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Development of myelofibrosis in mice genetically impaired for GATA-1 expression (GATA-1(low) mice).

Authors: Vannucchi, AM  Bianchi, L  Cellai, C  Paoletti, F  Rana, RA  Lorenzini, R  Migliaccio, G  Migliaccio, AR 
Citation: Vannucchi AM, etal., Blood. 2002 Aug 15;100(4):1123-32.
Pubmed: (View Article at PubMed) PMID:12149188
DOI: Full-text: DOI:10.1182/blood-2002-06-1913

The phenotype induced by the GATA-1(low) (neodeltaHS) mutation is here further characterized by analyzing the hemopoietic system during the aging (up to 20 months) of a GATA-1(low) colony (135 mutants and 40 normal littermates). Mutants expressed normal hematocrit values (Hct = 45.9 +/- 4.0) until 12 months but became anemic from 15 months on (Hct = 30.9 +/- 3.9; P <.05). Anemia was associated with several markers of myelofibrosis such as the presence of tear-drop poikilocytes and progenitor cells in the blood, collagen fibers in the marrow and in the spleen, and hemopoietic foci in the liver. Semiquantitative reverse transcription-polymerase chain reaction showed that growth factor genes implicated in the development of myelofibrosis (such as osteocalcin, transforming growth factor-beta1, platelet-derived growth factor, and vascular endothelial growth factor) were all expressed in the marrow from the mutants at higher levels than in corresponding normal tissues. The GATA-1(low) mutants experienced a slow progression of the disease because the final exitus was not observed until at least 15 months with a probability of survival more favorable than that of W/Wv mice concurrently kept in the animal facility (P <.001, by Kaplan-Meier analysis). In conclusion, impaired GATA-1 expression may contribute to the development of myelofibrosis, and the GATA-1(low) mutants may represent a suitable animal model for the human disease that may shed light on its pathogenesis.


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CRRD Object Information
CRRD ID: 10450737
Created: 2016-01-20
Species: All species
Last Modified: 2016-01-20
Status: ACTIVE


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.