X-Ray, UV and Chemical Mutagen Sensitivities of Skin Fibroblasts from Patients with Familial and Chromosome 13q-Retinoblastomas

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Retinoblastoma (RB), a malignant eye tumor, occurs sporadically and in genetically predisposed children1). The latter consists of the two groups of patients who inherit RB with an autosomal dominant mode and bear a specific chromosomal deletion of the long arm of chromosome 13 (13q14). Hereditary RB patients also have a risk for an increased incidence of other cancers and radiation-induced tumors in the fields of radiotherapy Weichselbaum et al. first showed that AG1142 [46XX, del(13)(q14-22)] fibroblasts exhibited a high sensitivity to X-ray killing (extrapolation number (n)=1.41, mean lethal dose (D_0)=94 rad), which was intermediate between those of radiation-sensitive ataxia telangiectasia (ATCC CRL1343:n=1.39, Do=46 rad) and normal (n=1.44, Do=149 rad). Arlett and Harcourt6) have also supported Weichselbaum et al. However, such a high X-ray sensitivity is not necessarily observed in all fibroblast strains from patients with 13q-RB.
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