RAGs and regulation of autoantibodies.
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.
Jankovic
Mila
M
Laboratory of Molecular Immunology, The Rockefeller University, New York, New York 10021, USA. jankovm@mail.rockefeller.edu
Casellas
Rafael
R
Yannoutsos
Nikos
N
Wardemann
Hedda
H
Nussenzweig
Michel C
MC
D000818:Animals; D000917:Antibody Formation / Q000235:genetics*; D001323:Autoantibodies / Q000235:genetics*; D001402:B-Lymphocytes / Q000276:immunology; D004268:DNA-Binding Proteins / Q000235:genetics / Q000276:immunology*; D015322:Gene Rearrangement, B-Lymphocyte; D005803:Genes, Immunoglobulin; D018398:Homeodomain Proteins / Q000235:genetics / Q000276:immunology*; D006801:Humans; D007135:Immunoglobulin Variable Region / Q000235:genetics; D009687:Nuclear Proteins
D001323:Autoantibodies; D004268:DNA-Binding Proteins; D018398:Homeodomain Proteins; D007135:Immunoglobulin Variable Region; D009687:Nuclear Proteins; C499311:RAG2 protein, human; C064655:V(D)J recombination activating protein 2; C064658:RAG-1 protein