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Aliases for MT-CO3 Gene

Aliases for MT-CO3 Gene

  • Mitochondrially Encoded Cytochrome C Oxidase III 2
  • Cytochrome C Oxidase III 2 3
  • MTCO3 3 4
  • COIII 3 4
  • Cytochrome C Oxidase Polypeptide III 4
  • Cytochrome C Oxidase Subunit III 3
  • EC 63
  • COXIII 4
  • COX3 3

External Ids for MT-CO3 Gene

Previous HGNC Symbols for MT-CO3 Gene

  • MTCO3

Previous GeneCards Identifiers for MT-CO3 Gene

  • GC00U921585

Summaries for MT-CO3 Gene

GeneCards Summary for MT-CO3 Gene

MT-CO3 (Mitochondrially Encoded Cytochrome C Oxidase III) is a Protein Coding gene. Diseases associated with MT-CO3 include myoglobinuria recurrent and leigh-like syndrome. Among its related pathways are Metabolism and Metabolism.

UniProtKB/Swiss-Prot for MT-CO3 Gene

  • Subunits I, II and III form the functional core of the enzyme complex

Gene Wiki entry for MT-CO3 Gene

No data available for Entrez Gene Summary , Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for MT-CO3 Gene

Genomics for MT-CO3 Gene

Genomic Location for MT-CO3 Gene

9,207 bp from pter
9,990 bp from pter
784 bases
Plus strand

Genomic View for MT-CO3 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:

RefSeq DNA sequence for MT-CO3 Gene

No data available for Regulatory Elements for MT-CO3 Gene

Proteins for MT-CO3 Gene

  • Protein details for MT-CO3 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Cytochrome c oxidase subunit 3
    Protein Accession:
    Secondary Accessions:
    • Q14Y83

    Protein attributes for MT-CO3 Gene

    261 amino acids
    Molecular mass:
    29951 Da
    Quaternary structure:
    No Data Available

neXtProt entry for MT-CO3 Gene

Proteomics data for MT-CO3 Gene at MOPED

Selected DME Specific Peptides for MT-CO3 Gene

Post-translational modifications for MT-CO3 Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Other Protein References for MT-CO3 Gene

ENSEMBL proteins:
Reactome Protein details:

Domains for MT-CO3 Gene

Suggested Antigen Peptide Sequences for MT-CO3 Gene

GenScript: Design optimal peptide antigens:

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the cytochrome c oxidase subunit 3 family.
  • Belongs to the cytochrome c oxidase subunit 3 family.
genes like me logo Genes that share domains with MT-CO3: view

Function for MT-CO3 Gene

Molecular function for MT-CO3 Gene

UniProtKB/Swiss-Prot Function:
Subunits I, II and III form the functional core of the enzyme complex

Enzyme Numbers (IUBMB) for MT-CO3 Gene

Gene Ontology (GO) - Molecular Function for MT-CO3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004129 cytochrome-c oxidase activity --
GO:0005515 protein binding --
GO:0015002 heme-copper terminal oxidase activity --
genes like me logo Genes that share ontologies with MT-CO3: view

No data available for Phenotypes , Animal Models , Transcription Factor Targets and HOMER Transcription for MT-CO3 Gene

Localization for MT-CO3 Gene

Subcellular locations from UniProtKB/Swiss-Prot for MT-CO3 Gene

Mitochondrion inner membrane; Multi-pass membrane protein.

Subcellular locations from

Jensen Localization Image for MT-CO3 Gene COMPARTMENTS Subcellular localization image for MT-CO3 gene
Compartment Confidence
mitochondrion 5
plasma membrane 3
lysosome 2
nucleus 2
extracellular 1

Gene Ontology (GO) - Cellular Components for MT-CO3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion --
GO:0005743 mitochondrial inner membrane --
GO:0016020 membrane --
GO:0016021 integral component of membrane --
GO:0045277 respiratory chain complex IV --
genes like me logo Genes that share ontologies with MT-CO3: view

Pathways for MT-CO3 Gene

genes like me logo Genes that share pathways with MT-CO3: view

Gene Ontology (GO) - Biological Process for MT-CO3 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0008535 respiratory chain complex IV assembly --
GO:0019646 aerobic electron transport chain --
GO:0022904 respiratory electron transport chain --
GO:0044237 cellular metabolic process --
GO:0044281 small molecule metabolic process --
genes like me logo Genes that share ontologies with MT-CO3: view

Drugs for MT-CO3 Gene

(2) Drugbank Compounds for MT-CO3 Gene

Compound Status Synonyms Cas Number PubChem ID Type Actions PubMed IDs
Cholic Acid
81-25-4 221493 Target approved
439750 Target experimental

(1) HMDB Compounds for MT-CO3 Gene

Compound Synonyms Cas Number PubMed IDs
Cholic acid
  • 17b-[1-Methyl-3-carboxypropyl]etiocholane-3a,7a,12a-triol

(4) Novoseek inferred chemical compound relationships for MT-CO3 Gene

Compound -log(P) Hits PubMed IDs
nadh 58.1 6
citrate 40.1 1
atp 35.1 5
glucose 0 1
genes like me logo Genes that share compounds with MT-CO3: view

Transcripts for MT-CO3 Gene

mRNA/cDNA for MT-CO3 Gene

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

Alternative Splicing Database (ASD) splice patterns (SP) for MT-CO3 Gene

No ASD Table

Relevant External Links for MT-CO3 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for MT-CO3 Gene

mRNA expression in normal human tissues for MT-CO3 Gene

mRNA expression in normal human tissues for MT-CO3 Gene

Protein differential expression in normal tissues for MT-CO3 Gene

This gene is overexpressed in Heart (14.7) and Nasal epithelium (8.2).

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, and MOPED for MT-CO3 Gene

genes like me logo Genes that share expressions with MT-CO3: view

Expression partners for MT-CO3 Gene

* - Elite partner

Primer Products

In Situ Assay Products

No data available for mRNA expression in embryonic tissues and stem cells from LifeMap Discovery , mRNA differential expression in normal tissues and mRNA Expression by UniProt/SwissProt for MT-CO3 Gene

Orthologs for MT-CO3 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for MT-CO3 Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia COX3 35
  • 79.23 (n)
  • 85.77 (a)
COX3 36
  • 88 (a)
(Canis familiaris)
Mammalia COX3 35
  • 74.71 (n)
  • 83.14 (a)
COX3 36
  • 85 (a)
(Mus musculus)
Mammalia COX3 35
  • 76.25 (n)
  • 85.82 (a)
mt-Co3 16
mt-Co3 36
  • 87 (a)
(Pan troglodytes)
Mammalia COX3 35
  • 90.42 (n)
  • 95.79 (a)
MT-CO3 36
  • 97 (a)
(Rattus norvegicus)
Mammalia COX3 35
  • 78.29 (n)
  • 86.21 (a)
(Monodelphis domestica)
Mammalia COIII 36
  • 85 (a)
(Ornithorhynchus anatinus)
Mammalia MT-CO3 36
  • 84 (a)
(Gallus gallus)
Aves COX3 35
  • 71.65 (n)
  • 73.18 (a)
MT-CO3 36
  • 75 (a)
(Anolis carolinensis)
Reptilia COX3 36
  • 78 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia COX3 35
  • 72.29 (n)
  • 78.93 (a)
Str.8155 35
(Danio rerio)
Actinopterygii COX3 35
  • 71.52 (n)
  • 78.16 (a)
Dr.24911 35
mt-co3 36
  • 81 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta COX3 35
  • 62.95 (n)
  • 65 (a)
fruit fly
(Drosophila melanogaster)
Insecta COX3 35
  • 64.61 (n)
  • 63.71 (a)
mt:CoIII 36
  • 65 (a)
(Caenorhabditis elegans)
Secernentea MTCE.23 36
  • 43 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AMI003W 35
  • 50.06 (n)
  • 46.69 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes COX3 35
  • 51.29 (n)
  • 44.19 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes COX3 35
  • 49.61 (n)
  • 44.19 (a)
COX3 36
  • 44 (a)
COX3 38
thale cress
(Arabidopsis thaliana)
eudicotyledons cox3 35
  • 59.04 (n)
  • 56.59 (a)
(Oryza sativa)
Liliopsida Os.24741 35
sea squirt
(Ciona savignyi)
Ascidiacea -- 36
  • 48 (a)
Species with no ortholog for MT-CO3:
  • 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)
  • fission yeast (Schizosaccharomyces pombe)
  • 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-CO3 Gene

Gene Tree for MT-CO3 (if available)
Gene Tree for MT-CO3 (if available)

Paralogs for MT-CO3 Gene

(4) SIMAP similar genes for MT-CO3 Gene using alignment to 1 proteins:

genes like me logo Genes that share paralogs with MT-CO3: view

No data available for Paralogs for MT-CO3 Gene

Variants for MT-CO3 Gene

Sequence variations from dbSNP and Humsavar for MT-CO3 Gene

SNP ID Clin Chr MT pos Sequence Context AA Info Type MAF
VAR_002167 Leber hereditary optic neuropathy (LHON)
VAR_002168 Leber hereditary optic neuropathy (LHON)
VAR_002169 -
VAR_008573 -
VAR_008574 -

Relevant External Links for MT-CO3 Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot and Structural Variations from Database of Genomic Variants (DGV) for MT-CO3 Gene

Disorders for MT-CO3 Gene


  • Leber hereditary optic neuropathy (LHON) [MIM:535000]: A maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. {ECO:0000269 PubMed:8240356}. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • 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:8630495}. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • Recurrent myoglobinuria mitochondrial (RM-MT) [MIM:550500]: Recurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine. Note=The gene represented in this entry may be involved in disease pathogenesis.
  • Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS) [MIM:540000]: Genetically heterogeneous disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness. {ECO:0000269 PubMed:18587274, ECO:0000269 PubMed:7496173}. Note=The disease may be caused by mutations affecting the gene represented in this entry.

(1) University of Copenhagen DISEASES for MT-CO3 Gene

(6) Novoseek inferred disease relationships for MT-CO3 Gene

Disease -log(P) Hits PubMed IDs
lebers hereditary optic neuropathy 58.5 1
mitochondrial myopathies 54.1 2
acidosis lactic 42.3 1
hepatitis b 5.23 4
tumors 0 1

Relevant External Links for MT-CO3

Genetic Association Database (GAD)
genes like me logo Genes that share disorders with MT-CO3: view

No data available for OMIM and Genatlas for MT-CO3 Gene

Publications for MT-CO3 Gene

  1. A MELAS syndrome family harboring two mutations in mitochondrial genome. (PMID: 18587274) Choi B.O. … Chung K.W. (Exp. Mol. Med. 2008) 3 4 23
  2. Sequence and organization of the human mitochondrial genome. (PMID: 7219534) Anderson S. … Young I.G. (Nature 1981) 3 4
  3. Mitochondrial DNA mutations with Leber's hereditary optic neuropathy in Japanese patients with open-angle glaucoma. (PMID: 16604388) Inagaki Y. … Fukuchi T. (Jpn. J. Ophthalmol. 2006) 3 48
  4. Cytochrome c oxidase mutations in Leber hereditary optic neuropathy. (PMID: 8240356) Johns D.R. … Neufeld M.J. (Biochem. Biophys. Res. Commun. 1993) 4 23
  5. Mutations in mtDNA-encoded cytochrome c oxidase subunit genes causing isolated myopathy or severe encephalomyopathy. (PMID: 16288875) HorvA!th R. … LochmA1ller H. (Neuromuscul. Disord. 2005) 3 23

Products for MT-CO3 Gene

Sources for MT-CO3 Gene

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