Radiation induction of p53 in cells from Nijmegen breakage syndrome is defective but not similar to ataxia-telangiectasia.

@article{Matsuura1998RadiationIO,
  title={Radiation induction of p53 in cells from Nijmegen breakage syndrome is defective but not similar to ataxia-telangiectasia.},
  author={K Matsuura and Timur S. Balmukhanov and Hiroshi Tauchi and C. Weemaes and Dominique F Smeets and Krystyna H. Chrzanowska and Shigehito Endou and Shinya Matsuura and Kenshi Komatsu},
  journal={Biochemical and biophysical research communications},
  year={1998},
  volume={242 3},
  pages={
          602-7
        }
}
p53-mediated signal transduction after exposure to ionizing radiation was examined in cells from patients with Nijmegen breakage syndrome (NBS), an autosomal recessive disease characterized by microcephaly, immunodeficiency, predisposition to malignancy, and a high sensitivity to ionizing radiation. NBS cells accumulated p53 protein in a dose-dependent fashion, with a peak level 2 hrs after irradiation with 5 Gy. However, the maximal level of p53 protein in NBS cells was constantly lower than… CONTINUE READING

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