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Protein

Copper chaperone for superoxide dismutase

Gene

Ccs

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Delivers copper to copper zinc superoxide dismutase (SOD1).

Cofactori

Protein has several cofactor binding sites:
  • Cu2+By similarityNote: Binds 2 copper ions per subunit.By similarity
  • Zn2+By similarityNote: Binds 1 zinc ion per subunit.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi22Copper 1PROSITE-ProRule annotation1
Metal bindingi25Copper 1PROSITE-ProRule annotation1
Metal bindingi147ZincPROSITE-ProRule annotation1
Metal bindingi155ZincPROSITE-ProRule annotation1
Metal bindingi164ZincPROSITE-ProRule annotation1
Metal bindingi167ZincPROSITE-ProRule annotation1
Metal bindingi244Copper 2PROSITE-ProRule annotation1
Metal bindingi246Copper 2PROSITE-ProRule annotation1

GO - Molecular functioni

GO - Biological processi

  • metal ion transport Source: InterPro
  • positive regulation of oxidoreductase activity Source: MGI

Keywordsi

Molecular functionChaperone
LigandCopper, Metal-binding, Zinc

Enzyme and pathway databases

ReactomeiR-MMU-3299685 Detoxification of Reactive Oxygen Species

Names & Taxonomyi

Protein namesi
Recommended name:
Copper chaperone for superoxide dismutase
Alternative name(s):
Superoxide dismutase copper chaperone
Gene namesi
Name:Ccs
Synonyms:Ccsd
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 19

Organism-specific databases

MGIiMGI:1333783 Ccs

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

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002135441 – 274Copper chaperone for superoxide dismutaseAdd BLAST274

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Cross-linki76Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)By similarity
Disulfide bondi141 ↔ 227By similarity
Cross-linki189Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)By similarity
Cross-linki216Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)By similarity
Cross-linki241Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)By similarity
Modified residuei267PhosphoserineCombined sources1

Post-translational modificationi

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 (By similarity).By similarity

Keywords - PTMi

Disulfide bond, Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiQ9WU84
MaxQBiQ9WU84
PaxDbiQ9WU84
PRIDEiQ9WU84

PTM databases

iPTMnetiQ9WU84
PhosphoSitePlusiQ9WU84
SwissPalmiQ9WU84

Expressioni

Tissue specificityi

Ubiquitous.

Gene expression databases

BgeeiENSMUSG00000034108 Expressed in 265 organ(s), highest expression level in membranous labyrinth
CleanExiMM_CCS
ExpressionAtlasiQ9WU84 baseline and differential
GenevisibleiQ9WU84 MM

Interactioni

Subunit structurei

Homodimer, and heterodimer with SOD1. Interacts with COMMD1 (By similarity). Interacts with XIAP/BIRC4 (By similarity).By similarity

GO - Molecular functioni

Protein-protein interaction databases

BioGridi198563, 2 interactors
DIPiDIP-48692N
IntActiQ9WU84, 2 interactors
STRINGi10090.ENSMUSP00000035486

Structurei

3D structure databases

ProteinModelPortaliQ9WU84
SMRiQ9WU84
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini12 – 75HMAPROSITE-ProRule annotationAdd BLAST64

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni88 – 234Superoxide dismutase-likeAdd BLAST147

Sequence similaritiesi

In the C-terminal section; belongs to the Cu-Zn superoxide dismutase family.Curated

Phylogenomic databases

eggNOGiENOG410IPMW Eukaryota
COG2032 LUCA
GeneTreeiENSGT00530000063226
HOGENOMiHOG000263450
HOVERGENiHBG018933
InParanoidiQ9WU84
KOiK04569
OMAiGVTLWEE
OrthoDBiEOG091G0FQ1
PhylomeDBiQ9WU84
TreeFamiTF105184

Family and domain databases

CDDicd00305 Cu-Zn_Superoxide_Dismutase, 1 hit
cd00371 HMA, 1 hit
Gene3Di2.60.40.200, 1 hit
InterProiView protein in InterPro
IPR006121 HMA_dom
IPR036163 HMA_dom_sf
IPR036423 SOD-like_Cu/Zn_dom_sf
IPR024134 SOD_Cu/Zn_/chaperone
IPR018152 SOD_Cu/Zn_BS
IPR001424 SOD_Cu_Zn_dom
PANTHERiPTHR10003 PTHR10003, 1 hit
PfamiView protein in Pfam
PF00403 HMA, 1 hit
PF00080 Sod_Cu, 1 hit
PRINTSiPR00068 CUZNDISMTASE
SUPFAMiSSF49329 SSF49329, 1 hit
SSF55008 SSF55008, 1 hit
PROSITEiView protein in PROSITE
PS50846 HMA_2, 1 hit
PS00332 SOD_CU_ZN_2, 1 hit

Sequencei

Sequence statusi: Complete.

Q9WU84-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MASKSGDGGT VCALEFAVQM SCQSCVDAVH KTLKGVAGVQ NVDVQLENQM
60 70 80 90 100
VLVQTTLPSQ EVQALLESTG RQAVLKGMGS SQLQNLGAAV AILEGCGSIQ
110 120 130 140 150
GVVRFLQLSS ELCLIEGTID GLEPGLHGLH VHQYGDLTRD CNSCGDHFNP
160 170 180 190 200
DGASHGGPQD TDRHRGDLGN VRAEAGGRAT FRIEDKQLKV WDVIGRSLVI
210 220 230 240 250
DEGEDDLGRG GHPLSKITGN SGKRLACGII ARSAGLFQNP KQICSCDGLT
260 270
IWEERGRPIA GQGRKDSAQP PAHL
Length:274
Mass (Da):28,912
Last modified:November 1, 1999 - v1
Checksum:i19DCE48376C9D5A2
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF121906 mRNA Translation: AAD23832.1
AF173379 mRNA Translation: AAF70242.1
AK010264 mRNA Translation: BAB26806.1
BC026938 mRNA Translation: AAH26938.1
CCDSiCCDS29438.1
RefSeqiNP_058588.1, NM_016892.3
UniGeneiMm.434411

Genome annotation databases

EnsembliENSMUST00000037246; ENSMUSP00000035486; ENSMUSG00000034108
GeneIDi12460
KEGGimmu:12460
UCSCiuc008gba.1 mouse

Similar proteinsi

Entry informationi

Entry nameiCCS_MOUSE
AccessioniPrimary (citable) accession number: Q9WU84
Secondary accession number(s): Q9CRJ9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 31, 2002
Last sequence update: November 1, 1999
Last modified: September 12, 2018
This is version 153 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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