@article{7488c747787247e28029013126b22638,
title = "Wiskoff-Aldrich syndrome protein-deficient mice reveal a role for WASP in T but not B cell activation",
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.",
author = "Snapper, {Scott B.} and Rosen, {Fred S.} and Emiko Mizoguchi and Paul Cohen and Wasif Khan and Liu, {Ching Hui} and Hagemann, {Tracy L.} and Kwan, {Sau Ping} and Roger Ferrini and Laurie Davidson and Bhan, {Atul K.} and Alt, {Frederick W.}",
note = "Funding Information: This work was supported by the Howard Hughes Medical Institute (F. W. A.) and National Institutes of Health (NIH) grants AI20047 (F. W. A.), HL03749 (S. B. S.), HL59561 (F. S. R. and F. W. A.), DK47677 (A. K. B), AI31587 and KO4–01044 (S.-P. K.), and by the Massachusetts General Hospital/New England Regional Primate Reseach NIH Center for the Study of Inflammatory Bowel Disease DK43551 (A. K. B and S. B. S.). S. B. S. was also supported initially by an NIH training grant (DK73354368) and as an Associate of the Howard Hughes Medical Institute (Physician Postdoctoral Research Fellowship Program). We thank the WAS fund of the Center for Blood Research and Abul Abbas, Frank Cronin, Paul Frenette, Jimmy Mond, Andy Lees, Dianne Kenney, Raif Geha, Tom Kirchhausen, Wojciech Swat, Timo Breit, Jayanta Chaudhuri, Barry Sleckman, John Hartwig, and Ulrich Von Andrian for advice and/or reagents. A request for submission of the WASP-deficient mice has been made to Jackson Laboratories.",
year = "1998",
month = jul,
doi = "10.1016/S1074-7613(00)80590-7",
language = "English (US)",
volume = "9",
pages = "81--91",
journal = "Immunity",
issn = "1074-7613",
publisher = "Cell Press",
number = "1",
}