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Prepublished online as a Blood First Edition Paper on May 1, 2003; DOI 10.1182/blood-2002-07-2099.
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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.
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