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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 (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 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 Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted April 30, 2004 Accepted July 16, 2004 Mechanisms of WASp-mediated haematological and immunological disease Siobhan Burns, Giles O Cory, William Vainchenker, and Adrian J Thrasher* Molecular Immunology Unit, Institute of Child Health, University College London, London, United Kingdom; Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom Biochemistry Department, University of Bristol, Bristol, United Kingdom Hematopoiese et cellules souches, Institut National de la sante et de la recherche medicale (INSERM) Unit 362 and Institut Gustave Roussy, VILLEJUIF, France * Corresponding author; email: a.thrasher{at}ich.ucl.ac.uk, s.burns@ich.ucl.ac.uk. The Wiskott-Aldrich syndrome protein (WASp) is a key regulator of actin polymerisation in haematopoietic cells. The dynamic nature of cytoskeletal changes during a variety of cellular processes demands complex mechanisms for co-ordinated integration of input signals, precise localisation 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 haematological and immunological 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]

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