期刊
EXPERT REVIEW OF HEMATOLOGY
卷 2, 期 3, 页码 315-334出版社
TAYLOR & FRANCIS LTD
DOI: 10.1586/EHM.09.17
关键词
animal model; GATA1; hematopoietic stem cell; megakaryocyte; primary myelofibrosis; transcription factor
类别
资金
- Ministero per la Ricerca Scientifica and Alleanza sul Cancro, Italy
- National Cancer Institute, NIH, USA [P01-CA108671]
- NATIONAL CANCER INSTITUTE [P01CA108671] Funding Source: NIH RePORTER
Primary myelofibrosis (PMF) belongs to the Philadelphia-negative myeloproliferative neoplasms and is a hematological disorder caused by abnormal function of the hematopoietic stem cells. The disease manifests itself with a plethora of alterations, including anemia, splenomegaly and extramedullary hematopoiesis. Its hallmarks are progressive marrow fibrosis and atypical megakaryocytic hyperplasia, two distinctive features used to clinically monitor disease progression. In an attempt to investigate the role of abnormal megakaryocytopoiesis in the pathogenesis of PMF, several transgenic mouse models have been generated. These models are based either on mutations that interfere with the extrinsic (thrombopoietin and its receptor, MPL) and intrinsic (the GATA1 transcription factor) control of normal megakaryocytopoiesis, or on known genetic lesions associated with the human disease. Here we provide an up-to-date review on the insights into the pathobiology of human PMF achieved by studying these animal models, with particular emphasis on results obtained with Gata1(low) mice.
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