Lymphoproliferative defects in mice lacking the expression of neurofibromin: functional and biochemical consequences of Nf1 deficiency in T-cell development and function

Blood. 2002 Nov 15;100(10):3656-62. doi: 10.1182/blood-2002-03-0734. Epub 2002 Jul 5.

Abstract

Ras plays an essential role in lymphocyte development and function. However, in vivo consequence(s) of regulation of Ras activity by guanosine triphosphatase (GTPase)-activating proteins (GAPs) on lymphocyte development and function are not known. In this study we demonstrate that neurofibromin, the protein encoded by the NF1 tumor suppressor gene functions as a GAP for Ras in T cells. Loss of Nf1 in T cells results in enhanced Ras activation, which is associated with thymic and splenic hyperplasia, and an increase in the absolute number of immature and mature T-cell subsets compared with control mice. Interestingly, in spite of a profound T-cell expansion and higher thymidine incorporation in unstimulated Nf1-deficient T cells, T-cell receptor and interleukin-2 receptor-mediated proliferation of thymocytes and mature T cells was substantially reduced compared with control mice. Collectively, these results identify neurofibromin as a GAP for Ras in T cells for maintaining immune homeostasis in vivo.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Lymphocyte Activation
  • Lymphoproliferative Disorders / etiology
  • Lymphoproliferative Disorders / immunology
  • Lymphoproliferative Disorders / metabolism*
  • Mice
  • Mice, Mutant Strains
  • Neurofibromin 1 / deficiency
  • Neurofibromin 1 / immunology
  • Neurofibromin 1 / physiology*
  • Spleen / cytology
  • T-Lymphocyte Subsets
  • T-Lymphocytes / cytology
  • T-Lymphocytes / immunology
  • Thymus Gland / cytology
  • ras Proteins / metabolism

Substances

  • Neurofibromin 1
  • ras Proteins