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Prepublished online as a Blood First Edition Paper on May 1, 2003; DOI 10.1182/blood-2002-07-2099. This Article Full Text Full Text (PDF) All Versions of this Article: 2002-07-2099v1 102/4/1282    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 Lacout, C. Articles by Duménil, D. Search for Related Content PubMed PubMed Citation Articles by Lacout, C. Articles by Duménil, D. Related Collections Hematopoiesis and Stem Cells Cell Adhesion and Motility Cytoskeleton Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 15 August 2003, Vol. 102, No. 4, pp. 1282-1289 HEMATOPOIESIS A defect in hematopoietic stem cell migration explains the nonrandom X-chromosome inactivation in carriers of Wiskott-Aldrich syndrome Catherine Lacout, Elie Haddad, Siham Sabri, Fedor Svinarchouk, Loic Garçon, Claude Capron, Adlen Foudi, Rym Mzali, Scott B. Snapper, Fawzia Louache, William Vainchenker, and Dominique Duménil From U362 Institut National de la Santé et de la Recherche Médicale (INSERM), Institut Gustave Roussy, Villejuif, France; Massachusetts General Hospital, Boston, MA; and INSERM U461, Faculté de Pharmacie, Chatenay-Malabry, France. A defect in cell trafficking and chemotaxis plays an important role in the immune deficiency observed in Wiskott-Aldrich syndrome (WAS). In this report, we show that marrow cells from WAS protein (WASP)–deficient mice also have a defect in chemotaxis. Serial transplantation and competitive reconstitution experiments demonstrated that marrow cells, including hematopoietic progenitors and stem cells (HSCs), have decreased homing capacities that were associated with a defect in adhesion to collagen. During development, HSCs migrate from the liver to the marrow and the spleen, prompting us to ask if a defect in HSC homing during development may explain the skewed X-chromosome inactivation in WAS carriers. Preliminary evidence has shown that, in contrast to marrow progenitor cells, fetal liver progenitor cells from heterozygous females had a random X-chromosome inactivation. When fetal liver cells from WASP-carrier females were injected into irradiated recipients, a nonrandom inactivation of the X-chromosome was found at the level of hematopoietic progenitors and HSCs responsible for the short- and long-term hematopoietic reconstitution. Therefore, the mechanism of the skewed X-chromosomal inactivation observed in WAS carriers may be related to a migration defect of WASP-deficient HSCs. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Mani, S. Venkatasubrahmanyam, M. Sanyal, S. Levy, A. Butte, K. Weinberg, and T. Jahn Wiskott-Aldrich syndrome protein is an effector of Kit signaling Blood, October 1, 2009; 114(14): 2900 - 2908. [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. 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