Otsuka F, Watanabe R, Moro A, Ohkochi H, Ishibashi Y
Department of Dermatology, Faculty of Medicine, University of Tokyo.
Jpn J Cancer Res. 1989 Jan;80(1):41-4. 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.
汗孔角化症是一种常染色体显性遗传性皮肤病。其损害的特征为局限性异常角化,且可能发展为恶性肿瘤。为确定与这种皮肤病相关的癌症易感性的细胞基础,我们检测了来自汗孔角化症患者外观正常皮肤的经X射线或紫外线(UV)照射的培养成纤维细胞的集落形成能力。来自4名汗孔角化症患者皮肤的4株成纤维细胞对X射线的致死效应显著敏感。然而,它们对来自正常供体的细胞株对254nm紫外线的敏感性均相似。汗孔角化症患者培养的皮肤成纤维细胞对X射线辐射的超敏感性提示细胞DNA存在内在不稳定性,且可能与这种皮肤病的癌症易患性有关。