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25 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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COX2
mitochondrially encoded cytochrome c oxidase II
Chromosome MT
NCBI Gene: 4513Ensembl: ENSG00000198712.1HGNC: HGNC:7421UniProt: P00403
179PubMed Papers
21Diseases
0Drugs
10Pathogenic Variants
FUNCTIONAL ROLE
Transporter
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Swiss-Prot Reviewed
Isolated cytochrome C oxidase deficiencymitochondrial diseaseMELAS syndromeleigh syndrome due to mitochondrial complex iv deficiency
✦AI Summary

COX2 (mitochondrially encoded cytochrome c oxidase II) is a critical component of mitochondrial complex IV that catalyzes the final step of the electron transport chain, reducing oxygen to water during oxidative phosphorylation. As part of cytochrome c oxidase, COX2 contains the dinuclear copper A center that transfers electrons from cytochrome c to the active site for oxygen reduction [UniProt]. Beyond its essential respiratory function, COX2 plays significant roles in cellular senescence and disease pathogenesis. COX2 methylation and downregulation serve as biomarkers of aging in heart mesenchymal stem cells, where increased methylation correlates with cellular senescence 1. In cancer contexts, COX2 upregulation mediates immune evasion and therapy resistance, particularly in KRAS-mutant lung cancer where it promotes immunotherapy resistance through prostaglandin E2 signaling 2. COX2 also facilitates cancer cell invasion in ovarian cancer through EGF-induced pathways 3. In metabolic syndrome-associated osteoarthritis, senescent preosteoclasts activate COX2-PGE2 signaling, contributing to disease progression 4. Additionally, COX2 participates in reproductive processes, being essential for endometrial decidualization through the COX2/mPGES1/PGE2 pathway 5. Mutations in mitochondrial COX2 cause complex IV deficiency, highlighting its fundamental importance in cellular respiration.

Sources cited
1
COX2 methylation and downregulation are biomarkers of aging in heart mesenchymal stem cells
PMID: 33416107
2
COX2 upregulation by oncogenic KRAS promotes immune evasion and immunotherapy resistance in lung cancer
PMID: 38635884
3
COX2 and PGE2 mediate EGF-induced ovarian cancer cell invasion
PMID: 24969217
4
Senescent preosteoclasts activate COX2-PGE2 signaling in metabolic syndrome-associated osteoarthritis
PMID: 35881544
5
COX2/mPGES1/PGE2 pathway is essential for mouse and human endometrial decidualization
PMID: 39885565
Disease Associationsβ“˜21
Isolated cytochrome C oxidase deficiencyOpen Targets
0.71Strong
mitochondrial diseaseOpen Targets
0.71Strong
MELAS syndromeOpen Targets
0.57Moderate
leigh syndrome due to mitochondrial complex iv deficiencyOpen Targets
0.54Moderate
MERRFOpen Targets
0.52Moderate
MERRF syndromeOpen Targets
0.52Moderate
inborn mitochondrial myopathyOpen Targets
0.49Moderate
Mitochondrial myopathyOpen Targets
0.49Moderate
mitochondrial non-syndromic sensorineural hearing lossOpen Targets
0.49Moderate
Abnormal mitral valve physiologyOpen Targets
0.45Moderate
Kearns-Sayre syndromeOpen Targets
0.44Moderate
progressive external ophthalmoplegiaOpen Targets
0.44Moderate
mitochondrial encephalomyopathyOpen Targets
0.42Moderate
familial hypertrophic cardiomyopathyOpen Targets
0.42Moderate
Mitochondrial encephalopathyOpen Targets
0.42Moderate
Leigh syndromeOpen Targets
0.41Moderate
External ophthalmoplegiaOpen Targets
0.40Weak
strokeOpen Targets
0.39Weak
rhabdomyolysisOpen Targets
0.37Weak
mitochondrial complex IV deficiency, nuclear-typeOpen Targets
0.37Weak
Mitochondrial complex IV deficiencyUniProt
Pathogenic Variants10
NC_012920.1(MT-CO2):m.7587T>CLikely pathogenic
Mitochondrial complex IV deficiency, nuclear type 1|Mitochondrial disease
β˜…β˜…β˜…β˜†2024
NC_012920.1(MT-CO2):m.8088delLikely pathogenic
Mitochondrial complex IV deficiency, nuclear type 1|Mitochondrial disease
β˜…β˜…β˜…β˜†2024
NC_012920.1(MT-CO2):m.7896G>APathogenic
Mitochondrial complex IV deficiency, nuclear type 1|Mitochondrial disease
β˜…β˜…β˜…β˜†2024
NC_012920.1(MT-CO2):m.8156delLikely pathogenic
Mitochondrial myopathy with reversible cytochrome C oxidase deficiency
β˜…β˜†β˜†β˜†2021
NC_012920.1(MT-CO2):m.7639delPathogenic
Tetralogy of Fallot
β˜†β˜†β˜†β˜†2018
NC_012920.1(MT-CO2):m.7661_7662insCCALikely pathogenic
Abnormal aortic valve physiology
β˜†β˜†β˜†β˜†
NC_012920.1(MT-CO2):m.7669_7670insCACLikely pathogenic
Abnormal aortic valve physiology
β˜†β˜†β˜†β˜†
NC_012920.1(MT-CO2):m.7680_7681insGTCPathogenic
Abnormal mitral valve physiology
β˜†β˜†β˜†β˜†
NC_012920.1(MT-CO2):m.7790_7791insTCCPathogenic
Abnormal mitral valve physiology
β˜†β˜†β˜†β˜†
NC_012920.1(MT-CO2):m.7815_7817dupPathogenic
Abnormal mitral valve physiology
β˜†β˜†β˜†β˜†
View on ClinVar β†—
Related Genes
COX10Protein interaction100%COX7A1Protein interaction100%SCO1Protein interaction98%UQCRFS1Protein interaction98%SCO2Protein interaction98%CYC1Protein interaction96%
Tissue Expression6 tissues
Heart
100%
Liver
88%
Brain
53%
Lung
16%
Bone Marrow
13%
Ovary
10%
Gene Interaction Network
Click a node to explore
COX2COX10COX7A1SCO1UQCRFS1SCO2CYC1
PROTEIN STRUCTURE
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PDB3VRJ Β· 1.90 Γ… Β· X-ray
View on RCSB β†—
RankingsWhere COX2 stands among ~20K protein-coding genes
  • #2,428of 20,598
    Most Researched179 Β· top quartile
  • #2,834of 5,498
    Most Pathogenic Variants10
Genes detectedCOX2
Sources retrieved25 papers
Response timeβ€”
πŸ“„ Sources
25β–Ό
1
CRISPR-Cas9 Screening Identifies KRAS-Induced COX2 as a Driver of Immunotherapy Resistance in Lung Cancer.
PMID: 38635884
Cancer Res Β· 2024
1.00
2
Silencing the ACAT1 Gene in Human SH-SY5Y Neuroblastoma Cells Inhibits the Expression of Cyclo-Oxygenase 2 (COX2) and Reduces Ξ²-Amyloid-Induced Toxicity Due to Activation of Protein Kinase C (PKC) and ERK.
PMID: 30541014
Med Sci Monit Β· 2018
0.92
3
Participation of COX2/mPGES1/PGE2 in mouse and human endometrial stromal decidualization.
PMID: 39885565
BMC Vet Res Β· 2025
0.90
4
Senescent preosteoclast secretome promotes metabolic syndrome associated osteoarthritis through cyclooxygenase 2.
PMID: 35881544
Elife Β· 2022
0.80
5
HER2 and COX2 expression in human prostate cancer.
PMID: 14687789
Eur J Cancer Β· 2004
0.72