Editing disease-associated autoantibodies

Immunity. 1997 Jan;6(1):97-105. doi: 10.1016/s1074-7613(00)80673-1.

Abstract

We have generated site-directed transgenic mice whose transgenes code for anti-DNA antibodies. These antibodies are representative of the lupus-associated anti-DNAs seen in mouse models of autoimmunity and human SLE, and have the usual characteristics of pathogenic autoantibodies. As conventional transgenics in nonautoimmune mice, anti-DNA B cells have been shown to be deleted or inactivated. Autoreactive B cells can also escape negative regulation by a process called receptor editing. Here we describe two combined immunoglobulin H and L chain site-directed transgenic mouse models and characterize their editing phenotypes. One model, 3H9R/Vkappa4R, has a deletion-prone phenotype and undergoes editing, primarily by inactivation of the light chain by leap-frogging events. In the other model, 3H9R/Vkappa8R, B cells are susceptible to anergy and maintain most of their HR and LR chains. These studies clarify the relationship between editing and other mechanisms of tolerance.

MeSH terms

  • Alleles
  • Animals
  • Antibodies, Antinuclear / genetics*
  • Antibody Affinity*
  • Autoimmune Diseases / immunology*
  • Gene Rearrangement, B-Lymphocyte*
  • Genes, Immunoglobulin*
  • Hybridomas
  • Immune Tolerance
  • Immunoglobulin Heavy Chains / genetics
  • Immunoglobulin Variable Region / genetics*
  • Immunoglobulin kappa-Chains / genetics
  • Mice
  • Mice, Transgenic
  • Sequence Deletion

Substances

  • Antibodies, Antinuclear
  • Immunoglobulin Heavy Chains
  • Immunoglobulin Variable Region
  • Immunoglobulin kappa-Chains