Impaired GATA-1 expression and myelofibrosis in an animal model

Pathol Biol (Paris). 2004 Jun;52(5):275-9. doi: 10.1016/j.patbio.2004.02.008.

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • DNA-Binding Proteins / deficiency
  • DNA-Binding Proteins / genetics*
  • Disease Models, Animal
  • Erythroid-Specific DNA-Binding Factors
  • GATA1 Transcription Factor
  • Gene Expression Regulation / genetics*
  • Mice
  • Mice, Knockout
  • Primary Myelofibrosis / genetics*
  • Transcription Factors / deficiency
  • Transcription Factors / genetics*

Substances

  • DNA-Binding Proteins
  • Erythroid-Specific DNA-Binding Factors
  • GATA1 Transcription Factor
  • Gata1 protein, mouse
  • Transcription Factors