Fang 2021 Cell Rep
» [[Has info::PMID: 33852835 Open Access]]
Was written by::Fang H, Was written by::Ye X, Was written by::Xie J, Was written by::Li Y, Was written by::Li H, Was written by::Bao X, Was written by::Yang Y, Was written by::Lin Z, Was written by::Jia M, Was written by::Han Q, Was written by::Zhu J, Was written by::Li X, Was written by::Zhao Q, Was written by::Yang Y, Was written by::Lyu J (Was published in year::2021) Was published in journal::Cell Rep
Abstract: [[has abstract::The assembly pathways of mitochondrial respirasome (supercomplex I+III2+IV) are not fully understood. Here, we show that an early sub-complex I assembly, rather than holo-complex I, is sufficient to initiate mitochondrial respirasome assembly. We find that a distal part of the membrane arm of complex I (PD-a module) is a scaffold for the incorporation of complexes III and IV to form a respirasome subcomplex. Depletion of PD-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.]] • Keywords: has publicationkeywords::Leigh syndrome, has publicationkeywords::TIMMDC1, has publicationkeywords::Cooperative assembly, has publicationkeywords::Mitochondrial respirasome, has publicationkeywords::Oxidative phosphorylation • Bioblast editor: [[has editor::Plangger M]]
Labels: MiParea: MiP area::Respiration
