Ontology highlight
ABSTRACT:
SUBMITTER: Koopman WJ
PROVIDER: S-EPMC3545297 | biostudies-literature | 2013 Jan
REPOSITORIES: biostudies-literature
Koopman Werner J H WJ Distelmaier Felix F Smeitink Jan A M JA Willems Peter H G M PH
The EMBO journal 20121113 1
Mitochondrial oxidative phosphorylation (OXPHOS) sustains organelle function and plays a central role in cellular energy metabolism. The OXPHOS system consists of 5 multisubunit complexes (CI-CV) that are built up of 92 different structural proteins encoded by the nuclear (nDNA) and mitochondrial DNA (mtDNA). Biogenesis of a functional OXPHOS system further requires the assistance of nDNA-encoded OXPHOS assembly factors, of which 35 are currently identified. In humans, mutations in both structur ...[more]