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Protein

DNA repair protein XRCC4

Gene

Xrcc4

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Involved in DNA nonhomologous end joining (NHEJ) required for double-strand break repair and V(D)J recombination. Binds to DNA and to DNA ligase IV (LIG4). The LIG4-XRCC4 complex is responsible for the NHEJ ligation step, and XRCC4 enhances the joining activity of LIG4. Binding of the LIG4-XRCC4 complex to DNA ends is dependent on the assembly of the DNA-dependent protein kinase complex DNA-PK to these DNA ends (By similarity).By similarity1 Publication

GO - Molecular functioni

GO - Biological processi

  • central nervous system development Source: MGI
  • DNA ligation Source: MGI
  • DNA ligation involved in DNA repair Source: MGI
  • double-strand break repair Source: MGI
  • double-strand break repair via nonhomologous end joining Source: BHF-UCL
  • immunoglobulin V(D)J recombination Source: MGI
  • in utero embryonic development Source: MGI
  • isotype switching Source: MGI
  • negative regulation of neuron apoptotic process Source: MGI
  • negative regulation of protection from non-homologous end joining at telomere Source: BHF-UCL
  • positive regulation of fibroblast proliferation Source: MGI
  • positive regulation of ligase activity Source: MGI
  • positive regulation of neurogenesis Source: MGI
  • pro-B cell differentiation Source: MGI
  • response to gamma radiation Source: MGI
  • response to ionizing radiation Source: MGI
  • response to X-ray Source: MGI
  • T cell differentiation in thymus Source: MGI
  • telomeric DNA-containing double minutes formation Source: BHF-UCL
  • V(D)J recombination Source: MGI

Keywordsi

Biological processDNA damage, DNA recombination, DNA repair

Names & Taxonomyi

Protein namesi
Recommended name:
DNA repair protein XRCC4
Alternative name(s):
X-ray repair cross-complementing protein 4
Gene namesi
Name:Xrcc4
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Unplaced

Organism-specific databases

MGIiMGI:1333799. Xrcc4.

Subcellular locationi

GO - Cellular componenti

  • cytosol Source: MGI
  • DNA-dependent protein kinase-DNA ligase 4 complex Source: MGI
  • DNA ligase IV complex Source: MGI
  • ERCC4-ERCC1 complex Source: BHF-UCL
  • nonhomologous end joining complex Source: UniProtKB
  • nuclear chromosome, telomeric region Source: BHF-UCL
  • nucleoplasm Source: MGI
  • nucleus Source: MGI

Keywords - Cellular componenti

Nucleus

Pathology & Biotechi

Disruption phenotypei

Mice show growth defects, premature senescence, IR sensitivity, and inability to support V(D)J recombination. XRCC4 deficiency causes late embryonic lethality accompanied by defective lymphogenesis and defective neurogenesis manifested by extensive apoptotic death of newly generated postmitotic neuronal cells.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000660481 – 326DNA repair protein XRCC4Add BLAST326

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei227PhosphotyrosineBy similarity1
Modified residuei231PhosphothreonineBy similarity1
Modified residuei235PhosphoserineBy similarity1
Modified residuei244PhosphothreonineCombined sources1
Modified residuei250PhosphoserineBy similarity1
Modified residuei254Phosphoserine; by PRKDCBy similarity1
Modified residuei296PhosphoserineBy similarity1
Modified residuei312Phosphoserine; by PRKDCBy similarity1
Modified residuei315PhosphothreonineCombined sources1
Modified residuei319PhosphoserineCombined sources1
Modified residuei320PhosphoserineCombined sources1

Post-translational modificationi

Phosphorylation by CK2 promotes interaction with APTX.By similarity
Monoubiquitinated.By similarity

Keywords - PTMi

Phosphoprotein, Ubl conjugation

Proteomic databases

PaxDbiQ924T3.
PeptideAtlasiQ924T3.
PRIDEiQ924T3.

PTM databases

iPTMnetiQ924T3.
PhosphoSitePlusiQ924T3.

Expressioni

Gene expression databases

BgeeiENSMUSG00000021615.

Interactioni

Subunit structurei

Homodimer and homotetramer in solution. The homodimer associates with LIG4. The LIG4-XRCC4 complex associates in a DNA-dependent manner with the DNA-PK complex composed of PRKDC, XRCC6/Ku70 and XRCC5/Ku86 to form the core non-homologous end joining (NHEJ) complex. Additional components of the NHEJ complex include NHEJ1/XLF and PAXX. Interacts directly with PRKDC but not with the XRCC6/Ku70 and XRCC5/Ku86 dimer. Interacts with APTX and APLF.By similarity

GO - Molecular functioni

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000022115.

Structurei

3D structure databases

ProteinModelPortaliQ924T3.
SMRiQ924T3.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni180 – 212Interaction with LIG4By similarityAdd BLAST33

Coiled coil

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Coiled coili131 – 165Sequence analysisAdd BLAST35
Coiled coili185 – 209Sequence analysisAdd BLAST25

Sequence similaritiesi

Belongs to the XRCC4 family.Curated

Keywords - Domaini

Coiled coil

Phylogenomic databases

eggNOGiENOG410IJSN. Eukaryota.
ENOG410Y7Z3. LUCA.
HOGENOMiHOG000013067.
HOVERGENiHBG059517.
InParanoidiQ924T3.
KOiK10886.
PhylomeDBiQ924T3.
TreeFamiTF101204.

Family and domain databases

InterProiView protein in InterPro
IPR010585. DNA_repair_prot_XRCC4.
IPR009089. XRCC4_N.
PfamiView protein in Pfam
PF06632. XRCC4. 1 hit.
SUPFAMiSSF50809. SSF50809. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q924T3-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MERKVSRIYL ASEPNVPYFL QVSWERAIGS GFVITLTDGH SAWTATVSEL
60 70 80 90 100
EISQEADDMA MEKGKYIDEL RKALVPGSGA AGTYKFLFSK ESQHFSLEKE
110 120 130 140 150
LKDVSFRLGS FNLDKVSNSA EVIRELICYC LDTITEKQAK NEHLQKENER
160 170 180 190 200
LLRDWNDVQG RFEKCVSAKE ALEADLYQRF ILVLNEKKTK IRSLHKLLNE
210 220 230 240 250
VQQLEESTKP ERENPCSDKT PEEHGLYDGS TDEESGAPVQ AAETLHKDDS
260 270 280 290 300
IFSSPDVTDI APSRKRRHRM QKNLGTEPKM APQELPLQEK ERLASSLPQT
310 320
LKEESTSAEN MSLETLRNSS PEDLFD
Length:326
Mass (Da):37,061
Last modified:December 1, 2001 - v1
Checksum:i58BD3422F7D2CBDB
GO
Isoform 2 (identifier: Q924T3-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     293-326: LASSLPQTLKEESTSAENMSLETLRNSSPEDLFD → KKKYPSIMSTKVQRSLGEGGHG

Note: No experimental confirmation available.
Show »
Length:314
Mass (Da):35,708
Checksum:i58C2D697E88C17C3
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti27A → T in BAB26604 (PubMed:15489334).Curated1
Sequence conflicti27A → T in BAC35447 (PubMed:15489334).Curated1
Sequence conflicti54Q → P in BAC40256 (PubMed:15489334).Curated1
Sequence conflicti93Q → R in BAB26604 (PubMed:15489334).Curated1
Sequence conflicti93Q → R in BAC35447 (PubMed:15489334).Curated1
Sequence conflicti125E → D in BAB26604 (PubMed:15489334).Curated1
Sequence conflicti125E → D in BAC35447 (PubMed:15489334).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_009475293 – 326LASSL…EDLFD → KKKYPSIMSTKVQRSLGEGG HG in isoform 2. 1 PublicationAdd BLAST34

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB055154 mRNA. Translation: BAB62316.1.
AK009951 mRNA. Translation: BAB26604.1.
AK053612 mRNA. Translation: BAC35447.1.
AK088281 mRNA. Translation: BAC40256.1.
BC025538 mRNA. Translation: AAH25538.1.
CCDSiCCDS26673.1. [Q924T3-1]
RefSeqiNP_082288.1. NM_028012.4.
XP_006517104.1. XM_006517041.3.
XP_006517105.1. XM_006517042.2.
XP_006517106.1. XM_006517043.3.
UniGeneiMm.37531.

Genome annotation databases

GeneIDi108138.
KEGGimmu:108138.
UCSCiuc007rjn.2. mouse. [Q924T3-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.

Entry informationi

Entry nameiXRCC4_MOUSE
AccessioniPrimary (citable) accession number: Q924T3
Secondary accession number(s): Q8BKC9, Q8BU02, Q9D6U6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 1, 2004
Last sequence update: December 1, 2001
Last modified: May 10, 2017
This is version 125 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

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families