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Protein

Cytochrome c oxidase copper chaperone

Gene

COX17

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

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).1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi23CopperCombined sources2 Publications1
Metal bindingi24CopperCombined sources2 Publications1

GO - Molecular functioni

  • copper chaperone activity Source: CAFA
  • copper ion binding Source: ProtInc
  • cuprous ion binding Source: CAFA

GO - Biological processi

  • brain development Source: Ensembl
  • copper ion transport Source: GO_Central
  • generation of precursor metabolites and energy Source: ProtInc
  • heart development Source: Ensembl
  • mitochondrial respiratory chain complex IV assembly Source: CAFA
  • positive regulation of cell proliferation Source: CAFA
  • positive regulation of cytochrome-c oxidase activity Source: CAFA

Keywordsi

Molecular functionChaperone
LigandCopper, Metal-binding

Enzyme and pathway databases

ReactomeiR-HSA-1268020 Mitochondrial protein import

Names & Taxonomyi

Protein namesi
Recommended name:
Cytochrome c oxidase copper chaperone
Gene namesi
Name:COX17
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 3

Organism-specific databases

EuPathDBiHostDB:ENSG00000138495.6
HGNCiHGNC:2264 COX17
MIMi604813 gene
neXtProtiNX_Q14061

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi10063
OpenTargetsiENSG00000138495
PharmGKBiPA26780

Polymorphism and mutation databases

BioMutaiCOX17
DMDMi2493873

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00002135382 – 63Cytochrome c oxidase copper chaperoneAdd BLAST62

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei14PhosphoserineCombined sources1
Disulfide bondi26 ↔ 55PROSITE-ProRule annotationCombined sources2 Publications
Disulfide bondi36 ↔ 45PROSITE-ProRule annotationCombined sources2 Publications

Keywords - PTMi

Disulfide bond, Phosphoprotein

Proteomic databases

EPDiQ14061
MaxQBiQ14061
PaxDbiQ14061
PeptideAtlasiQ14061
PRIDEiQ14061
ProteomicsDBi59805

PTM databases

iPTMnetiQ14061
PhosphoSitePlusiQ14061

Expressioni

Tissue specificityi

Ubiquitous.1 Publication

Gene expression databases

BgeeiENSG00000138495
CleanExiHS_COX17
ExpressionAtlasiQ14061 baseline and differential
GenevisibleiQ14061 HS

Organism-specific databases

HPAiHPA042226
HPA048158

Interactioni

Subunit structurei

Interacts with COA1. Interacts with the chaperone CHCHD4; this is important for correct folding and the formation of disulfide bonds that stabilize the structure.2 Publications

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi115374, 27 interactors
DIPiDIP-46087N
IntActiQ14061, 21 interactors
STRINGi9606.ENSP00000261070

Structurei

Secondary structure

163
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi22 – 24Combined sources3
Helixi27 – 39Combined sources13
Helixi42 – 44Combined sources3
Helixi49 – 62Combined sources14

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2L0YNMR-B1-63[»]
2LGQNMR-A1-63[»]
2RN9NMR-A1-63[»]
2RNBNMR-A1-63[»]
DisProtiDP00543
ProteinModelPortaliQ14061
SMRiQ14061
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ14061

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini23 – 63CHCHPROSITE-ProRule annotationAdd BLAST41

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi26 – 36Cx9C motif 1PROSITE-ProRule annotationAdd BLAST11
Motifi45 – 55Cx9C motif 2PROSITE-ProRule annotationAdd BLAST11

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi2 – 27Ala/Pro-richAdd BLAST26

Sequence similaritiesi

Belongs to the COX17 family.Curated

Phylogenomic databases

eggNOGiKOG3496 Eukaryota
ENOG410XVYQ LUCA
GeneTreeiENSGT00390000002329
HOGENOMiHOG000195037
HOVERGENiHBG054258
InParanoidiQ14061
KOiK02260
PhylomeDBiQ14061
TreeFamiTF105074

Family and domain databases

InterProiView protein in InterPro
IPR009069 Cys_alpha_HP_mot_SF
IPR007745 Cyt_c_oxidase_Cu-chaperone
PANTHERiPTHR16719 PTHR16719, 1 hit
PfamiView protein in Pfam
PF05051 COX17, 1 hit
ProDomiView protein in ProDom or Entries sharing at least one domain
PD014904 Cyt_c_oxidase_Cu-chaperone, 1 hit
SUPFAMiSSF47072 SSF47072, 1 hit
PROSITEiView protein in PROSITE
PS51808 CHCH, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q14061-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MPGLVDSNPA PPESQEKKPL KPCCACPETK KARDACIIEK GEEHCGHLIE
60
AHKECMRALG FKI
Length:63
Mass (Da):6,915
Last modified:January 23, 2007 - v2
Checksum:iE97090F939E78276
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L77701 mRNA Translation: AAA98114.1
AF269244, AF269243 Genomic DNA Translation: AAF82569.1
AK312143 mRNA Translation: BAG35079.1
CH471052 Genomic DNA Translation: EAW79545.1
CH471052 Genomic DNA Translation: EAW79547.1
CH471052 Genomic DNA Translation: EAW79548.1
CH471052 Genomic DNA Translation: EAW79550.1
CH471052 Genomic DNA Translation: EAW79551.1
BC010933 mRNA Translation: AAH10933.1
BC105280 mRNA Translation: AAI05281.1
BC108317 mRNA Translation: AAI08318.1
CCDSiCCDS2993.1
PIRiT09533
RefSeqiNP_005685.1, NM_005694.1
UniGeneiHs.534383

Genome annotation databases

EnsembliENST00000261070; ENSP00000261070; ENSG00000138495
ENST00000468918; ENSP00000417805; ENSG00000138495
ENST00000497116; ENSP00000417923; ENSG00000138495
GeneIDi10063
KEGGihsa:10063
UCSCiuc003ecz.2 human

Similar proteinsi

Entry informationi

Entry nameiCOX17_HUMAN
AccessioniPrimary (citable) accession number: Q14061
Secondary accession number(s): B2R5D2, D3DN84, Q3MHD6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: January 23, 2007
Last modified: June 20, 2018
This is version 150 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

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