Please use this identifier to cite or link to this item:
https://hdl.handle.net/2440/66648
Citations | ||
Scopus | Web of Science® | Altmetric |
---|---|---|
?
|
?
|
Type: | Journal article |
Title: | Hematopoietic defects in the Ts1Cje mouse model of Down syndrome |
Author: | Carmichael, C. Majewski, I. Alexander, W. Metcalf, D. Hilton, D. Hewitt, C. Scott, H. |
Citation: | Blood, 2009; 113(9):1929-1937 |
Publisher: | Amer Soc Hematology |
Issue Date: | 2009 |
ISSN: | 0006-4971 1528-0020 |
Statement of Responsibility: | Catherine L. Carmichael, Ian J. Majewski, Warren S. Alexander, Donald Metcalf, Douglas J. Hilton, Chelsee A. Hewitt and Hamish S. Scott |
Abstract: | Down syndrome (DS) persons 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 premalignant 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 analyzed 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 orthologs, when combined, do not appear to be sufficient to induce TMD or AMKL-like phenotypes in mice. |
Keywords: | Spleen Hematopoietic Stem Cells Cells, Cultured Chromosomes, Human, Pair 21 Bone Marrow Animals Mice, Inbred BALB C Mice, Inbred C57BL Mice, Transgenic Humans Mice Down Syndrome Hematologic Diseases Thrombocytosis Disease Models, Animal Survival Analysis Aging GATA1 Transcription Factor |
Rights: | Copyright © 2011 by American Society of Hematology |
DOI: | 10.1182/blood-2008-06-161422 |
Published version: | http://dx.doi.org/10.1182/blood-2008-06-161422 |
Appears in Collections: | Aurora harvest Molecular and Biomedical Science publications |
Files in This Item:
There are no files associated with this item.
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.