disease:Defects in SCO2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC) [MIM:604377, 220110]. This disease is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle show reductions in cytochrome c oxidase (COX) activity, whereas liver and fibroblasts show mild COX deficiencies.,function:Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2).,similarity:Belongs to the SCO1/2 family.,similarity:Contains 1 thioredoxin domain.,tissue specificity:Ubiquitous.,
Mitochondrion inner membrane ; Single-pass membrane protein .
Ubiquitous.
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