title: RAGs and regulation of autoantibodies.
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.
authors.affiliation: Laboratory of Molecular Immunology, The Rockefeller University, New York, New York 10021, USA. jankovm@mail.rockefeller.edu
authors.forename: Mila
authors.initials: M
authors.lastname: Jankovic
authors.forename: Rafael
authors.initials: R
authors.lastname: Casellas
authors.forename: Nikos
authors.initials: N
authors.lastname: Yannoutsos
authors.forename: Hedda
authors.initials: H
authors.lastname: Wardemann
authors.forename: Michel C
authors.initials: MC
authors.lastname: Nussenzweig
mesh_terms: 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
chemical_list: 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