Animal models of myelofibrosis

Abstract

The pathogenesis of idiopathic myelofibrosis has not yet been completely elucidated, but the availability of animal models of the disease has made it possible, in the past few years, to focus on the cells (megakaryocytes) and cytokines (transforming growth factor-β, among others) potentially involved. This review will deal with the two best characterized animal models (the TPO-overexpressing and the GATA-1low mouse) as well as with the newest ones that have been developed following the identification of the JAK2 V617F and MPL W515K/L mutations in the human form of the disease. Each of these unique animal models conceivably represents an invaluable tool for further characterizing the disease mechanisms as well as for testing novel therapeutic agents. © 2008 Humana Press Inc.