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9 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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COA4
cytochrome c oxidase assembly factor 4 homolog
Chromosome 11 · 11q13.4
NCBI Gene: 51287Ensembl: ENSG00000181924.9HGNC: HGNC:24604UniProt: Q9NYJ1
15PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingmitochondrionmitochondrial intermembrane spacemitochondrial respiratory chain complex IV assemblyneurodegenerative diseaselung adenocarcinomaneoplasmurethral syndrome
✦AI Summary

COA4 (cytochrome c oxidase assembly factor 4 homolog) is a mitochondrial protein localized to the intermembrane space that functions as a critical assembly factor for cytochrome c oxidase (COX), a multimeric copper-containing enzyme essential for cellular respiration 1. COA4 contains a conserved twin CX9C motif and plays a key role in the mitochondrial copper delivery pathway, particularly for copper site biogenesis in the Cox1 subunit 12. Mechanistically, COA4 functions through dual pathways: mitochondrial COA4 augments COX activity to enhance oxidative phosphorylation, while cytosolic COA4 binds and activates CDC42 to regulate pseudopodia formation and cell migration 3. These functions are evolutionarily conserved, as the yeast ortholog Coa4 can be functionally replaced by human COA4 1. COA4 is highly overexpressed in KRAS mutant tumors, where it drives cancer metastasis through COX-dependent and CDC42-dependent mechanisms independent of tumor proliferation 3. Additionally, COA4 participates in ferroptosis regulation through the SIRT6/COA4/FTH1 pathway, protecting cells from oxidative stress-induced ferroptotic death 4. COA4 is identified as a cancer-promoting protein whose downregulation correlates with reduced metastatic potential 5, establishing it as a therapeutic target in KRAS-driven malignancies.

Sources cited
1
COA4 is a twin CX9C motif-containing protein localized to mitochondrial intermembrane space; placed in copper delivery pathway to Cox1 subunit; human COA4 functionally replaces yeast Coa4
PMID: 35666203
2
COA4 is a twin CX9C motif mitochondrial protein in intermembrane space associated with inner membrane; cells lacking COA4 show depressed COX activity
PMID: 20624914
3
COA4 is highly overexpressed in KRAS mutant tumors; mitochondrial COA4 augments COX activity for oxidative phosphorylation; cytosolic COA4 binds and activates CDC42; overexpression enhances metastasis; dual functions evolutionarily conserved
PMID: 40936169
4
COA4 participates in SIRT6/COA4/FTH1 pathway to inhibit ferroptosis and protect against ferroptotic stress
PMID: 36463827
5
COA4 is identified as a cancer-promoting protein that is downregulated by natural peptide treatment in cancer cells
PMID: 35818360
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.54Moderate
lung adenocarcinomaOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.07Suggestive
urethral syndromeOpen Targets
0.03Suggestive
osteosarcomaOpen Targets
0.02Suggestive
pyogenic granulomaOpen Targets
0.02Suggestive
infectionOpen Targets
0.02Suggestive
pancreatic adenocarcinomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
systemic juvenile idiopathic arthritisOpen Targets
0.01Suggestive
breast cancerOpen Targets
0.01Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.01Suggestive
hepatocellular carcinomaOpen Targets
0.01Suggestive
autoimmune myocarditisOpen Targets
0.01Suggestive
influenzaOpen Targets
0.01Suggestive
secondary progressive multiple sclerosisOpen Targets
0.00Suggestive
metastatic neoplasmOpen Targets
0.00Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.00Suggestive
Pelvic Inflammatory DiseaseOpen Targets
0.00Suggestive
polyarteritis nodosaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
COX16Shared pathway100%PET117Shared pathway100%PET100Shared pathway100%COA5Shared pathway100%SMIM20Shared pathway100%COX20Shared pathway100%
Tissue Expression6 tissues
Liver
100%
Heart
63%
Ovary
42%
Brain
40%
Lung
39%
Bone Marrow
24%
Gene Interaction Network
Click a node to explore
COA4COX16PET117PET100COA5SMIM20COX20
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q9NYJ1
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.74LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.10 [0.69–1.74]
RankingsWhere COA4 stands among ~20K protein-coding genes
  • #15,524of 20,598
    Most Researched15
  • #16,294of 17,882
    Most Constrained (LOEUF)1.74
Genes detectedCOA4
Sources retrieved9 papers
Response time—
📄 Sources
9▼
1
Melatonin inhibits ferroptosis and delays age-related cataract by regulating SIRT6/p-Nrf2/GPX4 and SIRT6/NCOA4/FTH1 pathways.
PMID: 36463827
Biomed Pharmacother · 2023
1.00
2
Novel Evolutionarily Conserved Oncogene COA4 is Driven by KRAS Mutant and Promotes Cancer Metastasis Through Dual Mitochondrial Metabolism-Dependent and -Independent Mechanisms.
PMID: 40936169
Adv Sci (Weinh) · 2025
0.89
3
Interference with endothelial cell function by JG-03-14, an agent that binds to the colchicine site on microtubules.
PMID: 19576183
Biochem Pharmacol · 2009
0.78
4
A yeast suppressor screen links Coa4 to the mitochondrial copper delivery pathway for cytochrome c oxidase.
PMID: 35666203
Genetics · 2022
0.67
5
Analysis of Leigh syndrome mutations in the yeast SURF1 homolog reveals a new member of the cytochrome oxidase assembly factor family.
PMID: 20624914
Mol Cell Biol · 2010
0.56