Braun Benjamin S, Archard Joehleen A, Van Ziffle Jessica A G, Tuveson David A, Jacks Tyler E, Shannon Kevin
Department of Pediatrics, HSE-302, University of California-San Francisco, 513 Parnassus Avenue, San Francisco, CA 94143, USA.
Blood. 2006 Sep 15;108(6):2041-4. doi: 10.1182/blood-2006-01-013490. Epub 2006 May 23.
Somatic activation of a conditional targeted Kras(G12D) allele induces a fatal myeloproliferative disease in mice that closely models juvenile and chronic myelomonocytic leukemia. These mice consistently develop severe and progressive anemia despite adequate numbers of clonogenic erythroid progenitors in the bone marrow and expanded splenic hematopoiesis. Ineffective erythropoiesis is characterized by impaired differentiation. These results demonstrate that endogenous levels of oncogenic Ras have cell lineage-specific effects and support efforts to modulate Ras signaling for therapy of anemia in patients with myelodysplastic syndromes and myeloproliferative disorders.
条件性靶向Kras(G12D)等位基因的体细胞激活在小鼠中诱发一种致命的骨髓增殖性疾病,该疾病与青少年和慢性粒单核细胞白血病极为相似。尽管骨髓中克隆形成性红系祖细胞数量充足且脾造血功能增强,但这些小鼠仍持续出现严重且进行性的贫血。无效红细胞生成的特征是分化受损。这些结果表明,致癌性Ras的内源性水平具有细胞谱系特异性作用,并支持为治疗骨髓增生异常综合征和骨髓增殖性疾病患者的贫血而调节Ras信号传导的努力。