Recombinase activating gene enzymes of lymphocytes

Curr Opin Hematol. 2001 Jan;8(1):41-6. doi: 10.1097/00062752-200101000-00008.


Expression of T-cell receptor and surface immunoglobulins on T and B lymphocytes, respectively, is strictly dependent on the variable, (diversity) joining exon (V(D)J) recombination process, which is initiated by the lymphoid-specific recombinase activating gene proteins 1 and 2 (RAG1 and RAG2). Recent advances have highlighted the functional organization of the RAG1 and RAG2 proteins and have provided important information on the regulation of RAG gene expression. Depending on the severity of their effects on the V(D)J recombination process, mutations of the RAG genes account for a spectrum of combined immune deficiencies in humans.

Publication types

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

MeSH terms

  • DNA Nucleotidyltransferases / physiology*
  • DNA-Binding Proteins / physiology*
  • Gene Expression Regulation / immunology*
  • Genes, RAG-1 / physiology*
  • Humans
  • Integrases*
  • Lymphocytes / physiology*
  • Nuclear Proteins
  • Receptors, Antigen, T-Cell / physiology*
  • Recombinases
  • Recombination, Genetic


  • DNA-Binding Proteins
  • Nuclear Proteins
  • RAG2 protein, human
  • Receptors, Antigen, T-Cell
  • Recombinases
  • V(D)J recombination activating protein 2
  • DNA Nucleotidyltransferases
  • Integrases
  • integron integrase IntI1