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Science 22 June 1990:
Vol. 248. no. 4962, pp. 1517 - 1523
DOI: 10.1126/science.2360047
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Articles

Science, Vol 248, Issue 4962, 1517-1523
Copyright © 1990 by American Association for the Advancement of Science

articles

RAG-1 and RAG-2, adjacent genes that synergistically activate V(D)J recombination

MA Oettinger, DG Schatz, C Gorka, and D Baltimore

Whitehead Institute for Biomedical Research, Cambridge, MA 02142.

The vast repertoire of immunoglobulins and T cell receptors is generated, in part, by V(D)J recombination, a series of genomic rearrangements that occur specifically in developing lymphocytes. The recombination activating gene, RAG-1, which is a gene expressed exclusively in maturing lymphoid cells, was previously isolated. RAG-1 inefficiently induced V(D)J recombinase activity when transfected into fibroblasts, but cotransfection with an adjacent gene, RAG-2, has resulted in at least a 1000-fold increase in the frequency of recombination. The 2.1-kilobase RAG-2 complementary DNA encodes a putative protein of 527 amino acids whose sequence is unrelated to that of RAG-1. Like RAG-1, RAG-2 is conserved between species that carry out V(D)J recombination, and its expression pattern correlates precisely with that of V(D)J recombinase activity. In addition to being located just 8 kilobases apart, these convergently transcribed genes are unusual in that most, if not all, of their coding and 3' untranslated sequences are contained in single exons. RAG-1 and RAG-2 might activate the expression of the V(D)J recombinase but, more likely, they directly participate in the recombination reaction.


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A. Villa, C. Sobacchi, L. D. Notarangelo, F. Bozzi, M. Abinun, T. G. Abrahamsen, P. D. Arkwright, M. Baniyash, E. G. Brooks, M. E. Conley, et al. (2001)
Blood 97, 81-88
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c-Myb Binds to a Sequence in the Proximal Region of the RAG-2 Promoter and Is Essential for Promoter Activity in T-Lineage Cells.
Q.-F. Wang, J. Lauring, and M. S. Schlissel (2000)
Mol. Cell. Biol. 20, 9203-9211
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Science. ISSN 0036-8075 (print), 1095-9203 (online)