title: Murine model of hypereosinophilic syndromes/chronic eosinophilic leukemia.
abstract: Hypereosinophilic syndrome (HES) includes heterogeneous hematological disorders that are characterized by distinctive blood and tissue eosinophilia. In addition to classical HES criteria, the World Health Organization proposed a set of criteria that distinguish chronic eosinophilic leukemia (CEL) from HES. As such, the fusion gene FIP1L1/PDGFRA was found as a cause of CEL in a significant proportion of patients initially diagnosed as having HES. Several investigations have tried to dissect the mechanism of leukemogenesis; eosinophilia and signaling induced by FIP1L1/PDGFRalpha in cell lines, bone marrow mast cells, primary human eosinophils and in murine myeloproliferative disorder models. In this review, we introduce the current knowledge on the relationship between FIP1L1/PDGFRalpha and cell signaling, eosinophil proliferation, survival and activation and mastocytosis specially focusing on the evidence learned from murine models.
authors.affiliation: Division of Allergy and Immunology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. yamaday@gcmc.pref.gunma.jp
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Yamada
authors.forename: Jose A
authors.initials: JA
authors.lastname: Cancelas
authors.forename: Marc E
authors.initials: ME
authors.lastname: Rothenberg
mesh_terms: D000818:Animals; D001549:Benzamides; D002908:Chronic Disease; D004195:Disease Models, Animal; D004804:Eosinophils / Q000276:immunology*; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism*; D000068877:Imatinib Mesylate; D008415:Mastocytosis / Q000209:etiology / Q000378:metabolism; D051379:Mice; D015514:Oncogene Proteins, Fusion / Q000737:chemistry / Q000235:genetics; D010879:Piperazines / Q000627:therapeutic use; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000737:chemistry / Q000235:genetics; D015398:Signal Transduction; D039221:mRNA Cleavage and Polyadenylation Factors / Q000737:chemistry / Q000235:genetics
chemical_list: D001549:Benzamides; D015514:Oncogene Proteins, Fusion; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D039221:mRNA Cleavage and Polyadenylation Factors; D000068877:Imatinib Mesylate; C479819:FIP1L1-PDGFRA fusion protein, human; D020796:Receptor, Platelet-Derived Growth Factor alpha