NDUFA4 Is a Subunit of Complex IV of the Mammalian Electron Transport Chain
NDUFA4 Is a Subunit of Complex IV of the Mammalian Electron Transport Chain
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DOI：
10.1016/j.cmet.2012.07.015
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发表时间：
2012-09-05
期刊：
影响因子：
29
通讯作者：
Antonio Enriquez, Jose
中科院分区：
文献类型：
--
作者：
Balsa, Eduardo;Marco, Ricardo;Antonio Enriquez, Jose
氧化磷酸化系统是最具特征的代谢途径之一。在哺乳动物中，除了复合物I之外，所有复合物的蛋白质组分和X射线结构都被定义。在这里，我们表明，NDUFA 4，以前被认为是一个组成部分的NADH脱氢酶（CI），而不是一个组成部分的细胞色素c氧化酶（CIV）。NDUFA4的缺失不干扰Cl。相反，蛋白质组学、遗传学、进化和生物化学分析揭示NDUFA4在CIV功能和生物发生中起作用。NDUFA4蛋白属性的改变需要重新命名该基因并重新考虑CIV的结构。此外，NDUFA4应被认为是CIV的候选基因，而不是在人类中的Cl缺陷。
The oxidative phosphorylation system is one of the best-characterized metabolic pathways. In mammals, the protein components and X-ray structures are defined for all complexes except complex I. Here, we show that NDUFA4, formerly considered a constituent of NADH Dehydrogenase (CI), is instead a component of the cytochrome c oxidase (CIV). Deletion of NDUFA4 does not perturb Cl. Rather, proteomic, genetic, evolutionary, and biochemical analyses reveal that NDUFA4 plays a role in CIV function and biogenesis. The change in the attribution of the NDUFA4 protein requires renaming of the gene and reconsideration of the structure of CIV. Furthermore, NDUFA4 should be considered a candidate gene for CIV rather than Cl deficiencies in humans.