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Immunity. 1998 Jul;9(1):81-91.

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

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1
Howard Hughes Medical Institute, Children's Hospital, Boston, Massachusetts 02115, USA.

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-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.

PMID:
9697838
DOI:
10.1016/s1074-7613(00)80590-7
[Indexed for MEDLINE]
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