COX7A2L Is a Mitochondrial Complex III Binding Protein that Stabilizes the III2+IV Supercomplex without Affecting Respirasome Formation.

COX7A2L Is a Mitochondrial Complex III Binding Protein that Stabilizes the III2+IV Supercomplex without Affecting Respirasome Formation.
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DOI:
10.1016/j.celrep.2016.07.081
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发表时间:
2016-08-30
期刊:
影响因子:
8.8
通讯作者:
Ugalde C
Ugalde C
中科院分区:
生物学1区
文献类型:
--
作者:
Pérez-Pérez R;Lobo-Jarne T;Milenkovic D;Mourier A;Bratic A;García-Bartolomé A;Fernández-Vizarra E;Cadenas S;Delmiro A;García-Consuegra I;Arenas J;Martín MA;Larsson NG;Ugalde C

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线粒体呼吸链(MRC)复合物I、III和IV结合成各种超分子结构,称为超复合物和线粒体酶体。虽然COX7A2L最初被描述为负责复合物IV动态结合到这些结构中以使MRC功能适应代谢变化的超复合物特异性因子,但这种作用一直存在争议。在这里,我们进一步研究COX7A2L在哺乳动物呼吸链的结构组织中的功能意义。如在小鼠中一样,人COX7A2L主要结合游离线粒体复合物III,并在较小程度上结合复合物IV,以特异性地促进III2+IV超复合物的稳定而不影响线粒体酶体形成。此外,COX7A2L不影响单个OXPHOS复合物的生物发生、稳定性和功能。这些数据表明,独立的监管机制的生物起源和营业额的不同MRC超复杂结构共存。
Mitochondrial respiratory chain (MRC) complexes I, III and IV associate into a variety of supramolecular structures known as supercomplexes and respirasomes. While COX7A2L was originally described as a supercomplex-specific factor responsible for the dynamic association of complex IV into these structures to adapt MRC function to metabolic variations, this role has been disputed. Here we further examine the functional significance of COX7A2L in the structural organization of the mammalian respiratory chain. As in the mouse, human COX7A2L binds primarily to free mitochondrial complex III and to a minor extent to complex IV to specifically promote the stabilization of the III2+IV supercomplex without affecting respirasome formation. Furthermore, COX7A2L does not affect the biogenesis, stabilization and function of the individual OXPHOS complexes. These data show that independent regulatory mechanisms for the biogenesis and turnover of different MRC supercomplex structures co-exist.
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