Assembly of the membrane domain of ATP synthase in human mitochondria.

ABSTRACT: The ATP synthase in human mitochondria is a membrane-bound assembly of 29 proteins of 18 kinds. All but two membrane components are encoded in nuclear genes, synthesized on cytoplasmic ribosomes, and imported into the matrix of the organelle, where they are assembled into the complex with ATP6 and ATP8, the products of overlapping genes in mitochondrial DNA. Disruption of individual human genes for the nuclear-encoded subunits in the membrane portion of the enzyme leads to the formation of intermediate vestigial ATPase complexes that provide a description of the pathway of assembly of the membrane domain. The key intermediate complex consists of the F₁-c₈ complex inhibited by the ATPase inhibitor protein IF₁ and attached to the peripheral stalk, with subuni

SUBMITTER: He J 

PROVIDER: S-EPMC5866602 | biostudies-literature | 2018 Mar

REPOSITORIES: biostudies-literature