Q14061 · COX17_HUMAN

  • Protein
    Cytochrome c oxidase copper chaperone
  • Gene
    COX17
  • Status
    UniProtKB reviewed (Swiss-Prot)
  • Amino acids
  • Protein existence
    Evidence at protein level
  • Annotation score
    5/5

Function

function

Copper metallochaperone essential for the assembly of the mitochondrial respiratory chain complex IV (CIV), also known as cytochrome c oxidase. Binds two copper ions and delivers them to the metallochaperone SCO1 which transports the copper ions to the Cu(A) site on the cytochrome c oxidase subunit II (MT-CO2/COX2).

Features

Showing features for binding site.

16351015202530354045505560
MPGLVDSNPAPPESQEKKPLKPCCACPETKKARDACIIEKGEEHCGHLIEAHKECMRALGFKI
TypeIDPosition(s)Description
Binding site23Cu cation (UniProtKB | ChEBI)
Binding site24Cu cation (UniProtKB | ChEBI)

GO annotations

all annotationsall molecular functionvirus receptor activitydna bindingrna bindingcytoskeletal motor activitycatalytic activitygtpase activitystructural molecule activitytransporter activitycytoskeletal protein bindinglipid bindingcyclase activityantioxidant activityoxidoreductase activitytransferase activityhydrolase activitylyase activityisomerase activityligase activityprotein tag activitycargo receptor activityhistone bindingprotein folding chaperonetranslation regulator activitynutrient reservoir activityreceptor ligand activitymolecular transducer activitymolecular adaptor activitytoxin activitycell adhesion mediator activitymolecular function regulator activityvirus coreceptor activitycatalytic activity, acting on a proteincatalytic activity, acting on dnacatalytic activity, acting on rnamolecular carrier activitytranscription regulator activitygeneral transcription initiation factor activitymolecular sensor activitymolecular sequestering activityatp-dependent activityother molecular functionall biological processmitotic cell cyclecytokinesiscytoplasmic translationimmune system processmuscle system processcirculatory system processrenal system processrespiratory system processcarbohydrate metabolic processgeneration of precursor metabolites and energydna replicationdna repairdna recombinationchromatin organizationdna-templated transcriptionregulation of dna-templated transcriptiontrna metabolic processprotein foldingprotein glycosylationamino acid metabolic processmodified amino acid metabolic processlipid metabolic processvitamin metabolic processsulfur compound metabolic processintracellular protein transportnucleocytoplasmic transportautophagyinflammatory responsemitochondrion organizationcytoskeleton organizationmicrotubule-based movementperoxisome organizationlysosome organizationchromosome segregationcell adhesionestablishment or maintenance of cell polarityprogrammed cell deathphotosynthesismrna metabolic processsnrna metabolic processvesicle-mediated transportreproductive processdigestive system processsignalingcell differentiationprotein catabolic processextracellular matrix organizationregulatory ncrna-mediated gene silencingtelomere organizationcell junction organizationwound healingribosome biogenesiscilium organizationanatomical structure developmentcell motilitynervous system processendocrine processprotein maturationtransmembrane transportnucleobase-containing small molecule metabolic processhepaticobiliary system processmembrane organizationprotein-containing complex assemblycell wall organization or biogenesisnitrogen cycle metabolic processprotein localization to plasma membranedefense response to other organismdetoxificationmeiotic nuclear divisionmitotic nuclear divisionmitochondrial gene expressioncarbohydrate derivative metabolic processother biological processall cellular componentnuclear chromosomeextracellular regionextracellular spacecell wallnucleusnuclear envelopenucleoplasmchromosomenucleolusmitochondrionlysosomeendosomevacuoleperoxisomeendoplasmic reticulumgolgi apparatuslipid dropletmicrotubule organizing centercytosolribosomecytoskeletonplasma membraneciliumplastidthylakoidexternal encapsulating structureextracellular matrixcytoplasmic vesicleorganelleother cellular component
Cell color indicative of number of GO terms
AspectTerm
Cellular Componentcytoplasm
Cellular Componentmitochondrial intermembrane space
Molecular Functioncopper chaperone activity
Molecular Functioncopper ion binding
Molecular Functioncuprous ion binding
Molecular Functionenzyme activator activity
Biological Processcopper ion transport
Biological Processgeneration of precursor metabolites and energy
Biological Processmitochondrial cytochrome c oxidase assembly
Biological Processpositive regulation of cell population proliferation
Biological Processpositive regulation of cytochrome-c oxidase activity

Keywords

Enzyme and pathway databases

Names & Taxonomy

Protein names

  • Recommended name
    Cytochrome c oxidase copper chaperone

Gene names

    • Name
      COX17

Organism names

  • Taxonomic identifier
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Primates > Haplorrhini > Catarrhini > Hominidae > Homo

Accessions

  • Primary accession
    Q14061
  • Secondary accessions
    • B2R5D2
    • D3DN84
    • Q3MHD6

Proteomes

Organism-specific databases

Subcellular Location

Disease & Variants

Variants

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The viewer provides 88 variants from UniProt as well as other sources including ClinVar and dbSNP.

Go to variant viewer

Organism-specific databases

Miscellaneous

Chemistry

Genetic variation databases

PTM/Processing

Features

Showing features for initiator methionine, chain, modified residue (large scale data), modified residue, disulfide bond.

TypeIDPosition(s)SourceDescription
Initiator methionine1UniProtRemoved
ChainPRO_00002135382-63UniProtCytochrome c oxidase copper chaperone
Modified residue (large scale data)7PRIDEPhosphoserine
Modified residue14UniProtPhosphoserine
Modified residue (large scale data)14PRIDEPhosphoserine
Modified residue18UniProtN6-acetyllysine
Disulfide bond26↔55UniProt
Modified residue30UniProtN6-acetyllysine
Disulfide bond36↔45UniProt

Post-translational modification

Acetylation by KAT8 promotes assembly of the mitochondrial respiratory chain complex IV (CIV).

Keywords

Proteomic databases

PTM databases

Expression

Tissue specificity

Ubiquitous.

Gene expression databases

Organism-specific databases

Interaction

Subunit

Interacts with COA1. Interacts with the chaperone CHCHD4; this is important for correct folding and the formation of disulfide bonds that stabilize the structure.

Binary interactions

Protein-protein interaction databases

Miscellaneous

Family & Domains

Features

Showing features for region, domain, motif.

TypeIDPosition(s)Description
Region1-20Disordered
Domain23-63CHCH
Motif26-36Cx9C motif 1
Motif45-55Cx9C motif 2

Sequence similarities

Belongs to the COX17 family.

Phylogenomic databases

Family and domain databases

Sequence

  • Sequence status
    Complete
  • Length
    63
  • Mass (Da)
    6,915
  • Last updated
    2007-01-23 v2
  • Checksum
    E97090F939E78276
MPGLVDSNPAPPESQEKKPLKPCCACPETKKARDACIIEKGEEHCGHLIEAHKECMRALGFKI

Computationally mapped potential isoform sequences

There are 3 potential isoforms mapped to this entry

View all
EntryEntry nameGene nameLength
C9J8T6C9J8T6_HUMANCOX1798
H7C5P5H7C5P5_HUMANCOX1745
H7C4E5H7C4E5_HUMANCOX1758

Keywords

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
L77701
EMBL· GenBank· DDBJ
AAA98114.1
EMBL· GenBank· DDBJ
mRNA
AF269244
EMBL· GenBank· DDBJ
AAF82569.1
EMBL· GenBank· DDBJ
Genomic DNA
AF269243
EMBL· GenBank· DDBJ
AAF82569.1
EMBL· GenBank· DDBJ
Genomic DNA
AK312143
EMBL· GenBank· DDBJ
BAG35079.1
EMBL· GenBank· DDBJ
mRNA
CH471052
EMBL· GenBank· DDBJ
EAW79545.1
EMBL· GenBank· DDBJ
Genomic DNA
CH471052
EMBL· GenBank· DDBJ
EAW79547.1
EMBL· GenBank· DDBJ
Genomic DNA
CH471052
EMBL· GenBank· DDBJ
EAW79548.1
EMBL· GenBank· DDBJ
Genomic DNA
CH471052
EMBL· GenBank· DDBJ
EAW79550.1
EMBL· GenBank· DDBJ
Genomic DNA
CH471052
EMBL· GenBank· DDBJ
EAW79551.1
EMBL· GenBank· DDBJ
Genomic DNA
BC010933
EMBL· GenBank· DDBJ
AAH10933.1
EMBL· GenBank· DDBJ
mRNA
BC105280
EMBL· GenBank· DDBJ
AAI05281.1
EMBL· GenBank· DDBJ
mRNA
BC108317
EMBL· GenBank· DDBJ
AAI08318.1
EMBL· GenBank· DDBJ
mRNA

Genome annotation databases

Similar Proteins

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