Abstract
There are people in the population who show an unusual sensitivity to ionizing radiation compared with normal individuals. This effect has been demonstrated in the course of bona fide radiotherapy for malignant conditions in these patients with the result proving either lethal to the patient or subsequently giving rise to tumors in the irradiated field. These individuals are members of a small group with inherited disorders showing a predisposition to malignant disease. Although some attempts have been made to study the variation in radiosensitivity in normal members of the population (Weichselbaum et al., 1976) most work has been done on the inherited disorders. The underlying causes of the different responses to radiation are at present not understood, but it seems likely that more than a single factor is important. In ataxia telangiectasia, for example, particular tumor types are associated with radiation sensitivity in these patients. This suggests that particular cell types or differentiation states are more susceptible to the consequences of the possible repair deficiency in these patients.
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References
Arlett, C. F., Presymptomatic diagnosis of Huntington’s disease, Lancet (i), 540 (1980).
Bigelow, S. B., Rary, J. M., and Bender, M. A., G2 chromosomal radiosensitivity in Fanconi’s anaemia, Mutation Res., 63, 189–199 (1979).
Chen, P., Lavin, M. F., Kidson, C., and Moss, D., Identification of ataxia telangiectasia heterozygotes, a cancer prone population, Nature, 274, 484–486 (1978).
Chen, P., Kidson, C., and Imray, F. P., Huntington’s chorea: implications of associated cellular radiosensitivity, Clin. Genet., 20, 331–336 (1981).
Countryman, P. I., Heddle, J. A., and Crawford, E., The repair of x-ray induced chromosomal damage in trisomy 21 and normal diploid lymphocytes, Cancer Res., 37, 52–58 (1977).
Cox, R., A cellular description of the repair defect in ataxia telangiectasia, in: B. A. Bridges, D. G. Harnden, eds., Ataxia telangiectasia–A cellular and molecular link between cancer, neuropathology and immune deficiency, pp. 141–153, John Wiley and Sons, Ltd. (1982).
Cramer, P., and Painter, R. B., Bleomycin resistant DNA synthesis in ataxia telangiectasia cells, Nature, 291, 671–672 (1981).
Cunliffe, P. N., Mann, J. R., Cameron, A. H., Roberts, K. O., and Ward, H. W. C., Radiosensitivity in ataxia telangiectasia, Brit. J. Radiol., 48, 374–376 (1975).
Edwards, M. J., and Taylor, A. M. R., Unusual levels of (ADP-ribose)n and DNA synthesis in ataxia telangiectasia cells following yirradiation, Nature, 287, 745–747 (1980).
Edwards, M. J., Taylor, A. M. R., and Flude, E. J., Bleomycin induced inhibition of DNA synthesis in ataxia telangiectasia cell lines, Biochem. Biophys. Res. Commun., 102, 610–616 (1981).
Featherstone, T., A study of cultured cells from basal cell naevus syndrome patients, Ph.D. Thesis, University of Birmingham, England (1981).
Gorlin, R. J., and Sedano, H. O., The multiple naevoid basal cell carcinoma syndrome revisited, Birth Defects, 7 (8), 140–148 (1972).
Gotoff, S. P., Amirmokri, E., and Liebner, E. J., Ataxia telangiectasia: Neoplasia, untoward response to x-irradiation and tuberous sclerosis, Am. J. Dis. Child., 114, 617–625 (1967).
Harnden, D. G., Edwards, M. J., Featherstone, T., Morten, J., Morgan, G. R., and Taylor, A. M. R., Studies on cells from patients who are cancer prone and may be radiosensitive, in: H. V. Gelboin, B. MacMahon, T. Mutsushima, T. Sugimura, S. Takayama, and H. Takebe, eds., Genetic and Environmental Factors in Experimental and Human Cancer, pp. 231–246, Tokyo: Japan Scientific Societies Press (1980).
Houldsworth, J., and Lavin, M. F., Effect of ionizing radiation on DNA synthesis in ataxia telangiectasia cells, Nucleic Acids Res., 8, 3709–3720 (1980).
Inoue, T., Hirano, K., Yokoiyama, A., Kada, T., and Kato, H., DNA repair enzymes in ataxia telangiectasia and Bloom’s syndrome fibroblasts, Biochim. Biophys. Acta, 479, 497–500 (1977).
Jaspers, N. G. J., de Wit, J., Regulski, M. R., and Bootsma, D., Abnormal regulation of DNA replication and increased lethality in ataxia telangiectasia cells exposed to carcinogenic agents, Cancer Res., 42, 335–341 (1982).
Kidson, C., Chen, P., and Imray, P., Ataxia telangiectasia heterozygotes: dominant expression of ionizing radiation sensitive mutants, in: B. A. Bridges and D. G. Harnden, eds., Ataxia telangiectasia - A cellular and molecular link between cancer, neuropathology and immune deficiency, pp. 363–372, John Wiley and Sons (1982).
Kucerova, M., Comparison of radiation effects in vitro upon chromosomes of human subjects, Acta Radiol., 6, 441–448 (1967).
Lehmann, A. R., and Stevens, S., The response of ataxia telangiectasia cells to bleomycin, Nucleic Acids Res., 6, 1953–1960 (1979).
Morten, J. E. N., Harnden, D. G., and Taylor, A. M. R., Chromosome damage in Go x-irradiated lymphocytes from patients with hereditary retinoblastoma, Cancer Res., 41, 3635–3638 (1981).
Morgan, J. L., Holcomb, T. M., and Morrissey, R. W., Radiation reaction in ataxia telangiectasia, Am. J. Dis. Child., 116, 557–558 (1968).
Moshell, A. N., Tarone, R. E., Barratt, S. F., and Robbins, J. H., Radiosensitivity in Huntingston’s disease: implications for pathogenesis and presymptomatic diagnosis, Lancet (i), 9–11 (1980).
Natarajan, A. T., and Meyers, M., Chromosomal radiosensitivity of ataxia telangiectasia cells at different cell cycle stages, Hum. Genet., 52, 127–132 (1979).
Painter, R. B., and Young, B. R., Radiosensitivity in ataxia telangiectasia: a new explanation, Proc. Natl. Acad. Sci. USA, 77, 7315–7317 (1980).
Paterson, M. C., Smith, B. P., Lohmann, P. H. M., Anderson, A. K., and Fishman, L., Defective excision repair of gamma ray damaged DNA in human (ataxia telangiectasia) fibroblasts, Nature, 260, 444–447 (1976).
Remsen, J. F., and Cerutti, P. A., Deficiency of gamma-ray excision repair in skin fibroblasts from patients with Fanconi’s anaemia, Proc. Natl. Acad. Sci. USA, 73, 2419–2423 (1976).
Strong, L. C., Theories of pathogenesis: mutation and cancer, in: J. J. Mulvihill, R. W. Miller, and J. F. Fraumeni, eds., Genetics of human cancer, pp. 401–415, Raven Press (1977).
Swift, M., Sholman, L., Perry, M., and Chase, C., Malignant neoplasms in the families of patients with ataxia telangiectasia, Cancer Res., 36, 209–215 (1976).
Swift, M., Disease predisposition of ataxia telangiectasia heterozygotes, in: B. A. Bridges and D. G. Harnden, eds., Ataxia telangiectasia - A cellular and molecular link between cancer, neuropathology and immune deficiency, pp. 355–361, John Wiley and Sons (1982).
Taylor, A. M. R., Unrepaired DNA strand breaks in irradiated ataxia telangiectasia lymphocytes suggested from cytogenetic observations, Mutation Res., 50, 407–418 (1978).
Taylor, A. M. R., Cytogenetics of ataxia telangiectasia, in: B. A. Bridges and D. G. Harnden, eds., Ataxia telangiectasia — A cellular and molecular link between cancer, neuropathology, and immune deficiency, pp. 53–81, John Wiley and Sons (1982).
Taylor, A. M. R., Harnden, D. G., Arlett, C. F., Harcourt, S. A., Lehmann, A. R., Stevens, S., and Bridges, B. A., Ataxia telangiectasia: a human mutation with abnormal radiation sensitivity, Nature, 258, 427–429 (1975).
Taylor, A. M. R., Metcalfe, J. A., Oxford, J. M., and Harnden, D. G., Is chromatid type damage in ataxia telangiectasia after irradiation at Go a consequence of defective repair? Nature, 260, 441–443 (1976).
Taylor, A. M. R., Rosney, C. M., and Campbell, J. B., Unusual sensitivity of ataxia telangiectasia cells to bleomycin, Cancer Res., 39, 1046–1050 (1979).
Weichselbaum, R. R., Epstein, J., and Little, J. B., In vitro radiosensitivity of human diploid fibroblasts derived from patients with unusual clinical responses to radiation, Radiology, 121, 479–482 (1976).
Weichselbaum, R. R., Nove, J., and Little, J. B., Deficient recovery from potentially lethal radiation damage in ataxia telangiectasia and xeroderma pigmentosum, Nature, 271, 261–262 (1978).
de Wit, J., Jaspers, N. G. J., and Bootsma, D., The rate of DNA synthesis in normal human and ataxia telangiectasia cells after exposure to ionizing radiation, Mutation Res., 80, 221–226 (1981).
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Taylor, A.M.R. (1983). Cellular Studies on Patients with an Unusual Clinical Sensitivity to Ionizing Radiation. In: Castellani, A. (eds) The Use of Human Cells for the Evaluation of Risk from Physical and Chemical Agents. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-1117-2_21
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DOI: https://doi.org/10.1007/978-1-4757-1117-2_21
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