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DNA repair protein XRCC4; X-ray repair cross-complementing protein 4 (XRCC4)

Function: - role in DNA non-homologous end joining (NHEJ) required for double-strand break repair & V(D)J recombination - binds to DNA & to DNA ligase IV (LIG4) - the LIG4-XRCC4 complex is responsible for the NHEJ ligation step, & 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 - phosphorylated by PRKDC; phosphorylation seems not to be necessary for binding to DNA - phosphorylation by CK2 promotes interaction with APTX - monoubiquitinated - sumoylation at Lys-210 is required for nuclear localization & recombination efficiency; has no effect on ubiquitination - homodimer & homotetramer in solution - homodimer associates with LIG4, & the LIG4-XRCC4 complex associates in a DNA-dependent manner with the DNA-PK complex formed by the Ku p70/p86 dimer (G22P1/G22P2) & PRKDC - seems to interact directly with PRKDC but not with the Ku p70/86 dimer - interacts with XLF/cernunnos - interacts with APTX & APLF Structure: belongs to the XRCC4 family Compartment: nucleus Alternative splicing: named isoforms=3 Expression: widely expressed

Related

XRCC4 gene (X-ray repair, complementing defective repair in Chinese hamster cells-4)

General

nuclear protein phosphoprotein

Properties

SIZE: entity length = 336 aa MW = 38 kD COMPARTMENT: cell nucleus MOTIF: coiled coil {131-165} LIG4 interaction {180-213} MOTIF: coiled coil {184-212} Ser phosphorylation site {S232} Thr phosphorylation site {T233} Ser phosphorylation site {S260} Ser phosphorylation site {S320} Ser phosphorylation site {S327} Ser phosphorylation site {S328}

Database Correlations

OMIM 194363 UniProt Q13426 Pfam PF06632 Entrez Gene 7518 Kegg hsa:7518

References

  1. UniProt :accession Q13426
  2. NIEHS-SNPs http://egp.gs.washington.edu/data/xrcc4/