MGP Database

MGP004073

UniProt Annotations

Entry Information
Gene Namecopper chaperone for superoxide dismutase
Protein EntryCCS_HUMAN
UniProt IDO14618
SpeciesHuman
Comments
Comment typeDescription
CofactorName=Cu(2+); Xref=ChEBI:CHEBI:29036; Note=Binds 2 copper ions per subunit.;
CofactorName=Zn(2+); Xref=ChEBI:CHEBI:29105; Note=Binds 1 zinc ion per subunit.;
FunctionDelivers copper to copper zinc superoxide dismutase (SOD1).
PtmUbiquitinion by XIAP/BIRC4 leads to enhancement of its chaperone activity toward its physiologic target, SOD1, rather than proteasomal degradation. XIAP/BIRC4 preferentially ubiquitinates at Lys-241. {ECO:0000269|PubMed:20154138}.
SimilarityContains 1 HMA domain. {ECO:0000255|PROSITE- ProRule:PRU00280}.
SimilarityIn the C-terminal section; belongs to the Cu-Zn superoxide dismutase family. {ECO:0000305}.
Subcellular LocationCytoplasm {ECO:0000269|PubMed:9726962}.
SubunitHomodimer, and heterodimer with SOD1. Interacts with COMMD1. Interacts with XIAP/BIRC4. {ECO:0000269|PubMed:10677207, ECO:0000269|PubMed:20154138, ECO:0000269|PubMed:20595380, ECO:0000269|PubMed:22508683, ECO:0000269|PubMed:23625804, ECO:0000269|PubMed:9726962}.
Tissue SpecificityUbiquitous.
  logo