Human mitochondrial cytochrome c oxidase assembly factor COX18 acts transiently as a membrane insertase within the subunit 2 maturation module

M Bourens, A Barrientos - Journal of Biological Chemistry, 2017 - ASBMB
Defects in mitochondrial cytochrome c oxidase or respiratory chain complex IV (CIV)
assembly are a frequent cause of human mitochondrial disorders. Specifically, mutations in
four conserved assembly factors impinging the biogenesis of the mitochondrion-encoded
catalytic core subunit 2 (COX2) result in myopathies. These factors afford stability of newly
synthesized COX2 (the dystonia-ataxia syndrome protein COX20), a protein with two
transmembrane domains, and maturation of its copper center, Cu A (cardiomyopathy …
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