Free for academic non-profit institutions. Other users need a Commercial license

Aliases for MT-CO2 Gene

Aliases for MT-CO2 Gene

  • Mitochondrially Encoded Cytochrome C Oxidase II 2 5
  • MTCO2 3 4
  • COII 3 4
  • Cytochrome C Oxidase Polypeptide II 4
  • Cytochrome C Oxidase Subunit II 3
  • Cytochrome C Oxidase II 2
  • EC 56
  • COXII 4
  • COX2 3

External Ids for MT-CO2 Gene

Previous HGNC Symbols for MT-CO2 Gene

  • MTCO2

Summaries for MT-CO2 Gene

GeneCards Summary for MT-CO2 Gene

MT-CO2 (Mitochondrially Encoded Cytochrome C Oxidase II) is a Protein Coding gene. Diseases associated with MT-CO2 include Mitochondrial Complex Iv Deficiency and Familial Colorectal Cancer. Among its related pathways are Development EGFR signaling via small GTPases and Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.. Gene Ontology (GO) annotations related to this gene include oxidoreductase activity and cytochrome-c oxidase activity.

UniProtKB/Swiss-Prot for MT-CO2 Gene

  • Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. Subunit 2 transfers the electrons from cytochrome c via its binuclear copper A center to the bimetallic center of the catalytic subunit 1.

Gene Wiki entry for MT-CO2 Gene

Additional gene information for MT-CO2 Gene

No data available for Entrez Gene Summary , CIViC summary , Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for MT-CO2 Gene

Genomics for MT-CO2 Gene

Genomic Locations for MT-CO2 Gene

Genomic Locations for MT-CO2 Gene
684 bases
Plus strand

Genomic View for MT-CO2 Gene

Genes around MT-CO2 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
GeneLoc Logo Gene Density

RefSeq DNA sequence for MT-CO2 Gene

No data available for GeneHancer (GH) Regulatory Elements for MT-CO2 Gene

Proteins for MT-CO2 Gene

  • Protein details for MT-CO2 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Cytochrome c oxidase subunit 2
    Protein Accession:
    Secondary Accessions:
    • Q37526

    Protein attributes for MT-CO2 Gene

    227 amino acids
    Molecular mass:
    25565 Da
    Name=Cu cation; Xref=ChEBI:CHEBI:23378;
    Quaternary structure:
    No Data Available

    Three dimensional structures from OCA and Proteopedia for MT-CO2 Gene

neXtProt entry for MT-CO2 Gene

Selected DME Specific Peptides for MT-CO2 Gene


Post-translational modifications for MT-CO2 Gene

  • Ubiquitination at Lys171
  • Modification sites at PhosphoSitePlus

Other Protein References for MT-CO2 Gene

ENSEMBL proteins:

Domains & Families for MT-CO2 Gene

Gene Families for MT-CO2 Gene

Human Protein Atlas (HPA):
  • Disease related genes
  • Potential drug targets
  • Predicted membrane proteins
  • Transporters

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the cytochrome c oxidase subunit 2 family.
  • Belongs to the cytochrome c oxidase subunit 2 family.
genes like me logo Genes that share domains with MT-CO2: view

Function for MT-CO2 Gene

Molecular function for MT-CO2 Gene

UniProtKB/Swiss-Prot Function:
Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. Subunit 2 transfers the electrons from cytochrome c via its binuclear copper A center to the bimetallic center of the catalytic subunit 1.

Enzyme Numbers (IUBMB) for MT-CO2 Gene

Gene Ontology (GO) - Molecular Function for MT-CO2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004129 cytochrome-c oxidase activity NAS 2550900
GO:0005507 copper ion binding IEA --
GO:0005515 protein binding IPI 23125284
GO:0016491 oxidoreductase activity IEA --
GO:0046872 metal ion binding IEA --
genes like me logo Genes that share ontologies with MT-CO2: view

Phenotypes for MT-CO2 Gene

GenomeRNAi human phenotypes for MT-CO2:
genes like me logo Genes that share phenotypes with MT-CO2: view

Human Phenotype Ontology for MT-CO2 Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

Animal Model Products

Inhibitory RNA Products

Clone Products

No data available for Phenotypes From GWAS Catalog , Animal Models , Transcription Factor Targets and HOMER Transcription for MT-CO2 Gene

Localization for MT-CO2 Gene

Subcellular locations from UniProtKB/Swiss-Prot for MT-CO2 Gene

Mitochondrion inner membrane; Multi-pass membrane protein.

Subcellular locations from

Extracellular space Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi Apparatus Nucleus Mitochondrion 0 1 2 3 4 5 Confidence
COMPARTMENTS Subcellular localization image for MT-CO2 gene
Compartment Confidence
extracellular 5
mitochondrion 5
plasma membrane 2
nucleus 2

Gene Ontology (GO) - Cellular Components for MT-CO2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IDA 16826196
GO:0005743 mitochondrial inner membrane TAS,IDA --
GO:0005751 mitochondrial respiratory chain complex IV IBA --
GO:0016020 membrane HDA,IDA 19946888
GO:0016021 integral component of membrane IEA --
genes like me logo Genes that share ontologies with MT-CO2: view

No data available for Subcellular locations from the Human Protein Atlas (HPA) for MT-CO2 Gene

Pathways & Interactions for MT-CO2 Gene

genes like me logo Genes that share pathways with MT-CO2: view

Gene Ontology (GO) - Biological Process for MT-CO2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006123 mitochondrial electron transport, cytochrome c to oxygen NAS,TAS --
GO:0007595 lactation IEA --
GO:0009409 response to cold IEA --
GO:0022900 electron transport chain IEA --
GO:0042773 ATP synthesis coupled electron transport IBA --
genes like me logo Genes that share ontologies with MT-CO2: view

No data available for SIGNOR curated interactions for MT-CO2 Gene

Drugs & Compounds for MT-CO2 Gene

(12) Drugs for MT-CO2 Gene - From: DrugBank, HMDB, and Novoseek

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
cholic acid Approved Pharma Full agonist, Agonist, Target 0
Oxygen Approved, Vet_approved Pharma 0
Copper Approved, Investigational Pharma 202
Water Approved Pharma 0
N-Formylmethionine Experimental Pharma Target 0

(5) Additional Compounds for MT-CO2 Gene - From: Novoseek

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
genes like me logo Genes that share compounds with MT-CO2: view

Transcripts for MT-CO2 Gene

mRNA/cDNA for MT-CO2 Gene

(1) Ensembl transcripts including schematic representations, and UCSC links where relevant :

Inhibitory RNA Products

Clone Products

Alternative Splicing Database (ASD) splice patterns (SP) for MT-CO2 Gene

No ASD Table

Relevant External Links for MT-CO2 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for MT-CO2 Gene

mRNA expression in normal human tissues from GTEx, Illumina, BioGPS, and CGAP SAGE for MT-CO2 Gene

Protein differential expression in normal tissues from HIPED for MT-CO2 Gene

This gene is overexpressed in Heart (9.1) and Nasal epithelium (7.8).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, MaxQB, and MOPED for MT-CO2 Gene

Protein tissue co-expression partners for MT-CO2 Gene

- Elite partner

Evidence on tissue expression from TISSUES for MT-CO2 Gene

  • Liver(4.8)
  • Muscle(4.3)
  • Heart(4.1)
  • Kidney(3.9)
  • Nervous system(3.8)
  • Intestine(3.7)
  • Adrenal gland(3.6)
  • Skin(3.6)
  • Lung(3.5)
  • Gall bladder(3.4)
  • Pancreas(3.4)
  • Stomach(3.4)
  • Bone marrow(3.3)
  • Lymph node(3.3)
  • Spleen(3.2)
  • Thyroid gland(3.2)
  • Blood(2)
genes like me logo Genes that share expression patterns with MT-CO2: view

No data available for mRNA expression in embryonic tissues and stem cells from LifeMap Discovery , mRNA differential expression in normal tissues , mRNA Expression by UniProt/SwissProt and Phenotype-based relationships between genes and organs from Gene ORGANizer for MT-CO2 Gene

Orthologs for MT-CO2 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for MT-CO2 Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia MT-CO2 34
  • 98 (a)
COX2 33
  • 90.46 (n)
(Bos Taurus)
Mammalia MT-CO2 34
  • 73 (a)
COX2 33
  • 71.21 (n)
(Mus musculus)
Mammalia COX2 33
  • 72.58 (n)
mt-Co2 16 34
  • 71 (a)
(Rattus norvegicus)
Mammalia COX2 33
  • 72.27 (n)
(Canis familiaris)
Mammalia COX2 33 34
  • 71.52 (n)
(Ornithorhynchus anatinus)
Mammalia MT-CO2 34
  • 70 (a)
(Monodelphis domestica)
Mammalia COII 34
  • 69 (a)
(Gallus gallus)
Aves COX2 33
  • 69.41 (n)
gga-mir-3527 34
  • 66 (a)
(Anolis carolinensis)
Reptilia COX2 34
  • 68 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia COX2 33
  • 68.94 (n)
Str.8896 33
(Danio rerio)
Actinopterygii COX2 33
  • 68.48 (n)
mt-co2 34
  • 65 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta COX2 33
  • 59.67 (n)
fruit fly
(Drosophila melanogaster)
Insecta COX2 33
  • 57.69 (n)
mt:CoII 34
  • 56 (a)
(Caenorhabditis elegans)
Secernentea COX2 33
  • 51.22 (n)
MTCE.31 34
  • 39 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes COX2 33
  • 51.5 (n)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AMI001W 33
  • 50.55 (n)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes COX2 33 34 36
  • 48.5 (n)
thale cress
(Arabidopsis thaliana)
eudicotyledons cox2 33
  • 55.82 (n)
(Oryza sativa)
Liliopsida cox2 33
  • 54.21 (n)
Os.23374 33
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes cox2 33
  • 50.39 (n)
sea squirt
(Ciona savignyi)
Ascidiacea -- 34
  • 40 (a)
Species where no ortholog for MT-CO2 was found in the sources mined by GeneCards:
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for MT-CO2 Gene

Gene Tree for MT-CO2 (if available)
Gene Tree for MT-CO2 (if available)

Paralogs for MT-CO2 Gene

(2) SIMAP similar genes for MT-CO2 Gene using alignment to 1 proteins:

genes like me logo Genes that share paralogs with MT-CO2: view

No data available for Paralogs for MT-CO2 Gene

Variants for MT-CO2 Gene

Sequence variations from dbSNP and Humsavar for MT-CO2 Gene

SNP ID Clin Chr MT pos Variation AA Info Type
rs1057516060 uncertain-significance, Optic atrophy 7,373(+) A/G
rs1057516061 uncertain-significance, Myopathy 7,496(+) T/C
rs1057516062 likely-pathogenic, Optic neuropathy 8,418(+) T/C
rs1057518824 uncertain-significance, Focal white matter lesions, Ischemic stroke 8,084(+) A/G/T coding_sequence_variant, missense_variant
rs111033319 uncertain-significance, pathogenic, not specified, Mitochondrial cytochrome c oxidase deficiency, Deafness, sensorineural, with neurologic features, Deafness, nonsyndromic sensorineural, mitochondrial 7,466(+) CCCCCC/CCCCC/CCCCCCC

Additional Variant Information for MT-CO2 Gene

Human Gene Mutation Database (HGMD)
SNPedia medical, phenotypic, and genealogical associations of SNPs for

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot , Structural Variations from Database of Genomic Variants (DGV) and Variation tolerance for MT-CO2 Gene

Disorders for MT-CO2 Gene

MalaCards: The human disease database

(15) MalaCards diseases for MT-CO2 Gene - From: HGMD, ClinVar, Orphanet, Swiss-Prot, DISEASES, Novoseek, and GeneCards

Disorder Aliases PubMed IDs
mitochondrial complex iv deficiency
  • cytochrome c oxidase deficiency
familial colorectal cancer
  • colorectal cancer, familial
mitochondrial metabolism disease
  • mitochondrial diseases
mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes
  • melas
mitochondrial disorders
  • mitochondrial encephalomyopathies
- elite association - COSMIC cancer census association via MalaCards


  • Mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]: A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, exercise intolerance, developmental delay, delayed motor development and mental retardation. Some affected individuals manifest a fatal hypertrophic cardiomyopathy resulting in neonatal death. A subset of patients manifest Leigh syndrome. {ECO:0000269 PubMed:10486321}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Additional Disease Information for MT-CO2

Genetic Association Database
genes like me logo Genes that share disorders with MT-CO2: view

No data available for Genatlas for MT-CO2 Gene

Publications for MT-CO2 Gene

  1. Investigation on mitochondrial tRNA(Leu/Lys), NDI and ATPase 6/8 in Iranian multiple sclerosis patients. (PMID: 17619138) Ahari SE … Bahar MA (Cellular and molecular neurobiology 2007) 3 22 44 58
  2. Differential modulation of mitochondrial OXPHOS system during HIV-1 induced T-cell apoptosis: up regulation of Complex-IV subunit COX-II and its possible implications. (PMID: 19771519) Tripathy MK … Mitra D (Apoptosis : an international journal on programmed cell death 2010) 3 22 58
  3. Altered expression of 12S/MT-RNR1, MT-CO2/COX2, and MT-ATP6 mitochondrial genes in prostate cancer. (PMID: 18409190) Abril J … Nunes V (The Prostate 2008) 3 22 58
  4. Arachidonate lipoxygenase (ALOX) and cyclooxygenase (COX) polymorphisms and colon cancer risk. (PMID: 15308583) Goodman JE … Harris CC (Carcinogenesis 2004) 3 44 58
  5. Functional effects and gender association of COX-2 gene polymorphism G-765C in bronchial asthma. (PMID: 15316498) Szczeklik W … Szczeklik A (The Journal of allergy and clinical immunology 2004) 3 44 58

Products for MT-CO2 Gene

Sources for MT-CO2 Gene

Loading form....