Multiple checkpoint breach of B cell tolerance in Rasgrp1-deficient mice

J Immunol. 2013 Oct 1;191(7):3605-13. doi: 10.4049/jimmunol.1202892. Epub 2013 Aug 30.

Abstract

Lymphopenic hosts offer propitious microenvironments for expansion of autoreactive B and T cells. Despite this, many lymphopenic hosts do not develop autoimmune disease, suggesting that additional factors are required for breaching self-tolerance in the setting of lymphopenia. Mice deficient in guanine nucleotide exchange factor Rasgrp1 develop a lymphoproliferative disorder with features of human systemic lupus erythematosus. Early in life, Rasgrp1-deficient mice have normal B cell numbers but are T lymphopenic, leading to defective homeostatic expansion of CD4 T cells. To investigate whether B cell-intrinsic mechanisms also contribute to autoimmunity, Rasgrp1-deficient mice were bred to mice containing a knockin autoreactive BCR transgene (564Igi), thereby allowing the fate of autoreactive B cells to be assessed. During B cell development, the frequency of receptor-edited 564Igi B cells was reduced in Rasrp1-deficient mice compared with Rasgrp1-sufficient littermate control mice, suggesting that tolerance was impaired. In addition, the number of 564Igi transitional B cells was increased in Rasgrp1-deficient mice compared with control mice. Immature 564Igi B cells in bone marrow and spleen lacking RasGRP1 expressed lower levels of Bim mRNA and protein, suggesting that autoreactive B cells elude clonal deletion during development. Concomitant with increased serum autoantibodies, Rasgrp1-deficient mice developed spontaneous germinal centers at 8-10 wk of age. The frequency and number of 564Igi B cells within these germinal centers were significantly increased in Rasgrp1-deficient mice relative to control mice. Taken together, these studies suggest that autoreactive B cells lacking Rasgrp1 break central and peripheral tolerance through both T cell-independent and -dependent mechanisms.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Apoptosis Regulatory Proteins / metabolism
  • Autoantibodies / blood
  • Autoantibodies / immunology
  • Autoimmunity / genetics
  • Autoimmunity / immunology
  • B-Lymphocytes / immunology*
  • B-Lymphocytes / metabolism*
  • Bcl-2-Like Protein 11
  • Bone Marrow / immunology
  • Bone Marrow / metabolism
  • Germinal Center / immunology
  • Germinal Center / metabolism
  • Guanine Nucleotide Exchange Factors / deficiency
  • Guanine Nucleotide Exchange Factors / genetics*
  • Immune Tolerance / genetics*
  • Membrane Proteins / metabolism
  • Mice
  • Mice, Knockout
  • Proto-Oncogene Proteins / metabolism
  • Receptors, Antigen, B-Cell / metabolism
  • Spleen / immunology
  • Spleen / metabolism
  • Toll-Like Receptors / metabolism

Substances

  • Apoptosis Regulatory Proteins
  • Autoantibodies
  • BCL2L11 protein, human
  • Bcl-2-Like Protein 11
  • Bcl2l11 protein, mouse
  • Guanine Nucleotide Exchange Factors
  • Membrane Proteins
  • Proto-Oncogene Proteins
  • Rasgrp1 protein, mouse
  • Receptors, Antigen, B-Cell
  • Toll-Like Receptors