Notch 1 activation in the molecular pathogenesis of T-cell acute lymphoblastic leukaemia

C Grabher, H von Boehmer, AT Look - Nature Reviews Cancer, 2006 - nature.com
C Grabher, H von Boehmer, AT Look
Nature Reviews Cancer, 2006nature.com
Abstract The chromosomal translocation t (7; 9) in human T-cell acute lymphoblastic
leukaemia (T-ALL) results in deregulated expression of a truncated, activated form of Notch
1 (TAN1) under the control of the T-cell receptor-β (TCRB) locus. Although TAN1 efficiently
induces T-ALL in mouse models, t (7; 9) is present in less than 1% of human T-ALL cases.
The recent discovery of novel activating mutations in NOTCH1 in more than 50% of human T-
ALL samples has made it clear that Notch 1 is far more important in human T-ALL …
Abstract
The chromosomal translocation t(7;9) in human T-cell acute lymphoblastic leukaemia (T-ALL) results in deregulated expression of a truncated, activated form of Notch 1 (TAN1) under the control of the T-cell receptor-β (TCRB) locus. Although TAN1 efficiently induces T-ALL in mouse models, t(7;9) is present in less than 1% of human T-ALL cases. The recent discovery of novel activating mutations in NOTCH1 in more than 50% of human T-ALL samples has made it clear that Notch 1 is far more important in human T-ALL pathogenesis than previously suspected.
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