Reduced oncogenicity of p190 Bcr/Abl F-actin-binding domain mutants

Blood. 2000 Sep 15;96(6):2226-32.

Abstract

The deregulated Bcr/Abl tyrosine kinase is responsible for the development of Philadelphia (Ph)-positive leukemia in humans. To investigate the significance of the C-terminal Abl actin-binding domain within Bcr/Abl p190 in the development of leukemia/lymphoma in vivo, mutant p190 DNA constructs were used to generate transgenic mice. Eight founder and progeny mice of 5 different lines were monitored for leukemogenesis. Latency was markedly increased and occurrence decreased in the p190 del C lines as compared with nonmutated p190 BCR/ABL transgenics. Western blot analysis of involved hematologic tissues of the p190 del C transgenics with end-stage disease showed high-level expression of the transgene and tyrosine phosphorylation of Cbl and Hef1/Cas, proteins previously shown to be affected by Bcr/Abl. These results show that the actin-binding domain of Abl enhances leukemia development but does not appear to be an absolute requirement for leukemogenesis.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Actins / genetics
  • Actins / metabolism
  • Animals
  • Binding Sites
  • Cell Transformation, Neoplastic / genetics*
  • Fusion Proteins, bcr-abl / genetics*
  • Fusion Proteins, bcr-abl / metabolism
  • Humans
  • Leukemia, Experimental / genetics*
  • Leukemia, Experimental / metabolism
  • Leukemia, Experimental / pathology*
  • Mice
  • Mice, Transgenic
  • Mutation*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / metabolism
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology
  • Protein Binding

Substances

  • Actins
  • Fusion Proteins, bcr-abl