Defective inhibition of B-cell proliferation by Wiskott-Aldrich syndrome protein-deficient regulatory T cells

Blood. 2011 Jun 16;117(24):6608-11. doi: 10.1182/blood-2010-12-322834. Epub 2011 Apr 22.

Abstract

Wiskott-Aldrich syndrome (WAS) is an inherited immunodeficiency characterized by high incidence of autoantibody-mediated autoimmune complications. Such a feature has been associated with defective suppressor activity of WAS protein-deficient, naturally occurring CD4(+)CD25(+)Foxp3(+) regulatory T cells on responder T cells. However, it remains to be established whether the altered B-cell tolerance reported in WAS patients and Was knockout (WKO) mice is secondary to abnormalities in the direct suppression of B-cell function by nTreg cells or to impaired regulation of T-helper function. Because activated nTreg cells are known to induce granzyme B-mediated B-cell killing, we decided to evaluate the regulatory capabilities of WKO nTregs on B lymphocytes. We found that preactivated WKO nTreg cells failed to effectively suppress B-cell proliferation and that such a defect was associated with reduced killing of B cells and significantly decreased degranulation of granzyme B. Altogether, these results provide additional mechanistic insights into the loss of immune tolerance in WAS.

MeSH terms

  • Animals
  • B-Lymphocytes / metabolism
  • B-Lymphocytes / physiology*
  • Cell Death / genetics
  • Cell Death / immunology
  • Cell Degranulation / genetics
  • Cell Degranulation / immunology
  • Cell Proliferation*
  • Cells, Cultured
  • Down-Regulation / genetics
  • Down-Regulation / immunology
  • Granzymes / metabolism
  • Immune Tolerance / genetics
  • Immune Tolerance / immunology
  • Lymphocyte Activation / genetics
  • Lymphocyte Activation / immunology
  • Mice
  • Mice, Knockout
  • T-Lymphocytes, Regulatory / metabolism
  • T-Lymphocytes, Regulatory / physiology*
  • Wiskott-Aldrich Syndrome Protein / deficiency
  • Wiskott-Aldrich Syndrome Protein / genetics*
  • Wiskott-Aldrich Syndrome Protein / metabolism

Substances

  • Wiskott-Aldrich Syndrome Protein
  • Granzymes