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

Scott B. Snapper, Fred S. Rosen, Emiko Mizoguchi, Paul Cohen, Wasif Khan, Ching Hui Liu, Tracy L. Hagemann, Sau Ping Kwan, Roger Ferrini, Laurie Davidson, Atul K. Bhan, Frederick W. Alt

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396 Scopus citations

Abstract

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- indepedent 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-CD3ε stimulation. Yet, purified WASP-deficient B cells showed normal responses to anti-lg 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.

Original languageEnglish (US)
Pages (from-to)81-91
Number of pages11
JournalImmunity
Volume9
Issue number1
DOIs
StatePublished - Jul 1998
Externally publishedYes

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Infectious Diseases

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    Snapper, S. B., Rosen, F. S., Mizoguchi, E., Cohen, P., Khan, W., Liu, C. H., Hagemann, T. L., Kwan, S. P., Ferrini, R., Davidson, L., Bhan, A. K., & Alt, F. W. (1998). Wiskoff-Aldrich syndrome protein-deficient mice reveal a role for WASP in T but not B cell activation. Immunity, 9(1), 81-91. https://doi.org/10.1016/S1074-7613(00)80590-7