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    Ccs copper chaperone for superoxide dismutase [ Rattus norvegicus (Norway rat) ]

    Gene ID: 84485, updated on 9-Dec-2024

    Summary

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    Official Symbol
    Ccsprovided by RGD
    Official Full Name
    copper chaperone for superoxide dismutaseprovided by RGD
    Primary source
    RGD:620403
    See related
    EnsemblRapid:ENSRNOG00000047816 AllianceGenome:RGD:620403
    Gene type
    protein coding
    RefSeq status
    PROVISIONAL
    Organism
    Rattus norvegicus
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Rattus
    Summary
    Enables enzyme binding activity. Predicted to be involved in removal of superoxide radicals. Predicted to act upstream of or within positive regulation of oxidoreductase activity. Predicted to be located in mitochondrion and nucleus. Human ortholog(s) of this gene implicated in amyotrophic lateral sclerosis. Orthologous to human CCS (copper chaperone for superoxide dismutase). [provided by Alliance of Genome Resources, Dec 2024]
    Expression
    Biased expression in Liver (RPKM 294.2), Kidney (RPKM 212.4) and 9 other tissues See more
    Orthologs
    human mouse all
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    Genomic context

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    See Ccs in Genome Data Viewer
    Location:
    1q43
    Exon count:
    8
    Annotation release Status Assembly Chr Location
    RS_2024_02 current GRCr8 (GCF_036323735.1) 1 NC_086019.1 (211543192..211564354, complement)
    RS_2023_06 previous assembly mRatBN7.2 (GCF_015227675.2) 1 NC_051336.1 (202113792..202134931, complement)
    106 previous assembly Rnor_6.0 (GCF_000001895.5) 1 NC_005100.4 (220075251..220096319, complement)

    Chromosome 1 - NC_086019.1Genomic Context describing neighboring genes Neighboring gene RNA binding motif protein 14 Neighboring gene RNA binding motif protein 4B Neighboring gene proteasome subunit alpha type-3-like Neighboring gene RNA binding motif protein 4 Neighboring gene coiled-coil domain containing 87 Neighboring gene cathepsin F

    Genomic regions, transcripts, and products

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    Go to nucleotide: Graphics FASTA GenBank

    Expression

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    • Project title: A rat RNA-Seq transcriptomic BodyMap across 11 organs and 4 developmental stages
    • Description: 320 RNA samples isolated from 11 organs (adrenal gland, brain, heart, kidney, liver, lung, muscle, spleen, thymus, and testes or uterus) from both sexes of Fischer 344 rats across four developmental stages (2-, 6-, 21-, and 104-weeks-old)
    • BioProject: PRJNA238328
    • Publication: PMID 24510058
    • Analysis date: Mon Jun 6 17:44:12 2016

    Bibliography

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    Related articles in PubMed

    1. Copper delivery to the CNS by CuATSM effectively treats motor neuron disease in SOD(G93A) mice co-expressing the Copper-Chaperone-for-SOD. Williams JR, et al. Neurobiol Dis, 2016 May. PMID 26826269, Free PMC Article
    2. BACE1 cytoplasmic domain interacts with the copper chaperone for superoxide dismutase-1 and binds copper. Angeletti B, et al. J Biol Chem, 2005 May 6. PMID 15722349
    3. Copper modulates the degradation of copper chaperone for Cu,Zn superoxide dismutase by the 26 S proteosome. Bertinato J, et al. J Biol Chem, 2003 Sep 12. PMID 12832419
    4. Copper deficiency induces the upregulation of the copper chaperone for Cu/Zn superoxide dismutase in weanling male rats. Bertinato J, et al. J Nutr, 2003 Jan. PMID 12514262
    5. Molecular cloning and characterization of a copper chaperone for copper/zinc superoxide dismutase from the rat. Hiromura M, et al. Biochem Biophys Res Commun, 2000 Aug 28. PMID 10964676

    See all (12) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. Copper modulates the degradation of this protein, which prioritizes the utilization of copper when it is scarce.
      Title: Copper modulates the degradation of copper chaperone for Cu,Zn superoxide dismutase by the 26 S proteosome.
    2. Associated with copper deficiency were higher levels of Ccs in erythrocytes.
      Title: Cu,Zn-superoxide dismutase is lower and copper chaperone CCS is higher in erythrocytes of copper-deficient rats and mice.
    3. Beta-site amyloid precursor (APP) cleaving enzyme BACE1 expression reduces the activity of superoxide dismutase (SOD1) in cells consistent with direct competition for available CCS, as overexpression of CCS restores SOD1 activity.
      Title: BACE1 cytoplasmic domain interacts with the copper chaperone for superoxide dismutase-1 and binds copper.
    Submit: New GeneRIF Correction

    Pathways from PubChem

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    General gene information

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    Markers

    Homology

    Gene Ontology Provided by RGD

    Function Evidence Code Pubs
    enables copper ion binding IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables copper ion binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables copper ion binding ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables enzyme binding IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    enables superoxide dismutase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables superoxide dismutase copper chaperone activity IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables superoxide dismutase copper chaperone activity TAS
    Traceable Author Statement
    more info
    		 PubMed 
    Process Evidence Code Pubs
    acts_upstream_of_or_within positive regulation of oxidoreductase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in removal of superoxide radicals IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in removal of superoxide radicals IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in superoxide metabolic process IEA
    Inferred from Electronic Annotation
    more info
    		  
    Component Evidence Code Pubs
    located_in cytoplasm IEA
    Inferred from Electronic Annotation
    more info
    		  
    located_in cytoplasm ISO
    Inferred from Sequence Orthology
    more info
    		  
    located_in mitochondrion ISO
    Inferred from Sequence Orthology
    more info
    		 PubMed 
    located_in nucleus IEA
    Inferred from Electronic Annotation
    more info
    		  
    located_in nucleus ISO
    Inferred from Sequence Orthology
    more info
    		  

    General protein information

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    Preferred Names
    copper chaperone for superoxide dismutase
    Names
    superoxide dismutase copper chaperone

    NCBI Reference Sequences (RefSeq)

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    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    mRNA and Protein(s)

    1. NM_053425.1 → NP_445877.1  copper chaperone for superoxide dismutase

      See identical proteins and their annotated locations for NP_445877.1

      Status: PROVISIONAL

      Source sequence(s)
      AF255305
      UniProtKB/Swiss-Prot
      B0BMW1, Q9JK72
      UniProtKB/TrEMBL
      A0A0G2JSZ9, A6HYZ6
      Related
      ENSRNOP00000065189.2, ENSRNOT00000073983.4
      Conserved Domains (3) summary
      PLN02957
      Location:13 → 253
      PLN02957; copper, zinc superoxide dismutase
      cd00305
      Location:89 → 227
      Cu-Zn_Superoxide_Dismutase; Copper/zinc superoxide dismutase (SOD). superoxide dismutases catalyse the conversion of superoxide radicals to molecular oxygen. Three evolutionarily distinct families of SODs are known, of which the copper/zinc-binding family is one. Defects in the ...
      cd00371
      Location:15 → 73
      HMA; Heavy-metal-associated domain (HMA) is a conserved domain of approximately 30 amino acid residues found in a number of proteins that transport or detoxify heavy metals, for example, the CPx-type heavy metal ATPases and copper chaperones. HMA domain ...

    RefSeqs of Annotated Genomes: GCF_036323735.1-RS_2024_02

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference GRCr8

    Genomic

    1. NC_086019.1 Reference GRCr8

      Range
      211543192..211564354 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_039092800.2 → XP_038948728.1  copper chaperone for superoxide dismutase isoform X1

      Conserved Domains (1) summary
      PLN02957
      Location:13 → 148
      PLN02957; copper, zinc superoxide dismutase
    Nucleotide Protein Strain
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q9JK72.1 GenPept UniProtKB/Swiss-Prot:Q9JK72

    Gene LinkOut

    The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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