ZFIN ID: ZDB-PUB-091204-3
Notch 1 activation in the molecular pathogenesis of T-cell acute lymphoblastic leukaemia
Grabher, C., von Boehmer, H., and Look, A.T.
Date: 2006
Source: Nature reviews. Cancer   6(5): 347-359 (Review)
Registered Authors: Grabher, Clemens, Look, A. Thomas
Keywords: none
MeSH Terms:
  • Gene Expression Regulation, Leukemic*
  • Humans
  • Leukemia-Lymphoma, Adult T-Cell/metabolism*
  • Receptor, Notch1/genetics
  • Receptor, Notch1/metabolism*
  • Signal Transduction
PubMed: 16612405 Full text @ Nat. Rev. Cancer
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-beta (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.