COX assembly mitochondrial protein 1 homolog (CMC1)

COX assembly mitochondrial protein 1 homolog (CMC1) is a membrane-bound protein found in the mitochondrial inner membrane, facing the intermembrane space. It contains two conserved CX9C motifs characteristic of proteins involved in cysteine redox reactions and copper binding. CMC1 is essential for full expression and proper assembly of cytochrome c oxidase (COX), a key enzyme in mitochondrial respiration. It participates in copper trafficking and distribution inside mitochondria, binding copper(I) ions and regulating copper delivery to both COX and the enzyme Cu-Zn superoxide dismutase (Sod1p). CMC1 interacts physically with other twin CX9C motif proteins (e.g., Cmc2), and stability of COX1 subunit protein relies on CMC1 during early mitochondrial assembly. COX activity defects arising from CMC1 deficiency can be partially rescued by exogenous copper supplementation. Although not a classical drug target, genomic mutations in the human CMC1 gene are candidates for underlying some mitochondrial disorders associated with COX deficiency.

Other names

C-X9-C motif containing 1C3orf68Cmc1pMGC61571COX assembly mitochondrial protein homologCX9C mitochondrial protein required for full expression of COX 1mitochondrial metallochaperone-like protein