Summary
Tuberous sclerosis (TS) is an autosomal dominant neurocutaneous syndrome characterized by hamartomatous growth in multisystems. To determine the gene expression in cultured fibroblasts, we studied severalin vitro properties of skin fibroblasts. Measurements of the attachment ratio, the colony-forming efficiency, the population doubling time, and the saturation density in 10% fetal bovine serum show that fibroblasts from five TS patients proliferate in the same manner as do the age-matched controls. We also measured the survival of TS fibroblasts after treatment with a variety of chemical agents. TS fibroblasts are equal to controls in their sensitivity to four types of alkylating agents includingN-methyl-N′-nitro-N-nitrosoguanidine to which TS fibroblasts have been reported to be hypersensitive. These results suggest that thein vitro properties of TS fibroblasts do not resemble cells from patients with several autosomal recessive disorders predisposing to neoplasms and displaying hypersensitivity to DNA damages.
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Ohno, K., Takeshita, K. Patients with tuberous sclerosis have fibroblasts with normal limits for growth characteristics and sensitivities to DNA alkylating agents. Jap J Human Genet 29, 359–369 (1984). https://doi.org/10.1007/BF01871251
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DOI: https://doi.org/10.1007/BF01871251
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