生物
电子传递复合体Ⅰ
低温电子显微
线粒体
背景(考古学)
结构生物学
细胞生物学
线粒体DNA
突变
呼吸链
遗传学
生物物理学
基因
古生物学
作者
Karol Fiedorczuk,L.A. Sazanov
标识
DOI:10.1016/j.tcb.2018.06.006
摘要
Complex I has an essential role in ATP production by coupling electron transfer from NADH to quinone with translocation of protons across the inner mitochondrial membrane. Isolated complex I deficiency is a frequent cause of mitochondrial inherited diseases. Complex I has also been implicated in cancer, ageing, and neurodegenerative conditions. Until recently, the understanding of complex I deficiency on the molecular level was limited due to the lack of high-resolution structures of the enzyme. However, due to developments in single particle cryo-electron microscopy (cryo-EM), recent studies have reported nearly atomic resolution maps and models of mitochondrial complex I. These structures significantly add to our understanding of complex I mechanism and assembly. The disease-causing mutations are discussed here in their structural context.
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