Recombination Activating Gene 1 (RAG1)

[Edit]

RNF74; RING Finger Protein 74; V(D)J Recombination-Activating Protein 1; Endonuclease RAG1; E3 ubiquitin-protein ligase RAG1

Recombination Activating Gene 1 (RAG1)
Nucleotide sequencing of human and mouse RAG1 cDNA clones encode predicted 119-kD proteins of 1,043 and 1,040 amino acids, respectively, with 90% sequence identity. The RAG1 gene has been conserved between species that carry out the V(D)J recombination, and its pattern of expression correlates exactly with the pattern of expression of V(D)J recombinase activity. Oettinger et al. (1990) gave a corrected length of 6.6 kb for the RAG1 cDNA.
RAG1 is critical for joining in vivo and strongly suggesting that the postcleavage complex is important in end joining. The E423Q mutant was severely defective for both hairpin opening in vitro and coding joint formation in vivo. These data suggested that the hairpin opening activity of the RAG proteins plays an important physiologic role in V(D)J recombination.

Organism species: Homo sapiens (Human)

CATALOG NO.PRODUCT NAMEAPPLICATIONS
Proteinsn/aRecombinant Recombination Activating Gene 1 (RAG1)Recombinant Protein Customized Service Offer
Antibodiesn/aMonoclonal Antibody to Recombination Activating Gene 1 (RAG1)Monoclonal Antibody Customized Service Offer
n/aPolyclonal Antibody to Recombination Activating Gene 1 (RAG1)Polyclonal Antibody Customized Service Offer
Assay Kitsn/aCLIA Kit for Recombination Activating Gene 1 (RAG1)CLIA Kit Customized Service Offer
n/aELISA Kit for Recombination Activating Gene 1 (RAG1)ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO.PRODUCT NAMEAPPLICATIONS
Proteinsn/aRecombinant Recombination Activating Gene 1 (RAG1)Recombinant Protein Customized Service Offer
Antibodiesn/aMonoclonal Antibody to Recombination Activating Gene 1 (RAG1)Monoclonal Antibody Customized Service Offer
n/aPolyclonal Antibody to Recombination Activating Gene 1 (RAG1)Polyclonal Antibody Customized Service Offer
Assay Kitsn/aCLIA Kit for Recombination Activating Gene 1 (RAG1)CLIA Kit Customized Service Offer
n/aELISA Kit for Recombination Activating Gene 1 (RAG1)ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO.PRODUCT NAMEAPPLICATIONS
Proteinsn/aRecombinant Recombination Activating Gene 1 (RAG1)Recombinant Protein Customized Service Offer
Antibodiesn/aMonoclonal Antibody to Recombination Activating Gene 1 (RAG1)Monoclonal Antibody Customized Service Offer
n/aPolyclonal Antibody to Recombination Activating Gene 1 (RAG1)Polyclonal Antibody Customized Service Offer
Assay Kitsn/aCLIA Kit for Recombination Activating Gene 1 (RAG1)CLIA Kit Customized Service Offer
n/aELISA Kit for Recombination Activating Gene 1 (RAG1)ELISA Kit Customized Service Offer
  1. "The V(D)J recombination activating gene, RAG-1."Cell 59:1035-1048(1989) [PubMed] [Europe PMC] [Abstract]
  2. "Human chromosome 11 DNA sequence and analysis including novel gene identification." Nature 440:497-500(2006) [PubMed] [Europe PMC] [Abstract]
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  4. "RAG-1 interacts with the repeated amino acid motif of the human homologue of the yeast protein SRP1."Proc. Natl. Acad. Sci. U.S.A. 91:7633-7637(1994) [PubMed] [Europe PMC] [Abstract]
  5. "Two new variants of RAG-1 protein predicted by SSCP."Hum. Mutat. 8:191-192(1996) [PubMed] [Europe PMC] [Abstract]
  6. "RAG mutations in human B cell-negative SCID."Science 274:97-99(1996) [PubMed] [Europe PMC] [Abstract]
  7. "Partial V(D)J recombination activity leads to Omenn syndrome."Cell 93:885-896(1998) [PubMed] [Europe PMC] [Abstract]
  8. "Characterization of immune function and analysis of RAG gene mutations in Omenn syndrome and related disorders."Clin. Exp. Immunol. 119:148-155(2000) [PubMed] [Europe PMC] [Abstract]
  9. "V(D)J recombination defects in lymphocytes due to RAG mutations: severe immunodeficiency with a spectrum of clinical presentations." Blood 97:81-88(2001) [PubMed] [Europe PMC] [Abstract]
  10. "A novel immunodeficiency associated with hypomorphic RAG1 mutations and CMV infection."J. Clin. Invest. 115:3291-3299(2005) [PubMed] [Europe PMC] [Abstract]
  11. "An immunodeficiency disease with RAG mutations and granulomas."N. Engl. J. Med. 358:2030-2038(2008) [PubMed] [Europe PMC] [Abstract]
  12. "Molecular analysis of T-B-NK+ severe combined immunodeficiency and Omenn syndrome cases in Saudi Arabia."BMC Med. Genet. 10:116-116(2009) [PubMed] [Europe PMC] [Abstract]
  13. "Highly variable clinical phenotypes of hypomorphic RAG1 mutations."Pediatrics 126:E1248-E1252(2010) [PubMed] [Europe PMC] [Abstract]
  14. "Novel mutations in RAG1/2 and ADA genes in Israeli patients presenting with T-B-SCID or Omenn syndrome."Clin. Immunol. 140:284-290(2011) [PubMed] [Europe PMC] [Abstract]
  15. "Clinical characteristics and molecular analysis of three Chinese children with Omenn syndrome."Pediatr. Allergy Immunol. 22:482-487(2011) [PubMed] [Europe PMC] [Abstract]