The RAG proteins in V(D)J recombination: more than just a nuclease

Nucleic Acids Res. 2001 Apr 1;29(7):1399-409. doi: 10.1093/nar/29.7.1399.


V(D)J recombination is the process that generates the diversity among T cell receptors and is one of three mechanisms that contribute to the diversity of antibodies in the vertebrate immune system. The mechanism requires precise cutting of the DNA at segment boundaries followed by rejoining of particular pairs of the resulting termini. The imprecision of aspects of the joining reaction contributes significantly to increasing the variability of the resulting functional genes. Signal sequences target DNA recombination and must participate in a highly ordered protein-DNA complex in order to limit recombination to appropriate partners. Two proteins, RAG1 and RAG2, together form the nuclease that cleaves the DNA at the border of the signal sequences. Additional roles of these proteins in organizing the reaction complex for subsequent steps are explored.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • DNA Nucleotidyltransferases / genetics
  • DNA Nucleotidyltransferases / metabolism
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / metabolism*
  • Deoxyribonucleases / genetics
  • Deoxyribonucleases / metabolism
  • Gene Rearrangement*
  • Genes, Immunoglobulin / genetics*
  • Homeodomain Proteins / genetics
  • Homeodomain Proteins / metabolism*
  • Humans
  • Immunoglobulin Joining Region / genetics
  • Immunoglobulin Joining Region / metabolism
  • Immunoglobulin Variable Region / genetics
  • Immunoglobulin Variable Region / metabolism
  • Molecular Sequence Data
  • Nuclear Proteins
  • Sequence Homology, Amino Acid
  • VDJ Recombinases


  • DNA-Binding Proteins
  • Homeodomain Proteins
  • Immunoglobulin Joining Region
  • Immunoglobulin Variable Region
  • Nuclear Proteins
  • RAG2 protein, human
  • V(D)J recombination activating protein 2
  • RAG-1 protein
  • DNA Nucleotidyltransferases
  • VDJ Recombinases
  • Deoxyribonucleases