of two domains, inner boundary membrane and cristae membrane that are connected by crista junctions. Mitofilin/Fcj1 was reported to be involved in formation of crista junctions, however, different views exist on its function and possible partner proteins. We report that mitofilin plays a dual role. Mitofilin is part of a large inner membrane complex, and we identify five partner proteins as constituents of the mitochondrial inner membrane organizing system (MINOS) that is required for keeping cristae membranes connected to the inner boundary membrane. Additionally, mitofilin is coupled to the outer membrane and promotes protein import via the mitochondrial intermembrane space assembly pathway. Our findings indicate that mitofilin is a central component of MINOS and functions as a multifunctional regulator of mitochondrial architecture and protein biogenesis.