The x-ray repair cross-complementing (XRCC) proteins are responsible for efficiently repairing and maintaining genetic stability following DNA base damage. These genes share sequence similarity with the yeast DNA repair protein Rad51. XRCC4 is an accessory factor of DNA ligase IV that preferentially binds DNA with nicks or broken ends. XRCC4 binds to DNA ligase IV and enhances its joining activity, and it is also involved in V(D)J recombination. Any defect in one of the known components of the DNA repair/V(D)J recombination machinery (Ku-70, Ku-80, DNA-PKCS, XRCC4 and DNA ligase IV) leads to abortion of the V(D)J rearrangement process and early block in both T and B cell maturation.
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Protein Aliases: complementing defective; DNA repair protein XRCC4; repair in Chinese hamster; X-ray repair; X-ray repair complementing defective repair in Chinese hamster cells 4; X-ray repair cross-complementing protein 4
Gene Aliases: SSMED; XRCC4
UniProt ID: (Human) Q13426
Entrez Gene ID: (Human) 7518