Study of Basal Cell Nevus Syndrome Fibroblasts after Treatment with DNA-Damaging Agents
Basal cell nevus syndrome (BCNS) is a rare autosomal dominant inherited disorder (1). About 20% of gene carriers develop brain tumors in early childhood. Basal cell nevus syndrome patients are abnormally susceptible to radiation-induced cancer; several patients treated with radio-therapeutic doses have developed large numbers of basal cell tumors in the irradiated field within 6 mo to 3 yr of ex posure (2-5). Featherstone et al. (6) reported that fibroblasts from BCNS patients showed no increased susceptibility to X-ray-induced cell killing; however, GO-irradiated lymphocytes from BCNS patients were found to have a significantly higher level of X-ray-induced chromosomal aberrations (CAs) compared with normal cells. On the other hand, Chan and Little (7) reported that fibroblasts from BCNS patients were slightly hypersensitive to X-ray-induced lethality.
KeywordsSister Chromatid Exchange Ataxia Telangiectasia Ataxia Telangiectasia Confluent Culture Human Diploid Fibroblast
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- 1.Gorlin, R.J., and H.O. Sedano (1976) Cancer of the Skin, A. Andrade, S.L. Gumport, G.L. Popkin, and T.D. Rees, eds. W.B. Sanders, Philadelphia, pp. 883–898.Google Scholar
- 3.Strong, L.C. (1977) The Genetics of Human Cancer, J.J. Malvihill, R.W. Miller, and J.F. Fraumeni, Jr., eds. Raven Press, New York, pp. 401–414.Google Scholar
- 6.Featherstone, T., A.M.R. Taylor, and D.G. Harnden (1983) Studies on the radiosensitivity of cells from patients with Basal Cell Naevus Syndrome. Am. J. Human Genet. 35:58–66.Google Scholar
- 8.Friedberg, E.C., U.K. Ehman, and J.I. Williams (1979) Advances in Radiation Biology, Vol. 8, J.T. Lett, H. Adler, and M. Zelle, eds. Academic Press, New York, pp. 85–174.Google Scholar
- 10.Kohn, K.W., R.A.G. Ewig, L.C. Erickson, and L.A. Zwellig (1981) DNA Repair, Vol. 1, part B, E.G. Frieberg and P.C. Hanawalt, eds. Marcel Decker, New York and Basel, pp. 379–401.Google Scholar
- 11.Formace, A.J., Jr., and J.B. Little (1979) DNA-protein cross-linking by chemical carcinogens in mammalian cells. Cancer Res. 39:704–710.Google Scholar
- 20.Nagasawa, H., S. Latt, and J.B. Little (1983) Effects of xirradiation on cell cycle progression, induction of chromosomal aberrations and cell killing in homozygotic and heterozygotic ataxia-telagiectasia. Radiat. Res. 94:632–633.Google Scholar
- 22.Nagasawa, H., A.J. Fornace, Jr., M.R. Ritter, and J.B. Little (1982) Relationship of enhanced survival during confluent holding recovery in ultraviolet-irradiated human and mouse cells to chromosome aberrations, sister chromatid exchanges, and DNA repair. Radiat. Res. 92:483–496.PubMedCrossRefGoogle Scholar
- 23.Nagasawa, H., A.J. Fornace, Jr., J.B. Little, and J.R. Williams (1977) Potentially lethal damage repair:relationship to the induction of sister chromatid exchanges and DNA repair capacity. Radiat. Res. 70:706.Google Scholar