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Wiskott-Aldrich syndrome protein-deficient mice reveal a role for WASP in T but not B cell activation.

The Wiskott-Aldrich syndrome (WAS) is a human X-linked immunodeficiency resulting from mutations in a gene (WASP) encoding a cytoplasmic protein implicated in regulating the actin cytoskeleton. To elucidate WASP function, we disrupted the WASP gene in mice by gene-targeted mutation. WASP-deficient mice showed apparently normal lymphocyte development, normal serum immunoglobulin levels, and the capacity to respond to both T-dependent and T-independent type II antigens. However, these mice did have decreased peripheral blood lymphocyte and platelet numbers and developed chronic colitis. Moreover, purified WASP-deficient T cells showed markedly impaired proliferation and antigen receptor cap formation in response to anti-CD3epsilon stimulation. Yet, purified WASP-deficient B cells showed normal responses to anti-Ig stimulation. We discuss the implications of our findings regarding WASP function in receptor signaling and cytoskeletal reorganization in T and B cells and compare the effects of WASP deficiency in mice and humans.

Pubmed ID: 9697838


  • Snapper SB
  • Rosen FS
  • Mizoguchi E
  • Cohen P
  • Khan W
  • Liu CH
  • Hagemann TL
  • Kwan SP
  • Ferrini R
  • Davidson L
  • Bhan AK
  • Alt FW



Publication Data

July 1, 1998

Associated Grants

  • Agency: NIAID NIH HHS, Id: AI20047
  • Agency: NHLBI NIH HHS, Id: HL03749
  • Agency: NHLBI NIH HHS, Id: HL59561

Mesh Terms

  • Animals
  • Antigens, CD28
  • B-Lymphocytes
  • Cell Division
  • Colitis
  • Humans
  • Immunoglobulin M
  • Immunologic Capping
  • Lymph Nodes
  • Lymphocyte Activation
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Platelet Count
  • Proteins
  • Receptor-CD3 Complex, Antigen, T-Cell
  • T-Lymphocytes
  • Wiskott-Aldrich Syndrome
  • Wiskott-Aldrich Syndrome Protein