title: Impaired GATA-1 expression and myelofibrosis in an animal model.
abstract: 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.
authors.affiliation: UF di Ematologia, Az. Osp. Careggi, University of Florence, 50134 Florence, Italy. a.vannucchi@dac.unifi.it
authors.forename: Alessandro M
authors.initials: AM
authors.lastname: Vannucchi
authors.forename: Lucia
authors.initials: L
authors.lastname: Bianchi
authors.forename: Francesco
authors.initials: F
authors.lastname: Paoletti
authors.forename: Viviana
authors.initials: V
authors.lastname: Di Giacomo
authors.forename: Giovanni
authors.initials: G
authors.lastname: Migliaccio
authors.forename: Anna Rita
authors.initials: AR
authors.lastname: Migliaccio
mesh_terms: 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*
chemical_list: D004268:DNA-Binding Proteins; D050981:Erythroid-Specific DNA-Binding Factors; D050982:GATA1 Transcription Factor; C494689:Gata1 protein, mouse; D014157:Transcription Factors