DNA Cross Link Repair 1C Protein (DCLRE1C)

A-SCID; DCLREC1C; RS-SCID; SCIDA; ARTEMIS; SNM1C; SNM1C; Severe Combined Immunodeficiency,Type A(Athabascan)

DNA Cross Link Repair 1C Protein (DCLRE1C)
DCLRE1C encodes a nuclear protein that is involved in V(D)J recombination and DNA repair. The protein has single-strand-specific 5'-3' exonuclease activity; it also exhibits endonuclease activity on 5' and 3' overhangs and hairpins when complexed with protein kinase, DNA-activated, catalytic polypeptide.The purified Artemis protein alone possesses single-strand-specific 5-prime-to-3-prime exonuclease activity. Upon complex formation, PRKDC phosphorylates Artemis, and Artemis acquires endonucleolytic activity on 5-prime and 3-prime overhangs, as well as hairpins. PRKDC regulates Artemis by both phosphorylation and complex formation to permit enzymatic activities that are critical for the hairpin-opening step of V(D)J recombination and for the 5-prime and 3-prime overhang processing in nonhomologous DNA end joining.

Organism species: Homo sapiens (Human)

Organism species: Mus musculus (Mouse)

Organism species: Rattus norvegicus (Rat)