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首页> 外文期刊>Cell metabolism >Mitochondrial complex I plays an essential role in human respirasome assembly
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Mitochondrial complex I plays an essential role in human respirasome assembly

机译：线粒体复合体I在人类呼吸道组装中起重要作用

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The biogenesis and function of the mitochondrial respiratory chain (RC) involve the organization of RC enzyme complexes in supercomplexes or respirasomes through an unknown biosynthetic process. This leads to structural interdependences between RC complexes, which are highly relevant from biological and biomedical perspectives, because RC defects often lead to severe neuromuscular disorders. We show that in human cells, respirasome biogenesis involves a complex I assembly intermediate acting as a scaffold for the combined incorporation of complexes III and IV subunits, rather than originating from the association of preassembled individual holoenzymes. The process ends with the incorporation of complex I NADH dehydrogenase catalytic module, which leads to the respirasome activation. While complexes III and IV assemble either as free holoenzymes or by incorporation of free subunits into supercomplexes, the respirasomes constitute the structural units where complex I is assembled and activated, thus explaining the significance of the respirasomes for RC function.
机译：线粒体呼吸链（RC）的生物发生和功能涉及通过未知的生物合成过程在超级复合体或呼吸小体中组织RC酶复合体。这导致RC复合体之间的结构相互依赖性，这从生物学和生物医学角度来看都是高度相关的，因为RC缺陷通常会导致严重的神经肌肉疾病。我们显示，在人类细胞中，呼吸系统生物发生涉及复杂的I装配中间体，充当复合物III和IV亚基的结合掺入的支架，而不是源于预先组装的单个全酶的结合。该过程以掺入复合物I NADH脱氢酶催化模块结束，这导致呼吸小体活化。复合物III和IV既可以作为游离全酶或通过将游离亚基掺入超复合物中而组装，而呼吸小体构成了复合物I被组装和激活的结构单元，从而解释了呼吸小体对于RC功能的重要性。

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