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

Research output: Contribution to journalArticle

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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
Pages (from-to)81-91
Number of pages11
JournalImmunity
Volume9
Issue number1
DOIs
StatePublished - Jul 1 1998
Externally publishedYes

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Wiskott-Aldrich Syndrome
B-Lymphocytes
Proteins
Genes
T-Lymphocytes
Mutation
Antigen Receptors
Lymphocyte Count
Colitis
Platelet Count
Actin Cytoskeleton
Immunoglobulins
Lymphocytes
Antigens
Serum

ASJC Scopus subject areas

  • Immunology and Allergy
  • Infectious Diseases
  • Immunology

Cite this

Wiskoff-Aldrich syndrome protein-deficient mice reveal a role for WASP in T but not B cell activation. / Snapper, Scott B.; Rosen, Fred S.; Mizoguchi, Emiko; Cohen, Paul; Khan, Wasif; Liu, Ching Hui; Hagemann, Tracy L.; Kwan, Sau Ping; Ferrini, Roger; Davidson, Laurie; Bhan, Atul K.; Alt, Frederick W.

In: Immunity, Vol. 9, No. 1, 01.07.1998, p. 81-91.

Research output: Contribution to journalArticle

Snapper, SB, Rosen, FS, Mizoguchi, E, Cohen, P, Khan, W, Liu, CH, Hagemann, TL, Kwan, SP, Ferrini, R, Davidson, L, Bhan, AK & Alt, FW 1998, 'Wiskoff-Aldrich syndrome protein-deficient mice reveal a role for WASP in T but not B cell activation', Immunity, vol. 9, no. 1, pp. 81-91. https://doi.org/10.1016/S1074-7613(00)80590-7
Snapper, Scott B. ; Rosen, Fred S. ; Mizoguchi, Emiko ; Cohen, Paul ; Khan, Wasif ; Liu, Ching Hui ; Hagemann, Tracy L. ; Kwan, Sau Ping ; Ferrini, Roger ; Davidson, Laurie ; Bhan, Atul K. ; Alt, Frederick W. / Wiskoff-Aldrich syndrome protein-deficient mice reveal a role for WASP in T but not B cell activation. In: Immunity. 1998 ; Vol. 9, No. 1. pp. 81-91.
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AU - Khan, Wasif

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AU - Hagemann, Tracy L.

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