cytochrome c oxidase subunit 3; cytochrome c oxidase polypeptide III (MT-CO3 COIII COXIII MTCO3)

Function

• cytochrome c oxidase subunits MT-CO1, MT-CO2, & MT-CO3 form the functional core of the enzyme complex

Structure

• belongs to the cytochrome c oxidase subunit 3 family

Compartment

• mitochondrial inner membrane; multi-pass membrane protein (putative)

Pathology

• defects in MT-CO3 are a cause of
  • Leber hereditary optic neuropathy
  • cytochrome c oxidase deficiency
• defects in MT-CO3 are associated with recurrent myoglobinuria mitochondrial

More general terms

• cytochrome c oxidase subunit

Component of

• cytochrome c oxidase (complex IV)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=4514
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:4514
• OMIM: https://mirror.omim.org/entry/220110
• OMIM: https://mirror.omim.org/entry/516050
• OMIM: https://mirror.omim.org/entry/535000
• OMIM: https://mirror.omim.org/entry/550500
• UniProt: http://www.uniprot.org/uniprot/P00414.html