Impaired GATA-1 expression and myelofibrosis in an animal model.
The genetic lesion(s) underlying chronic idiopathic myelofibrosis, as well as the mechanisms leading to the typical fibro-osteosclerotic changes of the bone marrow microenvironment, are still undefined. Recently, animal models of the disease have been described. We will briefly review the characteristics of these models, the thrombopoietin-overexpressing mice and the GATA-1(low) mice (mice deficient for GATA-1 expression in megakaryocytes), and illustrate how they provided insights into pathogenetic mechanisms of myelofibrosis, with special regard to the role of abnormal megakaryocyte proliferation and maturation.
Vannucchi
Alessandro M
AM
UF di Ematologia, Az. Osp. Careggi, University of Florence, 50134 Florence, Italy. a.vannucchi@dac.unifi.it
Bianchi
Lucia
L
Paoletti
Francesco
F
Di Giacomo
Viviana
V
Migliaccio
Giovanni
G
Migliaccio
Anna Rita
AR
D000818:Animals; D004268:DNA-Binding Proteins / Q000172:deficiency / Q000235:genetics*; D004195:Disease Models, Animal; D050981:Erythroid-Specific DNA-Binding Factors; D050982:GATA1 Transcription Factor; D005786:Gene Expression Regulation / Q000235:genetics*; D051379:Mice; D018345:Mice, Knockout; D055728:Primary Myelofibrosis / Q000235:genetics*; D014157:Transcription Factors / Q000172:deficiency / Q000235:genetics*
D004268:DNA-Binding Proteins; D050981:Erythroid-Specific DNA-Binding Factors; D050982:GATA1 Transcription Factor; C494689:Gata1 protein, mouse; D014157:Transcription Factors