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Cytochrome c oxidase assembly protein COX18, mitochondrial (COX18)
- Target
- COX18
- Molecular classification
- Enzyme assembly factor, Membrane insertase, Mitochondrial inner membrane protein, Oxa1/YidC/Alb3 family of membrane protein insertases
01
Overview
Cytochrome c oxidase assembly protein COX18, mitochondrial (COX18), is a transmembrane protein localized to the mitochondrial inner membrane and essential for the biogenesis and functional assembly of cytochrome c oxidase (complex IV) of the respiratory chain. COX18 acts as a membrane insertase, specifically enabling the translocation of the C-terminus of cytochrome c oxidase subunit II (COX2/MT-CO2) across the mitochondrial inner membrane and facilitating its maturation step after initial stabilization by COX20 and before metallochaperone action by SCO1/SCO2/COA6. Deficiency of COX18 disrupts complex IV assembly, resulting in isolated mitochondrial respiratory chain defects and associated myopathies or cardiomyopathies. COX18 is evolutionarily conserved as a member of the Oxa1/YidC/Alb3 family, participating in membrane protein insertion across species from yeast to humans. No drugs are currently known to directly target COX18, and it is not used as a biomarker for patient selection or treatment monitoring.
- Other names
- COX18Cytochrome c oxidase assembly factor COX18Cytochrome c oxidase assembly protein 18OXA1L2COX18HSFLJ38991
02
Biological functions
Assembly of mitochondrial respiratory chain complex IV (cytochrome c oxidase)Translocation of cytochrome c oxidase subunit II C-terminus across mitochondrial inner membraneStabilization and maturation of COX2Protein insertion into mitochondrial inner membrane
03
Disease associations
Mitochondrial myopathiesCardiomyopathyQ fever (gene-disease associations, not mechanistically established)Trench fever (gene-disease associations, not mechanistically established)
04
Safety considerations
Deficiency leads to isolated complex IV (cytochrome c oxidase) deficiency, causing mitochondrial dysfunctionCould contribute to mitochondrial disease syndromes such as myopathy or cardiomyopathy depending on mutation
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