O03554 · COX1_TINMA

Function

function

Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules using 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix.

Catalytic activity

Cofactor

Protein has several cofactor binding sites:
heme (UniProtKB | Rhea| CHEBI:30413 )

Note: Binds 2 heme A groups non-covalently per subunit.
Cu cation (UniProtKB | Rhea| CHEBI:23378 )

Note: Binds a copper B center.

Pathway

Energy metabolism; oxidative phosphorylation.

Features

Showing features for binding site.

TypeIDPosition(s)Description
Binding site41Na+ (UniProtKB | ChEBI)
Binding site46Na+ (UniProtKB | ChEBI)
Binding site62Fe (UniProtKB | ChEBI) of Fe(II)-heme a (UniProtKB | ChEBI); low-spin; axial binding residue
Binding site241Cu cation B (UniProtKB | ChEBI)
Binding site245O2 (UniProtKB | ChEBI)
Binding site291Cu cation B (UniProtKB | ChEBI)
Binding site292Cu cation B (UniProtKB | ChEBI)

GO annotations

AspectTerm
Cellular Componentmitochondrial inner membrane
Cellular Componentrespiratory chain complex IV
Molecular Functioncytochrome-c oxidase activity
Molecular Functionheme binding
Molecular Functionmetal ion binding
Biological Processoxidative phosphorylation

Keywords

Enzyme and pathway databases

Names & Taxonomy

Protein names

  • Recommended name
    Cytochrome c oxidase subunit 1
  • EC number
  • Alternative names
    • Cytochrome c oxidase polypeptide I

Gene names

    • Name
      MT-CO1
    • Synonyms
      COI, COXI, MTCO1

Encoded on

  • Mitochondrion

Organism names

  • Taxonomic identifier
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Archelosauria > Archosauria > Dinosauria > Saurischia > Theropoda > Coelurosauria > Aves > Palaeognathae > Tinamiformes > Tinamidae > Tinamus

Accessions

  • Primary accession
    O03554

Subcellular Location

Features

Showing features for topological domain, transmembrane.

TypeIDPosition(s)Description
Topological domain1-12Mitochondrial matrix
Transmembrane13-41Helical; Name=I
Topological domain42-51Mitochondrial intermembrane
Transmembrane52-87Helical; Name=II
Topological domain88-95Mitochondrial matrix
Transmembrane96-118Helical; Name=III
Topological domain119-141Mitochondrial intermembrane
Transmembrane142-171Helical; Name=IV
Topological domain172-183Mitochondrial matrix
Transmembrane184-213Helical; Name=V
Topological domain214-228Mitochondrial intermembrane
Transmembrane229-262Helical; Name=VI
Topological domain263-270Mitochondrial matrix
Transmembrane271-287Helical; Name=VII
Topological domain288-299Mitochondrial intermembrane
Transmembrane300-328Helical; Name=VIII
Topological domain329-337Mitochondrial matrix

Keywords

PTM/Processing

Features

Showing features for chain, cross-link.

TypeIDPosition(s)Description
ChainPRO_00001834261-337Cytochrome c oxidase subunit 1
Cross-link241↔2451'-histidyl-3'-tyrosine (His-Tyr)

Interaction

Subunit

Component of the cytochrome c oxidase (complex IV, CIV), a multisubunit enzyme composed of 14 subunits. The complex is composed of a catalytic core of 3 subunits MT-CO1, MT-CO2 and MT-CO3, encoded in the mitochondrial DNA, and 11 supernumerary subunits COX4I, COX5A, COX5B, COX6A, COX6B, COX6C, COX7A, COX7B, COX7C, COX8 and NDUFA4, which are encoded in the nuclear genome. The complex exists as a monomer or a dimer and forms supercomplexes (SCs) in the inner mitochondrial membrane with NADH-ubiquinone oxidoreductase (complex I, CI) and ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII), resulting in different assemblies (supercomplex SCI1III2IV1 and megacomplex MCI2III2IV2) (By similarity).
As a newly synthesized protein, rapidly incorporates into a multi-subunit assembly intermediate in the inner membrane, called MITRAC (mitochondrial translation regulation assembly intermediate of cytochrome c oxidase) complex, whose core components are COA3/MITRAC12 and COX14. Within the MITRAC complex, interacts with COA3 and with SMIM20/MITRAC7; the interaction with SMIM20 stabilizes the newly synthesized MT-CO1 and prevents its premature turnover. Interacts with TMEM177 in a COX20-dependent manner (By similarity).

Structure

Family & Domains

Sequence similarities

Keywords

Family and domain databases

Sequence

  • Sequence status
    Fragment
  • Length
    337
  • Mass (Da)
    36,806
  • Last updated
    1997-07-01 v1
  • Checksum
    637DBE38B678F319
MTFITRWLFSTNHKDIGTLYLIFGAWAGMVGTALSLLIRAELGQPGTLLGDDQIYNVVVTAHAFVMIFFMVMPVMIGGFGNWLVPLMIGAPDMAFPRMNNMSFWLLPPSFLLLLASSTVETGVGTGWTVYPPLASNLAHAGASVDLAIFSLHLAGVSSILGAINFITTAINMKPPALTQYQTPLFVWSVLITAILLLLSLPVLAAGITMLLTDRNLNTTFFDPAGGGDPILYQHLFWFFGHPEVYILILPGFGIISHVVAYYAGKKEPFGYMGMVWAMLSIGFLGFIVWAHHMFTVGMDVDTRAYFTSATMIIAIPTGIKVFSWLATLHGGTIKWDP

Features

Showing features for non-terminal residue.

TypeIDPosition(s)Description
Non-terminal residue337

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
U76063
EMBL· GenBank· DDBJ
AAB61331.1
EMBL· GenBank· DDBJ
Genomic DNA

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