Names & Taxonomy
- Uniprot ID:
- P00395
- Entry Name:
- COX1_HUMAN
- Status:
- reviewed
- Protein Names:
- Cytochrome c oxidase subunit 1 (EC 1.9.3.1) (Cytochrome c oxidase polypeptide I)
- Gene Names:
- MT-CO1 COI COXI MTCO1
- Gene Names Primary:
- MT-CO1
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 513
- Sequence:
- MFADRWLFSTNHKDIGTLYLLFGAWAGVLGTALSLLIRAELGQPGNLLGNDHIYNVIVTAHAFVMIFFMVMPIMIGGFGNWLVPLMIGAPDMAFPRMNNMSFWLLPPSLLLLLASAMVEAGAGTGWTVYPPLAGNYSHPGASVDLTIFSLHLAGVSSILGAINFITTIINMKPPAMTQYQTPLFVWSVLITAVLLLLSLPVLAAGITMLLTDRNLNTTFFDPAGGGDPILYQHLFWFFGHPEVYILILPGFGMISHIVTYYSGKKEPFGYMGMVWAMMSIGFLGFIVWAHHMFTVGMDVDTRAYFTSATMIIAIPTGVKVFSWLATLHGSNMKWSAAVLWALGFIFLFTVGGLTGIVLANSSLDIVLHDTYYVVAHFHYVLSMGAVFAIMGGFIHWFPLFSGYTLDQTYAKIHFTIMFIGVNLTFFPQHFLGLSGMPRRYSDYPDAYTTWNILSSVGSFISLTAVMLMIFMIWEAFASKRKVLMVEEPSMNLEWLYGCPPPYHTFEEPVYMKS
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion inner membrane; Multi-pass membrane protein.
- Gene Encoded:
- Mitochondrion
Function
- 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. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B.
- Pathway:
- Energy metabolism; oxidative phosphorylation.
- Catalytic Activity:
- 4 ferrocytochrome c + O(2) + 4 H(+) = 4 ferricytochrome c + 2 H(2)O.
- Cross Reference Drug Bank:
- DB02659
- Gene Ontology Go:
- integral component of membrane
mitochondrial inner membrane
mitochondrial respiratory chain complex IV
respiratory chain complex IV
cytochrome-c oxidase activity
heme binding
iron ion binding
aerobic respiration
aging
cellular metabolic process
cerebellum development
gene expression
oxidative phosphorylation
respiratory electron transport chain
response to copper ion
response to electrical stimulus
response to oxidative stress
small molecule metabolic process
transcription initiation from RNA polymerase II promoter - Gene Ontology Biological Process:
- aerobic respiration
aging
cellular metabolic process
cerebellum development
gene expression
oxidative phosphorylation
respiratory electron transport chain
response to copper ion
response to electrical stimulus
response to oxidative stress
small molecule metabolic process
transcription initiation from RNA polymerase II promoter - Gene Ontology Molecular Function:
- cytochrome-c oxidase activity
heme binding
iron ion binding - Gene Ontology Cellular Component:
- integral component of membrane
mitochondrial inner membrane
mitochondrial respiratory chain complex IV
respiratory chain complex IV - Keywords:
- Complete proteome
Copper
Deafness
Disease mutation
Electron transport
Heme
Iron
Leber hereditary optic neuropathy
Membrane
Metal-binding
Mitochondrion
Mitochondrion inner membrane
Non-syndromic deafness
Oxidoreductase
Polymorphism
Reference proteome
Respiratory chain
Transmembrane
Transmembrane helix
Transport - Interacts With:
- Q9Y2R0; P13073; Q9BVV7