Multiple pathways coordinate assembly of human mitochondrial complex IV and stabilization of respiratory supercomplexes.

ABSTRACT: Mitochondrial respiratory chain complexes I, III, and IV can associate into larger structures termed supercomplexes or respirasomes, thereby generating structural interdependences among the individual complexes yet to be understood. In patients, nonsense mutations in complex IV subunit genes cause severe encephalomyopathies randomly associated with pleiotropic complex I defects. Using complexome profiling and biochemical analyses, we have explored the structural rearrangements of the respiratory chain in human cell lines depleted of the catalytic complex IV subunit COX1 or COX2. In the absence of a functional complex IV holoenzyme, several supercomplex I+III₂ species coexist, which differ in their content of COX subunits and COX7A2L/HIGD2A assembly factors. The incorporation of

SUBMITTER: Lobo-Jarne T 

PROVIDER: S-EPMC7360963 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature