Assembly of mammalian oxidative phosphorylation complexes I-V and supercomplexes.

ABSTRACT: The assembly of the five oxidative phosphorylation system (OXPHOS) complexes in the inner mitochondrial membrane is an intricate process. The human enzymes comprise core proteins, performing the catalytic activities, and a large number of 'supernumerary' subunits that play essential roles in assembly, regulation and stability. The correct addition of prosthetic groups as well as chaperoning and incorporation of the structural components require a large number of factors, many of which have been found mutated in cases of mitochondrial disease. Nowadays, the mechanisms of assembly for each of the individual complexes are almost completely understood and the knowledge about the assembly factors involved is constantly increasing. On the other hand, it is now well established that complexes I,

SUBMITTER: Signes A 

PROVIDER: S-EPMC6056720 | biostudies-literature | 2018 Jul

REPOSITORIES: biostudies-literature