线粒体
呼吸系统
生理学
呼吸生理学
生物
呼吸链
线粒体呼吸链
细胞生物学
医学
解剖
作者
Dusanka Milenkovic,Jelena Misic,Johannes F. Hevler,Thibaut Molinié,Injae Chung,Ilian Atanassov,Xinping Li,Roberta Filograna,Adam Antebi,Arnaud Mourier,Albert J. R. Heck,Judy Hirst,Nils‐Göran Larsson
出处
期刊:Cell Metabolism
[Elsevier]
日期:2023-10-01
卷期号:35 (10): 1799-1813.e7
被引量:6
标识
DOI:10.1016/j.cmet.2023.07.015
摘要
The mammalian respiratory chain complexes I, III2, and IV (CI, CIII2, and CIV) are critical for cellular bioenergetics and form a stable assembly, the respirasome (CI-CIII2-CIV), that is biochemically and structurally well documented. The role of the respirasome in bioenergetics and the regulation of metabolism is subject to intense debate and is difficult to study because the individual respiratory chain complexes coexist together with high levels of respirasomes. To critically investigate the in vivo role of the respirasome, we generated homozygous knockin mice that have normal levels of respiratory chain complexes but profoundly decreased levels of respirasomes. Surprisingly, the mutant mice are healthy, with preserved respiratory chain capacity and normal exercise performance. Our findings show that high levels of respirasomes are dispensable for maintaining bioenergetics and physiology in mice but raise questions about their alternate functions, such as those relating to the regulation of protein stability and prevention of age-associated protein aggregation.
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