Title : Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies.

Pub. Date : 2015 Jun 2

PMID : 25959673






3 Functional Relationships(s)
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1 COX1 and COX2 contain heme and copper redox centers, which are integrated during assembly of the enzyme. Copper mitochondrially encoded cytochrome c oxidase II Homo sapiens
2 COA6 interacts transiently with the copper-containing catalytic domain of newly synthesized COX2. Copper mitochondrially encoded cytochrome c oxidase II Homo sapiens
3 Our analyses define COA6 as a constituent of the mitochondrial copper relay system, linking defects in COX2 metallation to cardiac cytochrome c oxidase deficiency. Copper mitochondrially encoded cytochrome c oxidase II Homo sapiens