RAGs and regulation of autoantibodies

Annu Rev Immunol. 2004:22:485-501. doi: 10.1146/annurev.immunol.22.012703.104707.

Abstract

Autoreactive antibodies are etiologic agents in a number of autoimmune diseases. Like all other antibodies these antibodies are produced in developing B cells by V(D)J recombination in the bone marrow. Three mechanisms regulate autoreactive B cells: deletion, receptor editing, and anergy. Here we review the prevalence of autoantibodies in the initial antibody repertoire, their regulation by receptor editing, and the role of the recombinase proteins (RAG1 and RAG2) in this process.

Publication types

  • Review

MeSH terms

  • Animals
  • Antibody Formation / genetics*
  • Autoantibodies / genetics*
  • B-Lymphocytes / immunology
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / immunology*
  • Gene Rearrangement, B-Lymphocyte*
  • Genes, Immunoglobulin
  • Homeodomain Proteins / genetics
  • Homeodomain Proteins / immunology*
  • Humans
  • Immunoglobulin Variable Region / genetics
  • Nuclear Proteins

Substances

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