Assembly of mammalian oxidative phosphorylation complexes I-V and supercomplexes.
Assembly of mammalian oxidative phosphorylation complexes I-V and supercomplexes.
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DOI：
10.1042/ebc20170098
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发表时间：
2018-07-20
影响因子：
6.4
通讯作者：
Fernandez-Vizarra E
中科院分区：
文献类型：
--
作者：
Signes A;Fernandez-Vizarra E
线粒体内膜中五种氧化磷酸化系统（OXPHOS）复合物的组装是一个复杂的过程。人类酶包括执行催化活性的核心蛋白和大量在组装、调节和稳定中起重要作用的“额外”亚基。辅基的正确添加以及结构组分的陪伴和掺入需要大量的因子，其中许多因子已被发现在线粒体疾病的情况下发生突变。如今，每个单独的复合物的组装机制几乎完全理解，有关组装因素的知识不断增加。另一方面，现在已经确定复合物I、III和IV彼此相互作用，形成所谓的呼吸超复合物或“呼吸酶体”，尽管导致其形成的途径仍然不完全清楚。这篇综述是我们目前的知识的总结有关组装的配合物I-V和超配合物。
The assembly of the five oxidative phosphorylation system (OXPHOS) complexes in the inner mitochondrial membrane is an intricate process. The human enzymes comprise core proteins, performing the catalytic activities, and a large number of ‘supernumerary’ subunits that play essential roles in assembly, regulation and stability. The correct addition of prosthetic groups as well as chaperoning and incorporation of the structural components require a large number of factors, many of which have been found mutated in cases of mitochondrial disease. Nowadays, the mechanisms of assembly for each of the individual complexes are almost completely understood and the knowledge about the assembly factors involved is constantly increasing. On the other hand, it is now well established that complexes I, III and IV interact with each other, forming the so-called respiratory supercomplexes or ‘respirasomes’, although the pathways that lead to their formation are still not completely clear. This review is a summary of our current knowledge concerning the assembly of complexes I–V and of the supercomplexes.