From the Institut National de la Santé et de la Recherche Médicale (INSERM) U 119, IFR 57, Marseille, France; Université de la Méditerranée, Marseille, France; Department of Hematology Institut Paoli Calmettes, Marseille, France; Department of BioPathology, Institut Paoli Calmettes, Marseille, France; and INSERM EMI-0116, IFR 57, Marseille, France.
Constitutive activation of aberrant fibroblast growth factor receptor 1 (FGFR1) kinase as a consequence of gene fusion such as FOP-FGFR1 associated with t(6; 8)(q27;p11-12) translocation, is the hallmark of an atypical aggressive stem cell myeloproliferative disorder (MPD) in humans. In this study, we show that expression of FOP-FGFR1 in primary bone marrow cells induced by retroviral transduction generates a MPD in mice. Constitutive FOP-FGFR1 kinase activity was both essential and sufficient to cause a chronic myeloproliferative syndrome in the murine bone marrow transplantation model. In contrast to the human disorder, lymphoproliferation and progression to acute phase were not observed. Lymphoid symptoms, however, appeared when onset of the disease was delayed as the result of mutation of FOP-FGFR1 at tyrosine 511, the phospholipase C
(PLC
) binding site.
Acute graft-versus-host disease in patients with Fanconi anemia or acquired aplastic anemia undergoing bone marrow transplantation from HLA-identical sibling donors: risk factors and influence on outc
Adult human hematopoietic cells provide functional hemangioblast activity
Annexin II mediates plasminogen-dependent matrix invasion by human monocytes: enhanced expression by macrophages
Antibody response to DBY minor histocompatibility antigen is induced after allogeneic stem cell transplantation and in healthy female donors
Bcl-xL antagonism of BCR-coupled mitochondrial phospholipase A2 signaling correlates with protection from apoptosis in WEHI-231 B cells
CCL16 activates an angiogenic program in vascular endothelial cells
Combined deficiency of protease-activated receptor-4 and fibrinogen recapitulates the hemostatic defect but not the embryonic lethality of prothrombin deficiency
Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray
Effects of prednisone and genetic polymorphisms on etoposide disposition in children with acute lymphoblastic leukemia
Enhanced in vivo platelet adhesion in vasodilator-stimulated phosphoprotein (VASP)–deficient mice