一种在人类白血病中具有组成性激酶活性的TEL-JAK2融合蛋白。
A TEL-JAK2 fusion protein with constitutive kinase activity in human leukemia.
作者信息
Lacronique V, Boureux A, Valle V D, Poirel H, Quang C T, Mauchauffé M, Berthou C, Lessard M, Berger R, Ghysdael J, Bernard O A
机构信息
U 301 de l'Institut National de la Santé et de la Recherche Médicale and SD 401 No. 301 CNRS, Institut de Génétique Moléculaire, 27 rue Juliette Dodu, 75010 Paris, France.
出版信息
Science. 1997 Nov 14;278(5341):1309-12. doi: 10.1126/science.278.5341.1309.
The Janus family of tyrosine kinases (JAK) plays an essential role in development and in coupling cytokine receptors to downstream intracellular signaling events. A t(9;12)(p24;p13) chromosomal translocation in a T cell childhood acute lymphoblastic leukemia patient was characterized and shown to fuse the 3' portion of JAK2 to the 5' region of TEL, a gene encoding a member of the ETS transcription factor family. The TEL-JAK2 fusion protein includes the catalytic domain of JAK2 and the TEL-specific oligomerization domain. TEL-induced oligomerization of TEL-JAK2 resulted in the constitutive activation of its tyrosine kinase activity and conferred cytokine-independent proliferation to the interleukin-3-dependent Ba/F3 hematopoietic cell line.
酪氨酸激酶（JAK）的Janus家族在发育过程以及细胞因子受体与下游细胞内信号转导事件的偶联中起着至关重要的作用。对一名儿童T细胞急性淋巴细胞白血病患者的t(9;12)(p24;p13)染色体易位进行了特征分析，结果显示JAK2的3'部分与TEL的5'区域融合，TEL是一个编码ETS转录因子家族成员的基因。TEL-JAK2融合蛋白包含JAK2的催化结构域和TEL特异性寡聚化结构域。TEL诱导的TEL-JAK2寡聚化导致其酪氨酸激酶活性的组成性激活，并赋予依赖白细胞介素-3的Ba/F3造血细胞系不依赖细胞因子的增殖能力。
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