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Blood, 26 February 2009, Vol. 113, No. 9, pp. 1929-1937. Prepublished online as a Blood First Edition Paper on December 24, 2008; DOI 10.1182/blood-2008-06-161422. This Article Full Text (PDF) Supplemental Tables and Figures All Versions of this Article: blood-2008-06-161422v1 113/9/1929    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 Carmichael, C. L Articles by Scott, H. S Search for Related Content PubMed PubMed Citation Articles by Carmichael, C. L Articles by Scott, H. S Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted June 5, 2008 Accepted October 27, 2008 Hematopoietic defects in the Ts1Cje mouse model of Down syndrome Catherine L Carmichael*, Ian J Majewski, Warren S Alexander, Donald Metcalf, Douglas J Hilton, Chelsee A Hewitt, and Hamish S Scott Molecular Medicine, Walter and Eliza Hall Institute of Medical Research (WEHI), Melbourne, VIC, Australia Department of Medical Biology, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Parkville, VIC, Australia Cancer and Haematology, Walter and Eliza Hall Institute of Medical Research, Melbourne, VIC, Australia Department of Pathology, Peter MacCallum Cancer Centre, Melbourne, VIC, Australia Division of Molecular Pathology, The Institute of Medical and Veterinary Science and the Hanson Institute, Adelaide, SA, Australia * Corresponding author; email: carmichael{at}wehi.edu.au. Down syndrome (DS) individuals are born with various hematopoietic abnormalities, ranging from relatively benign, such as neutrophilia and macrocytosis, to a more severe transient myeloproliferative disorder (TMD). In most cases these abnormalities resolve in the first few months to years of life. However, sometimes the TMD represents a pre-malignant disease that develops into Acute Megakaryocytic Leukemia (AMKL), usually in association with acquired GATA1 mutations. To gain insight into the mechanisms responsible for these abnormalities, we analysed the hematopoietic development of the Ts1Cje mouse model of DS. Our analyses identified defects in mature blood cells, including macrocytosis and anemia, as well as abnormalities in fetal liver and bone marrow stem and progenitor cell function. Despite these defects the Ts1Cje mice do not develop disease resembling either TMD or AMKL, and this was not altered by a loss of function allele of Gata1. Thus loss of Gata1 and partial trisomy of chromosome 21 orthologues, when combined, do not appear to be sufficient to induce TMD or AMKL-like phenotypes in mice. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: S. Malinge, S. Izraeli, and J. D. Crispino Insights into the manifestations, outcomes, and mechanisms of leukemogenesis in Down syndrome Blood, March 19, 2009; 113(12): 2619 - 2628. [Abstract] [Full Text] [PDF]

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