Regulation of the B cell receptor repertoire and self-reactivity by BAFF

J Immunol. 2010 Oct 1;185(7):4128-36. doi: 10.4049/jimmunol.1002176. Epub 2010 Sep 3.

Abstract

The TNF-family cytokine BAFF (BLyS) promotes B lymphocyte survival and is overexpressed in individuals with systemic lupus erythematosus and Sjögren's Syndrome. BAFF can rescue anergic autoreactive B cells from death, but only when competition from nonautoreactive B cells is lacking. Yet, high BAFF levels promote autoantibody formation in individuals possessing diverse B cells. To better understand how excess BAFF promotes autoimmunity in a polyclonal immune system, Ig L chain usage was analyzed in 3H9 site-directed IgH chain transgenic mice, whose B cells recognize DNA and chromatin when they express certain endogenous L chains. BAFF levels were manipulated in 3H9 mice by introducing transgenes expressing either BAFF or its natural inhibitor ΔBAFF. B cells in BAFF/3H9 mice were elevated in number, used a broad L chain repertoire, including L chains generating high-affinity autoreactivity, and produced abundant autoantibodies. Comparison of spleen and lymph node B cells suggested that highly autoreactive B cells were expanded. By contrast, ΔBAFF/3H9 mice had reduced B cell numbers with a repertoire similar to that of 3H9 mice, but lacking usage of a subset of Vκ genes. The results show that limiting BAFF signaling only slightly selects against higher affinity autoreactive B cells, whereas its overexpression leads to broad tolerance escape and positive selection of autoreactive cells. The results have positive implications for the clinical use of BAFF-depleting therapy.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Autoantibodies / immunology
  • Autoimmunity / immunology*
  • B-Cell Activating Factor / immunology*
  • B-Lymphocytes / cytology
  • B-Lymphocytes / immunology*
  • B-Lymphocytes / metabolism
  • Cell Separation
  • Enzyme-Linked Immunosorbent Assay
  • Flow Cytometry
  • Mice
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Polymerase Chain Reaction
  • Receptors, Antigen, B-Cell / immunology*
  • Receptors, Antigen, B-Cell / metabolism
  • Self Tolerance / immunology*

Substances

  • Autoantibodies
  • B-Cell Activating Factor
  • Receptors, Antigen, B-Cell
  • Tnfsf13b protein, mouse