A membrane arm of mitochondrial complex I sufficient to promote respirasome formation
Hezhi Fang, Xianglai Ye, Jie Xie, Yuanyuan Li, Haiyan Li, Xinzhu Bao, Yue Yang, Zifan Lin, Manli Jia, Qing Han, Jingjing Zhu, Xueyun Li, Qiongya Zhao, Yanling Yang, Jianxin Lyu
Abstract
-a, rather than other complex I modules, decreases the steady-state levels of complexes III and IV. Both HEK293T cells lacking TIMMDC1 and patient-derived cells with disease-causing mutations in TIMMDC1 showed accumulation of this respirasome subcomplex. This suggests that TIMMDC1, previously known as a complex-I assembly factor, may function as a respirasome assembly factor. Collectively, we provide a detailed, cooperative assembly model in which most complex-I subunits are added to the respirasome subcomplex in the lateral stages of respirasome assembly.
Topics & Concepts
HEK 293 cellsCell biologyMitochondrionInner mitochondrial membraneScaffold proteinBiologyChemistryBiophysicsBiochemistrySignal transductionGeneMitochondrial Function and PathologyATP Synthase and ATPases ResearchRNA modifications and cancer