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Blood, 3 September 2009, Vol. 114, No. 10, pp. 2107-2120. Prepublished online as a Blood First Edition Paper on July 1, 2009; DOI 10.1182/blood-2009-03-211680. This Article Full Text (PDF) Supplemental Tables and Figures All Versions of this Article: blood-2009-03-211680v1 114/10/2107    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 CrossRef Google Scholar Articles by Migliaccio, A. R. Articles by Fibbe, W. E PubMed PubMed Citation Articles by Migliaccio, A. R. Articles by Fibbe, W. E Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted March 18, 2009 Accepted June 5, 2009 GATA1 expression driven by the alternative HS2 enhancer in the spleen rescues the hematopoietic failure induced by the hypomorphic GATA1low mutation Anna Rita Migliaccio*, Fabrizio Martelli, Maria Verrucci, Massimo Sanchez, Mauro Valeri, Giovanni Migliaccio, Alessandro Maria Vannucchi, Maria Zingariello, Angela Di Baldassarre, Barbara Ghinassi, Rosa Alba Rana, Yvette van Hensbergen, and Willem E Fibbe Department of Hematology/Oncology and Molecular Medicine, Istituto Superiore Sanita, Rome, Italy Department of Cell Biology and Neurosciences, Istituto Superiore Sanita, Rome, Italy Department of Quality and Security of Animal Experimentation, Istituto Superiore Sanita, Rome, Italy Hematology, Azienda Ospedaliera Careggi, Florence, Italy Biomorphology and Medical Genetics, University G. D'Annunzio, Chieti, Italy Medicine, Mount Sinai School of Medicine and the Myeloproliferative Disease Consortium, New York, NY, United States Sanquin Blod Foundation, Leiden University Medical Center, Leiden, Netherlands Immunohematology and Blood Transfusion, Leiden University Medical Center, Leiden, Netherlands * Corresponding author; email: annarita.migliaccio{at}mssm.edu. Rigorously defined reconstitution assays developed in recent years have allowed recognition of the delicate relationship which exists between hematopoietic stem cells and their niches. This balance ensures that hematopoiesis occurs in the marrow under steady state conditions. However, during development, recovery from hemopoietic stress and in myeloproliferative disorders, hematopoiesis occurs in extramedullary sites whose microenvironments are still poorly defined. The hypomorphic Gata1low mutation deletes the regulatory sequences of the gene necessary for its expression in hematopoietic cells generated in the marrow. By analyzing the mechanism that rescues hematopoiesis in mice carrying this mutation, we provide evidence that extramedullary microenvironments sustain maturation of stem cells that would be otherwise incapable of maturing in the marrow. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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