Specific Features of p53 Protein Induction after Ionizing Radiation in Cells of Patients with Nijmegen Breakage Syndrome

Specific Features of p53 Protein Induction after Ionizing Radiation in Cells of Patients with... Synthesis of p53 and WAF1 (p21) proteins was studied in cells of patients with Nijmegen breakage syndrome (NBS) and of patients with ataxia telangiectasia (AT), as well as in normal cells with respect to their response to ionizing radiation (IR). In the NBS cells, the p53 protein was progressively accumulated with increasing radiation dose and reached the maximum 2 h after exposure to radiation at a dose of 5 Gy. The amount of p53 protein was consistently lower than that in normal cells, which was correlated with low content of the WAF1, the protein regulated by p53 at the level of transcription. Suboptimal induction of p53 observed in NBS cells was also characteristic of the AT cells, though the quantitative parameters of the protein synthesis in AT cells were intermediate relative to those in normal and NBS cells. In four NBS lines, the time schedule of p53 synthesis was similar to that observed in normal cells, whereas in AT cells, induction of p53 was significantly delayed as compared to control. In response to irradiation, the amount of p53 protein synthesized in patients with AT and NBS was significantly lower than that in normal cells. The results obtained, as well as the previously published medical and genetic evidence, suggest that the two diseases are of different origin and different genes are responsible for their development. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Russian Journal of Genetics Springer Journals

Specific Features of p53 Protein Induction after Ionizing Radiation in Cells of Patients with Nijmegen Breakage Syndrome

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Publisher
Kluwer Academic Publishers-Plenum Publishers
Copyright
Copyright © 2002 by MAIK “Nauka/Interperiodica”
Subject
Biomedicine; Human Genetics
ISSN
1022-7954
eISSN
1608-3369
D.O.I.
10.1023/A:1016352007643
Publisher site
See Article on Publisher Site

Abstract

Synthesis of p53 and WAF1 (p21) proteins was studied in cells of patients with Nijmegen breakage syndrome (NBS) and of patients with ataxia telangiectasia (AT), as well as in normal cells with respect to their response to ionizing radiation (IR). In the NBS cells, the p53 protein was progressively accumulated with increasing radiation dose and reached the maximum 2 h after exposure to radiation at a dose of 5 Gy. The amount of p53 protein was consistently lower than that in normal cells, which was correlated with low content of the WAF1, the protein regulated by p53 at the level of transcription. Suboptimal induction of p53 observed in NBS cells was also characteristic of the AT cells, though the quantitative parameters of the protein synthesis in AT cells were intermediate relative to those in normal and NBS cells. In four NBS lines, the time schedule of p53 synthesis was similar to that observed in normal cells, whereas in AT cells, induction of p53 was significantly delayed as compared to control. In response to irradiation, the amount of p53 protein synthesized in patients with AT and NBS was significantly lower than that in normal cells. The results obtained, as well as the previously published medical and genetic evidence, suggest that the two diseases are of different origin and different genes are responsible for their development.

Journal

Russian Journal of GeneticsSpringer Journals

Published: Oct 13, 2004

References

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