JAK2异常在血液病发病机制中的研究进展  

Advances in the Research of JAIG Abnormalities in Heamatological Disorders

作　　者：任艳玲[1] 佟红艳[1] 

机构地区：[1]浙江大学医学院附属第一医院血液病研究所,杭州310003

出　　处：《国际遗传学杂志》2008年第5期365-368,共4页International Journal of Genetics

摘　　要：JAK2是非受体酪氨酸激酶Janus家族一员，通过与多种细胞因子特别是造血因子（EPO、TPO、G-CSF）受体胞内端偶联参与细胞内信号转导，调节血细胞的生成，分化与成熟。它既可以因染色体易位形成融合基因编码融合蛋白参与恶性血液病也可因突变使激酶活性增强引起髓系增殖性疾病。近年来，国内外学者对此做了大量研究并取得突破性进展，现对此作一简要的综述。JAK2 is a member of non-receptor tyrosine kinase family, which participate in cell signaling by associating with intracellular part of the eytokine receptor, especially hematopoiesis factor receptor, thus regulating the genesis, differention and maturation of the blood cells. The abnormally activated JAK2 by chromosomal translocation lead to fusion genes generally resulting in novel chimeric proteins or aeequaired activating muta- tion can enhance the cell signaling and invole in the pathophysiology of haematological malignancy. Recently, seholors at home and abroad have done a lot of researches and have made breakthrough. This paper introduces the progress of JAK2 in the pathogenesis of the hematological malignancy.

关 键 词：JAK2 突变 融合蛋白 骨髓增殖性疾病 

分 类 号：R733[医药卫生—肿瘤] R733.704[医药卫生—临床医学]