Cultured Skin Fibroblasts from Patients with Porokeratosis Are Hypersensitive to the Lethal Effects of X‐radiation

• Published in: Cancer science 1989-01, Vol.80 (1), p.41-44
• Main Authors: Otsuka, Fujio, Watanabe, Ryohji, Moro, Akiko, Ohkochi, Hitoshi, Ishibashi, Yasumasa
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Cancer‐prone genodermatosis; Cell Survival - radiation effects; Cells, Cultured; Chromosome Aberrations; Dermatology; DNA Damage; DNA‐damaging agent; Fibroblasts; Fibroblasts - radiation effects; Hereditary diseases; Hereditary diseases of the skin. Congenital diseases of the skin. Haemangioma of the skin, of mucosae and of soft tissue; Humans; Hypersensitivity; Keratinization; Keratosis - complications; Keratosis - genetics; Medical sciences; Porokeratosis; Skin - radiation effects; Skin diseases; Skin Neoplasms - etiology; Tumors; Ultraviolet radiation; Ultraviolet Rays; X-Rays; X‐radiation
• ISSN: 0910-5050; 1347-9032; 1349-7006; 1876-4673
• DOI: 10.1111/j.1349-7006.1989.tb02242.x

Porokeratosis is an autosomal dominant inherited skin disorder. The lesions are characterized by localized abnormal keratinization and may develop into malignant tumors. To determine the cellular basis of the cancer susceptibility associated with this skin condition, we examined the colony‐forming ability of X‐ray or ultraviolet (UV) light irradiated, cultured fibroblasts derived from porokeratosis patients' normal‐appearing skin. Four fibroblast strains derived from four porokeratosis patients' skin were significantly hypersensitive to the lethal effects of X‐radiation. However, they all showed a similar sensitivity to strains from normal donors to 254 nm UV light. The hypersensitivity to X‐ray radiation in cultured skin fibroblasts from porokeratosis patients suggests an inherent instability of cellular DNA and may probably be associated with the cancer‐prone nature of this skin condition.