Enables cytochrome-c oxidase activity. Predicted to be involved in aerobic respiration; respiratory electron transport chain; and response to oxidative stress. Part of respiratory chain complex IV. Is active in mitochondrial inner membrane. Is expressed in several structures, including brain; brown fat; liver; musculature; and stomach. Orthologous to human MT-CO1 (mitochondrially encoded cytochrome c oxidase I).
MGI Description
PHENOTYPE: Transmitochondrial mice carrying homoplasmic mtDNAs with a missense mutation in this gene show growth retardation, reduced COX activity in cardiac cells, increased blood lactate levels, mitochondrial myopathy, heart inflammation and edema, abnormal bloodvessel morphology, and severe cardiomyopathy. [provided by MGI curators]