SEARCH:   Advanced

Blood, 1 February 2005, Vol. 105, No. 3, pp. 1144-1152. Prepublished online as a Blood First Edition Paper on September 21, 2004; DOI 10.1182/blood-2004-03-1003. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-03-1003v1 105/3/1144    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Westerberg, L. Articles by Severinson, E. Search for Related Content PubMed PubMed Citation Articles by Westerberg, L. Articles by Severinson, E. Related Collections Immunobiology Cell Adhesion and Motility Cytoskeleton Chemokines, Cytokines, and Interleukins Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  IMMUNOBIOLOGY Wiskott-Aldrich syndrome protein deficiency leads to reduced B-cell adhesion, migration, and homing, and a delayed humoral immune response Lisa Westerberg, Malin Larsson, Samantha J. Hardy, Carmen Fernández, Adrian J. Thrasher, and Eva Severinson From the Department of Cell and Molecular Biology, Medical Nobel Institute, Karolinska Institutet, Stockholm, Sweden; the Institute of Child Health, University College London, London, United Kingdom; and the Department of Immunology, The Wenner-Gren Institute, Stockholm University, Stockholm, Sweden. The Wiskott-Aldrich syndrome protein (WASp) is mutated in the severe immunodeficiency disease Wiskott-Aldrich syndrome (WAS). The function of B cells and the physiologic alterations in WAS remain unclear. We show that B cells from WAS patients exhibited decreased motility and had reduced capacity to migrate, adhere homotypically, and form long protrusions after in vitro culture. WASp-deficient murine B cells also migrated less well to chemokines. Upon antigen challenge, WASp-deficient mice mounted a reduced and delayed humoral immune response to both T-cell–dependent and –independent antigens. This was at least in part due to deficient migration and homing of B cells. In addition, the germinal center reaction was reduced in WASp-deficient mice. Thus, WASp is crucial for optimal B-cell responses and plays a pivotal role in the primary humoral immune response. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. P. Blundell, G. Bouma, J. Metelo, A. Worth, Y. Calle, L. A. Cowell, L. S. Westerberg, D. A. Moulding, S. Mirando, C. Kinnon, et al. Phosphorylation of WASp is a key regulator of activity and stability in vivo PNAS, September 15, 2009; 106(37): 15738 - 15743. [Abstract] [Full Text] [PDF] M. Bosticardo, F. Marangoni, A. Aiuti, A. Villa, and M. Grazia Roncarolo Recent advances in understanding the pathophysiology of Wiskott-Aldrich syndrome Blood, June 18, 2009; 113(25): 6288 - 6295. [Abstract] [Full Text] [PDF] A. Astrakhan, H. D. Ochs, and D. J. Rawlings Wiskott-Aldrich Syndrome Protein Is Required for Homeostasis and Function of Invariant NKT Cells J. Immunol., June 15, 2009; 182(12): 7370 - 7380. [Abstract] [Full Text] [PDF] L. D. Notarangelo and R. Badolato Leukocyte trafficking in primary immunodeficiencies J. Leukoc. Biol., March 1, 2009; 85(3): 335 - 343. [Abstract] [Full Text] [PDF] E. Severinson WASp stings mature lymphocytes Blood, November 15, 2008; 112(10): 3921 - 3922. [Full Text] [PDF] A. Meyer-Bahlburg, S. Becker-Herman, S. Humblet-Baron, S. Khim, M. Weber, G. Bouma, A. J. Thrasher, F. D. Batista, and D. J. Rawlings Wiskott-Aldrich syndrome protein deficiency in B cells results in impaired peripheral homeostasis Blood, November 15, 2008; 112(10): 4158 - 4169. [Abstract] [Full Text] [PDF] L. S. Westerberg, M. A. de la Fuente, F. Wermeling, H. D. Ochs, M. C. I. Karlsson, S. B. Snapper, and L. D. Notarangelo WASP confers selective advantage for specific hematopoietic cell populations and serves a unique role in marginal zone B-cell homeostasis and function Blood, November 15, 2008; 112(10): 4139 - 4147. [Abstract] [Full Text] [PDF] L. Quemeneur, V. Angeli, M. Chopin, and R. Jessberger SWAP-70 deficiency causes high-affinity plasma cell generation despite impaired germinal center formation Blood, March 1, 2008; 111(5): 2714 - 2724. [Abstract] [Full Text] [PDF] G. Bouma, S. Burns, and A. J. Thrasher Impaired T-cell priming in vivo resulting from dysfunction of WASp-deficient dendritic cells Blood, December 15, 2007; 110(13): 4278 - 4284. [Abstract] [Full Text] [PDF] V. Cotta-de-Almeida, L. Westerberg, M. H. Maillard, D. Onaldi, H. Wachtel, P. Meelu, U.-i. Chung, R. Xavier, F. W. Alt, and S. B. Snapper Wiskott Aldrich syndrome protein (WASP) and N-WASP are critical for T cell development PNAS, September 25, 2007; 104(39): 15424 - 15429. [Abstract] [Full Text] [PDF] M. H. Maillard, V. Cotta-de-Almeida, F. Takeshima, D. D. Nguyen, P. Michetti, C. Nagler, A. K. Bhan, and S. B. Snapper The Wiskott-Aldrich syndrome protein is required for the function of CD4+CD25+Foxp3+ regulatory T cells J. Exp. Med., February 19, 2007; 204(2): 381 - 391. [Abstract] [Full Text] [PDF]

	Copyright © 2005 by American Society of Hematology         Online ISSN: 1528-0020