Preparing your results

Our searching services are busy right now. Your search will reload in five seconds.

Forgot Password

If you have forgotten your password you can enter your email here and get a temporary password sent to your email.

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