IMGT Lexique

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Recombination Activating Gene (RAG)

RAG1 (Recombination activating gene 1) and RAG2 (Recombination activating gene 2) are the V(D)J recombination activating genes [1]. RAG1 and RAG2 synergistically activate V(D)J recombination [2]. RAG1 and RAG2 are adjacent genes.

The RAG1 coding region is contained within a single exon, excepted in the rainbow trout [11] and the zebrafish [17] genes in which the RAG1 genes has one and two introns, respectively.

RAG1 deficient mice [5] and RAG2 deficient mice [6] have no mature B and T lymphocytes.

The RAG1 protein possesses:

a conserved homeodomain which binds the nonamer [13]
BIIa, a basic motif in the N-terminal region of RAG1 which enhances V(D)J recombination activity [15]

aa (xxx-xxx) is essential for V(D)J recombination [9,10].

Low levels of RAG1 transcripts were detected in the murine central nervous system CNS, whereas RAG2 is not clearly expressed [3].

Human [1] M29474
mouse [1] M29475
chicken [4] M58530
rabbit [7] M77666
Xenopus leavis [8] L19324
rainbow trout (Onchorhynchus mykiss) [11] U15663
opposum (Monodelphis domestica) [12] U51897
shark [12] U62645
zebrafish [17] U71093
Recombination-related genes are necessary for normal neuronal development [18,19]
Pleurodeles waltl (Urodele amphibian) [20] AJ010258

References:
[1]	Schatz, D.G. et al. Cell 59,1035-1048 (1989)
[2]	Oettinger, M. A. et al. Science 248, 1517-1522 (1990)
[3]	Chun, J. J. et al. Cell 64, 189-200 (1991)
[4]	Carlson, L. M. et al. Cell 64,201-208 (1991)
[5]	Mombaerts, P. et al. Cell 68, 869-877 (1992)
6]	Shinkai, Y. et al. Cell 68, 855-867 (1992)
[7]	Fuschiotti, P. et al. Mol. Immunol. 30, 1021-1032 (1993)
[8]	Greenhalgh, P. et al. J. Immunol. 151, 3100-3110 (1993)
[9]	Sadofsky, M. J. et al. Nucleic.Acids Res. 21, 5644-5650 (1993)
[10]	Silver, D. P. et al. Pro. Natl. Acad. Sci. USA 90, 6100-6104 (1993)
[11]	Hansen, J. D. and Kaattari, S. L. Immunogenetics 42, 188-195 (1995)
[12]	Bernstein, R. M. et al. Proc. Nalt. Acad. Sci. USA 93, 9454-9459 (1996)
[13]	Difilippantonio, M. J. et al. Cell 87, 253-262 (1996)
[14]	Spanopoulo, E. et al. Cell 87, 263-276 (1996)
[15]	Mc Mahan, C. J. et al. Mol. Cell. Biol. 17, 4544-4552 (1997)
[16]	Miller, R. D. And Rosenberg, G. H. Immunogenetics 45, 341-342 (1997)
[17]	Willett, C. E. et al. Immunogenetics 45, 394-404 (1997)
[18]	Barnes, D. E. et al. Curr. Biol. 8, 1395-1398 (1998)
[19]	Gao, Y. et al. Cell 95, 891-902 (1998)
[20]	Frippiat, C. et al. Immunogenetics 52, 264-275 (2001)