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Target intelligence / Profile preview
Cytochrome c oxidase assembly factor 6 (COA6)
- Target
- COA6
- Molecular classification
- Mitochondrial respiratory chain complex assembly factor, Enzyme cofactor/chaperone
01
Overview
Cytochrome c oxidase assembly factor 6 is a mitochondrial protein encoded by the COA6 gene and is integral to the biogenesis of cytochrome c oxidase (Complex IV) in the electron transport chain. Located in the intermembrane space, COA6 facilitates copper delivery, stabilizes newly formed COX2, and supports mitochondrial oxidative phosphorylation. Mutations in COA6 lead to severe mitochondrial diseases, including fatal infantile cardioencephalomyopathy, and its upregulation is associated with poor prognosis in several cancers, indicating its biomarker and therapeutic target potential[1][2][3][4][5][6][7].
- Other names
- C1orf31CEMCOX4cytochrome c oxidase assembly factor 6 homologMC4DN13
02
Mechanism of action
Not fully established for drugs. COA6 functions by aiding copper transfer and stabilizing COX2 in Complex IV assembly; modulating its expression affects mitochondrial respiration and cell proliferation
03
Biological functions
Mitochondrial oxidative phosphorylationElectron transportCopper delivery to Complex IVComplex IV biogenesisCell proliferation
04
Disease associations
Mitochondrial complex IV deficiencyCardiomyopathy (infantile, fatal)Cancer (particularly breast cancer, lung adenocarcinoma)
05
Safety considerations
Potential for mitochondrial toxicity—inhibiting COA6 can cause ATP synthesis disorders and fatal neonatal cardiomyopathyOn-target effects in non-cancerous tissues due to widespread mitochondrial expression
06
Interacting drugs
None directly identified
07
Biomarkers
COA6 expressionCOX2 level
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