A TEL-JAK2 fusion protein with constitutive kinase activity in human leukemia

V Lacronique, A Boureux, V Della Valle, H Poirel… - Science, 1997 - science.org
V Lacronique, A Boureux, V Della Valle, H Poirel, CT Quang, M Mauchauffé, C Berthou
Science, 1997science.org
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 …
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 ofJAK2 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.
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