Peng Cong, Li Shaoguang
Division of Hematology & Oncology, Department of Medicine, University of Massachusetts Medical School, Worcester, MA, USA.
Methods Mol Biol. 2010;602:253-66. doi: 10.1007/978-1-60761-058-8_15.
Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by increased proliferation of granulocytic cells without the loss of their capability to differentiate. CML is derived from the hematopoietic stem cells (1) with the Philadelphia chromosome resulting from of a reciprocal translocation between the chromosomes 9 and 22 t(9;22)-(q34;q11). This translocation produces a fusion gene known as BCR-ABL which acquires uncontrolled tyrosine kinase activity, constantly turning on its downstream signaling molecules/pathways, and promoting proliferation of leukemia cell through anti-apoptosis and acquisition of additional mutations. To evaluate the role of each critical downstream signaling molecule of BCR-ABL and test therapeutic drugs in vivo, it is important to use physiological mouse disease models. In this chapter, we describe a mouse model of CML induced by BCR-ABL retrovirus (MSCV-BCR-ABL-GFP; MIG-BCR-ABL) and how to use this model in translational research.
慢性髓性白血病(CML)是一种骨髓增殖性疾病,其特征是粒细胞增殖增加,且不丧失分化能力。CML起源于造血干细胞(1),伴有9号和22号染色体之间相互易位产生的费城染色体t(9;22)-(q34;q11)。这种易位产生一种称为BCR-ABL的融合基因,该基因获得不受控制的酪氨酸激酶活性,持续激活其下游信号分子/通路,并通过抗凋亡和获得额外突变促进白血病细胞增殖。为了评估BCR-ABL每个关键下游信号分子的作用并在体内测试治疗药物,使用生理性小鼠疾病模型很重要。在本章中,我们描述了一种由BCR-ABL逆转录病毒诱导的CML小鼠模型(MSCV-BCR-ABL-GFP;MIG-BCR-ABL)以及如何在转化研究中使用该模型。