Development of ET, primary myelofibrosis and PV in mice expressing JAK2 V617F

摘要: An acquired JAK2 V617F mutation is found in most patients with polycythemia vera (PV), and about half of essential thrombocythemia (ET) or primary myelofibrosis (PMF). Mice transplanted bone marrow cells which was retrovirally expressed developed PV-like features, but not ET PMF. To address the contribution this to pathogenesis these three MPDs, we generated two lines transgenic mice. One line showed granulocytosis after 4 months age. Among 43 mice, 8 (19%) 15 (35%) thrombocythemia. The second extreme leukocytosis thromobocytosis. They anemia that means Hb value from 9 10 g per 100 ml when 1 month old. Myeloid megakaryocytes were predominant animals, splenomegaly observed. expression mRNA 0.45 1.35 endogenous wild-type lines, respectively. In vitro analysis both constitutive activation ERK1/2, STAT5 AKT, augmentation their phosphorylations by cytokine stimulation. We conclude vivo results ET-, PMF- disease.