SEARCH:   Advanced

Blood, 1 December 2004, Vol. 104, No. 12, pp. 3454-3462. Prepublished online as a Blood First Edition Paper on August 12, 2004; DOI 10.1182/blood-2004-04-1678. This Article Full Text Full Text (PDF) All Versions of this Article: 2004-04-1678v1 104/12/3454    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 Burns, S. Articles by Thrasher, A. J. Search for Related Content PubMed PubMed Citation Articles by Burns, S. Articles by Thrasher, A. J. Related Collections Hemostasis, Thrombosis, and Vascular Biology Immunobiology Cell Adhesion and Motility Cytoskeleton Signal Transduction Gene Expression Review Articles Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  REVIEW ARTICLES Mechanisms of WASp-mediated hematologic and immunologic disease Siobhan Burns, Giles O. Cory, William Vainchenker, and Adrian J. Thrasher From the Molecular Immunology Unit, Institute of Child Health, University College London, United Kingdom; Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom; the Biochemistry Department, University of Bristol, Bristol, United Kingdom; and the Institut National de la santé etdela recherche médicale (INSERM) Unit 362 and Institut Gustave Roussy, INSERM U 362, Hematopoièse et cellules souches, Villejuif, Cedex, France. The Wiskott-Aldrich syndrome protein (WASp) is a key regulator of actin polymerization in hematopoietic cells. The dynamic nature of cytoskeletal changes during a variety of cellular processes demands complex mechanisms for coordinated integration of input signals, precise localization within the cell, and regulated activation of the Arp2/3 complex. Mutations in the Wiskott-Aldrich syndrome gene either inhibit or dysregulate normal WASp function, resulting in clinical diseases with complex and disparate phenotypes. This review highlights recent advances that have enhanced our understanding of the mechanisms by which these molecular defects cause hematologic and immunologic disease. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: J. Pulecio, E. Tagliani, A. Scholer, F. Prete, L. Fetler, O. R. Burrone, and F. Benvenuti Expression of Wiskott-Aldrich Syndrome Protein in Dendritic Cells Regulates Synapse Formation and Activation of Naive CD8+ T Cells J. Immunol., July 15, 2008; 181(2): 1135 - 1142. [Abstract] [Full Text] [PDF] S. Tsuboi and J. Meerloo Wiskott-Aldrich Syndrome Protein Is a Key Regulator of the Phagocytic Cup Formation in Macrophages J. Biol. Chem., November 23, 2007; 282(47): 34194 - 34203. [Abstract] [Full Text] [PDF] D. A. Moulding, M. P. Blundell, D. G. Spiller, M. R.H. White, G. O. Cory, Y. Calle, H. Kempski, J. Sinclair, P. J. Ancliff, C. Kinnon, et al. Unregulated actin polymerization by WASp causes defects of mitosis and cytokinesis in X-linked neutropenia J. Exp. Med., September 3, 2007; 204(9): 2213 - 2224. [Abstract] [Full Text] [PDF] X.-S. Liu, X.-H. Li, Y. Wang, R.-Z. Shu, L. Wang, S.-Y. Lu, H. Kong, Y.-E Jin, L.-J. Zhang, J. Fei, et al. Disruption of palladin leads to defects in definitive erythropoiesis by interfering with erythroblastic island formation in mouse fetal liver Blood, August 1, 2007; 110(3): 870 - 876. [Abstract] [Full Text] [PDF] Y. Li, N. Clough, X. Sun, W. Yu, B. L. Abbott, C. J. Hogan, and Z. Dai Bcr-Abl induces abnormal cytoskeleton remodeling, beta1 integrin clustering and increased cell adhesion to fibronectin through the Abl interactor 1 pathway J. Cell Sci., April 15, 2007; 120(8): 1436 - 1446. [Abstract] [Full Text] [PDF] S. Tsuboi Requirement for a Complex of Wiskott-Aldrich Syndrome Protein (WASP) with WASP Interacting Protein in Podosome Formation in Macrophages J. Immunol., March 1, 2007; 178(5): 2987 - 2995. [Abstract] [Full Text] [PDF] A. Konno, M. Kirby, S. A. Anderson, P. L. Schwartzberg, and F. Candotti The expression of Wiskott-Aldrich syndrome protein (WASP) is dependent on WASP-interacting protein (WIP) Int. Immunol., February 1, 2007; 19(2): 185 - 192. [Abstract] [Full Text] [PDF] S. Trifari, G. Sitia, A. Aiuti, S. Scaramuzza, F. Marangoni, L. G. Guidotti, S. Martino, P. Saracco, L. D. Notarangelo, M.-G. Roncarolo, et al. Defective Th1 Cytokine Gene Transcription in CD4+ and CD8+ T Cells from Wiskott-Aldrich Syndrome Patients J. Immunol., November 15, 2006; 177(10): 7451 - 7461. [Abstract] [Full Text] [PDF] V. Binder, M. H. Albert, M. Kabus, M. Bertone, A. Meindl, and B. H. Belohradsky The Genotype of the Original Wiskott Phenotype N. Engl. J. Med., October 26, 2006; 355(17): 1790 - 1793. [Abstract] [Full Text] [PDF] P. J. Ancliff, M. P. Blundell, G. O. Cory, Y. Calle, A. Worth, H. Kempski, S. Burns, G. E. Jones, J. Sinclair, C. Kinnon, et al. Two novel activating mutations in the Wiskott-Aldrich syndrome protein result in congenital neutropenia Blood, October 1, 2006; 108(7): 2182 - 2189. [Abstract] [Full Text] [PDF] S. Sabri, A. Foudi, S. Boukour, B. Franc, S. Charrier, M. Jandrot-Perrus, R. W. Farndale, A. Jalil, M. P. Blundell, E. M. Cramer, et al. Deficiency in the Wiskott-Aldrich protein induces premature proplatelet formation and platelet production in the bone marrow compartment Blood, July 1, 2006; 108(1): 134 - 140. [Abstract] [Full Text] [PDF] M. I. Lutskiy, D. S. Beardsley, F. S. Rosen, and E. Remold-O'Donnell Mosaicism of NK cells in a patient with Wiskott-Aldrich syndrome Blood, October 15, 2005; 106(8): 2815 - 2817. [Abstract] [Full Text] [PDF]

	Copyright © 2004 by American Society of Hematology         Online ISSN: 1528-0020