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Aliases for MT-CO3 Gene

Aliases for MT-CO3 Gene

  • Mitochondrially Encoded Cytochrome C Oxidase III 2 5
  • 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 56
  • 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 Leber Hereditary Optic Neuropathy and Genetic Recurrent Myoglobinuria. Among its related pathways are Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. and Gene Expression. Gene Ontology (GO) annotations related to this gene include cytochrome-c oxidase activity and heme-copper terminal oxidase activity.

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

Additional gene information for MT-CO3 Gene

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

Genomics for MT-CO3 Gene

Genomic Locations for MT-CO3 Gene

Genomic Locations for MT-CO3 Gene
784 bases
Plus strand

Genomic View for MT-CO3 Gene

Genes around MT-CO3 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
GeneLoc Logo Gene Density

RefSeq DNA sequence for MT-CO3 Gene

No data available for GeneHancer (GH) 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

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:

Domains & Families for MT-CO3 Gene

Gene Families for MT-CO3 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 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 IEA --
GO:0005515 protein binding IPI 12059041
GO:0015002 heme-copper terminal oxidase activity IEA --
genes like me logo Genes that share ontologies with MT-CO3: view

Human Phenotype Ontology for MT-CO3 Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

Animal Model Products

Clone Products

No data available for Phenotypes From GWAS Catalog , 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

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-CO3 gene
Compartment Confidence
mitochondrion 5
plasma membrane 3
nucleus 3
extracellular 2
lysosome 2
cytosol 1

Subcellular locations from the

Human Protein Atlas (HPA)

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

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IEA --
GO:0005743 mitochondrial inner membrane TAS --
GO:0016020 membrane IEA --
GO:0016021 integral component of membrane IEA --
GO:0045277 respiratory chain complex IV IDA 1651240
genes like me logo Genes that share ontologies with MT-CO3: view

Pathways & Interactions 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:0006123 mitochondrial electron transport, cytochrome c to oxygen TAS --
GO:0008535 respiratory chain complex IV assembly IMP 8630495
GO:0019646 aerobic electron transport chain IEA --
GO:0022904 respiratory electron transport chain IEA --
GO:1902600 hydrogen ion transmembrane transport IEA --
genes like me logo Genes that share ontologies with MT-CO3: view

No data available for SIGNOR curated interactions for MT-CO3 Gene

Drugs & Compounds for MT-CO3 Gene

(6) Drugs for MT-CO3 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
N-Formylmethionine Experimental Pharma Target 0
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 :

Clone Products

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 from GTEx, Illumina, BioGPS, and CGAP SAGE for MT-CO3 Gene

mRNA expression in normal human tissues for MT-CO3 Gene

Protein differential expression in normal tissues from HIPED for MT-CO3 Gene

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

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, and MOPED for MT-CO3 Gene

Protein tissue co-expression partners for MT-CO3 Gene

Evidence on tissue expression from TISSUES for MT-CO3 Gene

  • Muscle(4.3)
  • Heart(4.2)
  • Kidney(4.2)
  • Liver(4)
  • Stomach(4)
  • Gall bladder(3.9)
  • Intestine(3.9)
  • Skin(3.9)
  • Nervous system(3.9)
  • Adrenal gland(3.8)
  • Pancreas(3.8)
  • Lung(3.7)
  • Bone marrow(3.5)
  • Lymph node(3.4)
  • Thyroid gland(3.3)
  • Spleen(3.2)
  • Blood(2.4)
genes like me logo Genes that share expression patterns with MT-CO3: 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-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
(Pan troglodytes)
Mammalia MT-CO3 34
  • 97 (a)
COX3 33
  • 90.42 (n)
(Mus musculus)
Mammalia mt-Co3 16 34
  • 87 (a)
COX3 33
  • 76.25 (n)
(Monodelphis domestica)
Mammalia COIII 34
  • 85 (a)
(Ornithorhynchus anatinus)
Mammalia MT-CO3 34
  • 84 (a)
(Bos Taurus)
Mammalia COX3 34 33
  • 79.23 (n)
(Rattus norvegicus)
Mammalia COX3 33
  • 78.29 (n)
(Canis familiaris)
Mammalia COX3 34 33
  • 74.71 (n)
(Gallus gallus)
Aves MT-CO3 34
  • 75 (a)
COX3 33
  • 71.65 (n)
(Anolis carolinensis)
Reptilia COX3 34
  • 78 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia COX3 33
  • 72.29 (n)
Str.8155 33
(Danio rerio)
Actinopterygii mt-co3 34
  • 81 (a)
COX3 33
  • 71.52 (n)
Dr.24911 33
fruit fly
(Drosophila melanogaster)
Insecta mt:CoIII 34
  • 65 (a)
COX3 33
  • 64.61 (n)
African malaria mosquito
(Anopheles gambiae)
Insecta COX3 33
  • 62.95 (n)
(Caenorhabditis elegans)
Secernentea MTCE.23 34
  • 43 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes COX3 33
  • 51.29 (n)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AMI003W 33
  • 50.06 (n)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes COX3 33 34 36
  • 49.61 (n)
thale cress
(Arabidopsis thaliana)
eudicotyledons cox3 33
  • 59.04 (n)
(Oryza sativa)
Liliopsida Os.24741 33
sea squirt
(Ciona savignyi)
Ascidiacea -- 34
  • 48 (a)
Species where no ortholog for MT-CO3 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)
  • 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 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
rs1057516063 likely-pathogenic, Optic neuropathy 9,166(+) T/C
rs1057516064 likely-pathogenic, Developmental delay, Mitochondrial encephalopathy, Seizure Disorders 9,237(+) G/A coding_sequence_variant, missense_variant

Additional Variant Information for MT-CO3 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-CO3 Gene

Disorders for MT-CO3 Gene

MalaCards: The human disease database

(15) MalaCards diseases for MT-CO3 Gene - From: HGMD, ClinVar, GTR, Orphanet, Swiss-Prot, DISEASES, Novoseek, and GeneCards

- elite association - COSMIC cancer census association via MalaCards


  • 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.
  • 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.
  • 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. {ECO:0000269 PubMed:8630495}. Note=The gene represented in this entry may be involved in disease pathogenesis.

Additional Disease Information for MT-CO3

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

No data available for Genatlas for MT-CO3 Gene

Publications for MT-CO3 Gene

  1. A MELAS syndrome family harboring two mutations in mitochondrial genome. (PMID: 18587274) Choi BO … Chung KW (Experimental & molecular medicine 2008) 3 4 22 58
  2. Mitochondrial DNA mutations with Leber's hereditary optic neuropathy in Japanese patients with open-angle glaucoma. (PMID: 16604388) Inagaki Y … Glaucoma Gene Research Group (Japanese journal of ophthalmology 2006) 3 44 58
  3. Mutations in mtDNA-encoded cytochrome c oxidase subunit genes causing isolated myopathy or severe encephalomyopathy. (PMID: 16288875) Horváth R … Lochmüller H (Neuromuscular disorders : NMD 2005) 3 22 58
  4. A microdeletion in cytochrome c oxidase (COX) subunit III associated with COX deficiency and recurrent myoglobinuria. (PMID: 8630495) Keightley JA … Kennaway NG (Nature genetics 1996) 3 4 58
  5. Cytochrome c oxidase mutations in Leber hereditary optic neuropathy. (PMID: 8240356) Johns DR … Neufeld MJ (Biochemical and biophysical research communications 1993) 4 22 58

Products for MT-CO3 Gene

Sources for MT-CO3 Gene

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