Seroatlas · Human Serome Atlas

MT-CO2

Cytochrome c oxidase subunit 2

Also known as: CO2, COX2, COX2_HUMAN, MTCO2

Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene

Protein identityUniProt · HPA

UniProt accession
P00403
Gene
MT-CO2
Ensembl
ENSG00000198712
Chromosome
MT
Canonical length
227 aa
Protein class
Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets, Predicted membrane proteins, Transporters

OverviewNCBI Gene

Contributes to cytochrome-c oxidase activity. Predicted to be involved in mitochondrial electron transport, cytochrome c to oxygen and positive regulation of vasoconstriction. Located in mitochondrial inner membrane. Part of respiratory chain complex IV. Biomarker of Huntington's disease and stomach cancer. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

227 residues, UniProt reviewed canonical sequence.

>P00403|MT-CO2
     1  MAHAAQVGLQ DATSPIMEEL ITFHDHALMI IFLICFLVLY ALFLTLTTKL TNTNISDAQE
    61  METVWTILPA IILVLIALPS LRILYMTDEV NDPSLTIKSI GHQWYWTYEY TDYGGLIFNS
   121  YMLPPLFLEP GDLRLLDVDN RVVLPIEAPI RMMITSQDVL HSWAVPTLGL KTDAIPGRLN
   181  QTTFTATRPG VYYGQCSEIC GANHSFMPIV LELIPLKIFE MGPVFTL

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against MT-CO2 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability
Other membrane
Secreted
No
Transmembrane segments
2
Mean surface accessibility (rSASA)
0.33
Highest tissue expression
214,511 nTPM

Expression across tissuesHPA

Tissue

  • heart muscle: 214,511 nTPM
  • basal ganglia: 153,612 nTPM
  • hippocampal formation: 122,807 nTPM
  • midbrain: 118,522 nTPM
  • kidney: 112,733 nTPM
  • amygdala: 110,908 nTPM

Single-cell type

  • hepatocytes: 57,294 nCPM
  • colonocytes: 40,015 nCPM
  • enterocytes: 36,307 nCPM
  • goblet cells: 25,070 nCPM
  • enteric transient amplifying cells: 24,655 nCPM
  • paneth cells: 24,338 nCPM

Immune cell

  • basophil: 21,134 nTPM
  • eosinophil: 15,810 nTPM
  • classical monocyte: 13,577 nTPM
  • memory B-cell: 11,896 nTPM
  • naive B-cell: 10,997 nTPM
  • non-classical monocyte: 10,118 nTPM

Brain region

  • medulla oblongata: 88,940 nTPM
  • pons: 83,622 nTPM
  • thalamus: 80,500 nTPM
  • choroid plexus: 80,069 nTPM
  • cerebral cortex: 78,180 nTPM
  • midbrain: 63,566 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about MT-CO2.

Disease | AllUniProt

Conditions MT-CO2 is implicated in, by any mechanism.

  • Mitochondrial complex IV deficiency (MT-C4D) MIM:220110

Disease | GeneticClinVar

10 pathogenic / likely-pathogenic of 130 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

Disease | ImmuneIEDB

Conditions an epitope on MT-CO2 was assayed in.

OntologyGO

Biological processes

Molecular functions

Cellular components

Protein domainsUniProt · Pfam · InterPro

  • Cupredoxin
  • Copper centre Cu(A)
  • Cytochrome c oxidase subunit II-like C-terminal
  • Cytochrome C oxidase subunit II, transmembrane domain
  • Cytochrome c oxidase, subunit II
  • Cytochrome c oxidase subunit 2, C-terminal
  • Cytochrome C oxidase subunit II, transmembrane domain superfamily
  • Cytochrome c/quinol oxidase subunit II
  • Cytochrome C oxidase subunit II, periplasmic domain
  • Cytochrome C oxidase subunit II, transmembrane domain

KeywordsUniProt

InteractionsUniProt · HPA

Protein binding partners of MT-CO2 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

Antibody and autoantibody relevanceSeroatlas analysis

Seroatlas reads MT-CO2 as an antibody target. Whether an autoantibody or antibody against MT-CO2 could matter depends on whether native MT-CO2 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

MT-CO2 is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.

Annotation status

The present source text does not explicitly label MT-CO2 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

Canonical record: https://seroatlas.com/gene/MT-CO2. Study-independent annotations aggregated from UniProt, Human Protein Atlas, PubMed, IEDB, Pfam, InterPro, Gene Ontology, AlphaFold, gnomAD, DepMap, ClinVar, TCGA. Catalog release seroatlas-reviewed-human-uniprot-20260313.

Seroatlas is the reference for exploring autoantibody and antibody serology at the human-protein level: the autoreactome and human serome, multiplex serology (HuProt, HuScan, VirScan, PhIP-Seq).

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