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Target intelligence / Profile preview
COX assembly mitochondrial protein 2 homolog (CMC2)
- Target
- CMC2
- Molecular classification
- Other (Mitochondrial assembly protein)
01
Overview
COX assembly mitochondrial protein 2 homolog (CMC2) is a mitochondrial protein that contains a conserved twin Cx9C structural motif, required for import into the mitochondrial intermembrane space via the Mia40 pathway[2][4]. It is essential for the assembly and function of cytochrome c oxidase (complex IV), helping regulate mitochondrial respiration[2][4]. CMC2 acts cooperatively with CMC1, showing physical and genetic interaction, but their functions are non-redundant: loss of CMC2 results in a complete absence of cytochrome c oxidase activity, which cannot be rescued by compensation from CMC1 or copper supplementation[2][4]. While strongly conserved and functionally essential in yeast, its direct biochemical role in humans—though presumed similar—has not been fully characterized experimentally. Known disease associations involve mitochondrial dysfunctions, including hypertrophic cardiomyopathy and cerebral angioma[5].
- Other names
- C-X9-C motif containing 2CMC2C16orf61DC132310061C15RikCOXM2COXM2_HUMAN
02
Biological functions
Cytochrome c oxidase biogenesisMitochondrial respirationPotential role in copper trafficking within mitochondria
03
Disease associations
Cardiomyopathy, familial hypertrophicCerebral angiomaOther (potential roles inferred from mitochondrial dysfunction)
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