CCS (gene)

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Copper chaperone for superoxide dismutase
Protein CCS PDB 1do5.png
PDB rendering based on 1do5.
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols CCS ; MGC138260
External IDs OMIM603864 MGI1333783 HomoloGene3762 GeneCards: CCS Gene
RNA expression pattern
PBB GE CCS 203522 at tn.png
More reference expression data
Species Human Mouse
Entrez 9973 12460
Ensembl ENSG00000173992 ENSMUSG00000034108
UniProt O14618 Q9WU84
RefSeq (mRNA) NM_005125 NM_016892
RefSeq (protein) NP_005116 NP_058588
Location (UCSC) Chr 11:
66.36 – 66.37 Mb
Chr 19:
4.83 – 4.84 Mb
PubMed search [1] [2]

Copper chaperone for superoxide dismutase is a metalloprotein that in humans is encoded by the CCS gene.[1][2]

Copper chaperone for superoxide dismutase specifically delivers Cu to copper/zinc superoxide dismutase and may activate copper/zinc superoxide dismutase through direct insertion of the Cu cofactor.[2]


CCS (gene) has been shown to interact with SOD1[3] and APBA1.[4]


  1. ^ Culotta VC, Klomp LW, Strain J, Casareno RL, Krems B, Gitlin JD (Oct 1997). "The copper chaperone for superoxide dismutase". J Biol Chem 272 (38): 23469–72. doi:10.1074/jbc.272.38.23469. PMID 9295278. 
  2. ^ a b "Entrez Gene: CCS copper chaperone for superoxide dismutase". 
  3. ^ Casareno, R L; Waggoner D; Gitlin J D (Sep 1998). "The copper chaperone CCS directly interacts with copper/zinc superoxide dismutase". J. Biol. Chem. (UNITED STATES) 273 (37): 23625–8. doi:10.1074/jbc.273.37.23625. ISSN 0021-9258. PMID 9726962. 
  4. ^ McLoughlin, D M; Standen C L; Lau K F; Ackerley S; Bartnikas T P; Gitlin J D; Miller C C (Mar 2001). "The neuronal adaptor protein X11alpha interacts with the copper chaperone for SOD1 and regulates SOD1 activity". J. Biol. Chem. (United States) 276 (12): 9303–7. doi:10.1074/jbc.M010023200. ISSN 0021-9258. PMID 11115513. 

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