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Aliases for CCS Gene

Aliases for CCS Gene

  • Copper Chaperone For Superoxide Dismutase 2 3
  • Superoxide Dismutase Copper Chaperone 4

External Ids for CCS Gene

Previous GeneCards Identifiers for CCS Gene

  • GC11P068894
  • GC11P068043
  • GC11P066610
  • GC11P066136
  • GC11P066117
  • GC11P066360
  • GC11P062688

Summaries for CCS Gene

Entrez Gene Summary for CCS Gene

  • 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. [provided by RefSeq, Jul 2008]

GeneCards Summary for CCS Gene

CCS (Copper Chaperone For Superoxide Dismutase) is a Protein Coding gene. Among its related pathways are Cellular Senescence and Amyotrophic lateral sclerosis (ALS). GO annotations related to this gene include copper ion binding and copper ion transmembrane transporter activity. An important paralog of this gene is SOD3.

UniProtKB/Swiss-Prot for CCS Gene

  • Delivers copper to copper zinc superoxide dismutase (SOD1)

Gene Wiki entry for CCS Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for CCS Gene

Genomics for CCS Gene

Regulatory Elements for CCS Gene

Epigenetics Products

  • DNA Methylation CpG Assay Predesigned for Pyrosequencing in human,mouse,rat

Genomic Location for CCS Gene

66,592,821 bp from pter
66,606,019 bp from pter
13,199 bases
Plus strand

Genomic View for CCS Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for CCS Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for CCS Gene

Proteins for CCS Gene

  • Protein details for CCS Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Copper chaperone for superoxide dismutase
    Protein Accession:
    Secondary Accessions:
    • Q2M366
    • Q8NEV0

    Protein attributes for CCS Gene

    274 amino acids
    Molecular mass:
    29041 Da
    Name=Cu(2+); Xref=ChEBI:CHEBI:29036; Note=Binds 2 copper ions per subunit.;
    Name=Zn(2+); Xref=ChEBI:CHEBI:29105; Note=Binds 1 zinc ion per subunit.;
    Quaternary structure:
    • Homodimer, and heterodimer with SOD1. Interacts with COMMD1. Interacts with XIAP/BIRC4.

    Three dimensional structures from OCA and Proteopedia for CCS Gene

neXtProt entry for CCS Gene

Proteomics data for CCS Gene at MOPED

Post-translational modifications for CCS Gene

  • Ubiquitinion 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.
  • Ubiquitination at Lys76, Lys189, Lys216, and Lys241
  • Modification sites at PhosphoSitePlus

Other Protein References for CCS Gene

ENSEMBL proteins:
Reactome Protein details:
REFSEQ proteins:

No data available for DME Specific Peptides for CCS Gene

Domains for CCS Gene

Suggested Antigen Peptide Sequences for CCS Gene

Graphical View of Domain Structure for InterPro Entry



  • Contains 1 HMA domain.
  • In the C-terminal section; belongs to the Cu-Zn superoxide dismutase family.
  • Contains 1 HMA domain.
  • In the C-terminal section; belongs to the Cu-Zn superoxide dismutase family.
genes like me logo Genes that share domains with CCS: view

No data available for Gene Families for CCS Gene

Function for CCS Gene

Molecular function for CCS Gene

GENATLAS Biochemistry:
copper chaperone for superoxide dismutase,S cerevisiae LYS7 ortholog
UniProtKB/Swiss-Prot Function:
Delivers copper to copper zinc superoxide dismutase (SOD1)

Gene Ontology (GO) - Molecular Function for CCS Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004784 superoxide dismutase activity IBA --
GO:0005375 copper ion transmembrane transporter activity TAS 9726962
GO:0005507 copper ion binding IBA --
GO:0005515 protein binding IPI 20154138
GO:0008270 zinc ion binding IBA --
genes like me logo Genes that share ontologies with CCS: view
genes like me logo Genes that share phenotypes with CCS: view

Animal Models for CCS Gene

MGI Knock Outs for CCS:

Animal Model Products

miRNA for CCS Gene

miRTarBase miRNAs that target CCS

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for CCS

In Situ Assay Products

Flow Cytometry Products

No data available for Enzyme Numbers (IUBMB) , Transcription Factor Targeting and HOMER Transcription for CCS Gene

Localization for CCS Gene

Subcellular locations from UniProtKB/Swiss-Prot for CCS Gene


Subcellular locations from

Jensen Localization Image for CCS Gene COMPARTMENTS Subcellular localization image for CCS gene
Compartment Confidence
nucleus 5
cytosol 3
mitochondrion 3
extracellular 2
chloroplast 1
cytoskeleton 1
plasma membrane 1

Gene Ontology (GO) - Cellular Components for CCS Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005634 nucleus IDA 9726962
GO:0005737 cytoplasm IDA 9726962
GO:0005829 cytosol TAS --
genes like me logo Genes that share ontologies with CCS: view

Pathways for CCS Gene

genes like me logo Genes that share pathways with CCS: view

Pathways by source for CCS Gene

1 BioSystems pathway for CCS Gene
1 KEGG pathway for CCS Gene

PCR Array Products

Gene Ontology (GO) - Biological Process for CCS Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0000302 response to reactive oxygen species TAS --
GO:0006801 superoxide metabolic process TAS 9295278
GO:0015680 intracellular copper ion transport TAS 9726962
GO:0019430 removal of superoxide radicals IBA --
GO:0030001 metal ion transport --
genes like me logo Genes that share ontologies with CCS: view

Compounds for CCS Gene

(2) HMDB Compounds for CCS Gene

Compound Synonyms Cas Number PubMed IDs
  • Cu
  • Ondansetron

(6) Novoseek inferred chemical compound relationships for CCS Gene

Compound -log(P) Hits PubMed IDs
zinc superoxide 77.4 3
copper 77 47
superoxide 65.8 15
zinc 48.7 2
cysteine 36.6 3
genes like me logo Genes that share compounds with CCS: view

Transcripts for CCS Gene

Unigene Clusters for CCS Gene

Copper chaperone for superoxide dismutase:
Representative Sequences:

miRNA Products

Inhibitory RNA Products

  • Predesigned siRNA for gene silencing in human,mouse,rat for CCS

Flow Cytometry Products

Alternative Splicing Database (ASD) splice patterns (SP) for CCS Gene

ExUns: 1a · 1b ^ 2 ^ 3 ^ 4a · 4b · 4c · 4d ^ 5a · 5b · 5c ^ 6a · 6b · 6c ^ 7 ^ 8a · 8b
SP1: - - - -
SP2: - - -
SP3: - - -
SP5: - - -
SP7: -
SP8: -

Relevant External Links for CCS Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for CCS Gene

mRNA expression in normal human tissues for CCS Gene

Protein differential expression in normal tissues for CCS Gene

This gene is overexpressed in Peripheral blood mononuclear cells (6.7).

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for CCS Gene

SOURCE GeneReport for Unigene cluster for CCS Gene Hs.502917

mRNA Expression by UniProt/SwissProt for CCS Gene

Tissue specificity: Ubiquitous
genes like me logo Genes that share expressions with CCS: view

Expression partners for CCS Gene

* - Elite partner

Primer Products

In Situ Assay Products

No data available for mRNA expression in embryonic tissues and stem cells from LifeMap Discovery and mRNA differential expression in normal tissues for CCS Gene

Orthologs for CCS Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for CCS Gene

Organism Taxonomy Gene Similarity Type Details
(Canis familiaris)
Mammalia CCS 35
  • 87.35 (n)
  • 89.05 (a)
CCS 36
  • 60 (a)
(Mus musculus)
Mammalia Ccs 35
  • 86.01 (n)
  • 86.86 (a)
Ccs 16
Ccs 36
  • 87 (a)
(Pan troglodytes)
Mammalia CCS 35
  • 99.15 (n)
  • 100 (a)
CCS 36
  • 99 (a)
(Rattus norvegicus)
Mammalia Ccs 35
  • 85.89 (n)
  • 85.04 (a)
(Bos Taurus)
Mammalia CCS 36
  • 91 (a)
(Monodelphis domestica)
Mammalia CCS 36
  • 77 (a)
(Anolis carolinensis)
Reptilia CCS 36
  • 66 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia ccs 35
  • 60.76 (n)
  • 62.73 (a)
(Danio rerio)
Actinopterygii ccs 35
  • 65.87 (n)
  • 71.71 (a)
ccs 36
  • 68 (a)
rainbow trout
(Oncorhynchus mykiss)
Actinopterygii Omy.5828 35
fruit fly
(Drosophila melanogaster)
Insecta CCS 37
  • 48 (a)
CCS 35
  • 55.6 (n)
  • 52.46 (a)
CCS 36
  • 44 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP007026 35
  • 53.45 (n)
  • 46.4 (a)
(Caenorhabditis elegans)
Secernentea sod-1 36
  • 43 (a)
sod-5 36
  • 43 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0F26917g 35
  • 43.94 (n)
  • 31.82 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes CCS1 35
  • 41.78 (n)
  • 30.22 (a)
SOD1 36
  • 42 (a)
CCS1 38
thale cress
(Arabidopsis thaliana)
eudicotyledons CCS 35
  • 47.27 (n)
  • 43.64 (a)
(Oryza sativa)
Liliopsida Os04g0573200 35
  • 48.34 (n)
  • 41.56 (a)
bread mold
(Neurospora crassa)
Ascomycetes NCU07851 35
  • 49.58 (n)
  • 37.82 (a)
Species with no ortholog for CCS:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • chicken (Gallus gallus)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • platypus (Ornithorhynchus anatinus)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea squirt (Ciona intestinalis)
  • sea squirt (Ciona savignyi)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for CCS Gene

Gene Tree for CCS (if available)
Gene Tree for CCS (if available)

Paralogs for CCS Gene

Paralogs for CCS Gene

(1) SIMAP similar genes for CCS Gene using alignment to 5 proteins:

genes like me logo Genes that share paralogs with CCS: view

Variants for CCS Gene

Sequence variations from dbSNP and Humsavar for CCS Gene

SNP ID Clin Chr 11 pos Sequence Context AA Info Type MAF
rs10061 -- 66,605,730(+) GCTCC(C/G)CTGGC missense, reference, nc-transcript-variant
rs498045 -- 66,595,526(+) GCTGT(A/G)GTTTT intron-variant
rs503250 -- 66,604,595(+) ATTCA(C/T)CCTGT intron-variant
rs556759 -- 66,604,919(+) GACAG(C/T)CAAGG intron-variant
rs1127141 -- 66,605,783(+) ACCAT(C/T)TGGGA reference, synonymous-codon, nc-transcript-variant

Structural Variations from Database of Genomic Variants (DGV) for CCS Gene

Variant ID Type Subtype PubMed ID
dgv1187n71 CNV Loss 21882294
nsv522277 CNV Loss 19592680
nsv363 CNV Insertion 18451855
nsv513321 CNV Insertion 21212237
nsv897789 CNV Gain 21882294

Relevant External Links for CCS Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for CCS Gene

Disorders for CCS Gene

(1) University of Copenhagen DISEASES for CCS Gene

(2) Novoseek inferred disease relationships for CCS Gene

Disease -log(P) Hits PubMed IDs
amyotrophic lateral sclerosis 79.6 4
alzheimers disease 1.1 1

Relevant External Links for CCS

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with CCS: view

No data available for MalaCards , OMIM , UniProtKB/Swiss-Prot and Genatlas for CCS Gene

Publications for CCS Gene

  1. The copper chaperone CCS directly interacts with copper/zinc superoxide dismutase. (PMID: 9726962) Casareno R.L.B. … Gitlin J.D. (J. Biol. Chem. 1998) 3 4 23
  2. Cysteine-to-serine mutants of the human copper chaperone for superoxide dismutase reveal a copper cluster at a domain III dimer interface. (PMID: 15736924) Stasser J.P. … Blackburn N.J. (Biochemistry 2005) 3 4 23
  3. Regulation of the copper chaperone CCS by XIAP-mediated ubiquitination. (PMID: 20154138) Brady G.F. … Duckett C.S. (Mol. Cell. Biol. 2010) 3 4 23
  4. The copper chaperone for superoxide dismutase. (PMID: 9295278) Culotta V.C. … Gitlin J.D. (J. Biol. Chem. 1997) 2 3 4
  5. Oxidative stress, telomere length and biomarkers of physical aging in a cohort aged 79 years from the 1932 Scottish Mental Survey. (PMID: 18977241) Starr J.M. … Deary I.J. (Mech. Ageing Dev. 2008) 3 48

Products for CCS Gene

Sources for CCS Gene

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