Censoring of autoreactive B cell development by the pre-B cell receptor

Science. 2008 Aug 1;321(5889):696-9. doi: 10.1126/science.1157533. Epub 2008 Jun 19.

Abstract

Antibody diversity occurs randomly as B cells recombine their immunoglobulin (Ig) heavy- and light-chain genes during development. This process inevitably generates reactivity against self structures, and several mechanisms prevent the development of autoreactive B cells. We report here a role for the pre-B cell receptor, composed of Ig heavy and surrogate light chains, in the negative selection of cells expressing Ig heavy chains with the potential to generate autoantibodies. Surrogate light-chain-deficient (SLC-/-) mice harbored elevated levels of antinuclear antibodies (ANAs) in their serum and showed evidence of escape of pre-B cells expressing prototypic autoantibody heavy chains from negative selection, leading to mature autoantibody secreting CD21-CD23- B cells in the periphery. Thus, the pre-B cell receptor appears to censor the development of certain autoantibody-secreting cells and may represent an important factor in multifactorial autoimmune diseases.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antibodies, Antinuclear / biosynthesis
  • Antibodies, Antinuclear / blood
  • Autoantibodies / biosynthesis
  • Autoantibodies / blood*
  • Autoimmunity*
  • B-Lymphocyte Subsets / immunology
  • B-Lymphocytes / immunology*
  • Complementarity Determining Regions / genetics
  • Complementarity Determining Regions / immunology
  • Immunoglobulin Heavy Chains / genetics
  • Immunoglobulin Heavy Chains / immunology
  • Immunoglobulin Light Chains, Surrogate / genetics
  • Immunoglobulin Light Chains, Surrogate / immunology
  • Lymphopoiesis
  • Mice
  • Pre-B Cell Receptors / immunology*
  • Precursor Cells, B-Lymphoid / immunology*
  • Self Tolerance*
  • Spleen / immunology

Substances

  • Antibodies, Antinuclear
  • Autoantibodies
  • Complementarity Determining Regions
  • Immunoglobulin Heavy Chains
  • Immunoglobulin Light Chains, Surrogate
  • Pre-B Cell Receptors