Featherstone T, Taylor A M, Harnden D G
Am J Hum Genet. 1983 Jan;35(1):58-66.
No difference in survival was observed between cultured cells from basal cell naevus syndrome (BCNS) patients and normal controls following exposure of fibroblasts to ionizing radiation. Potential lethal damage repair in BCNS cells, measured by holding experiments, was also no different from normal. G0-irradiated lymphocytes from BCNS patients were found to have a significantly higher level of X-ray-induced chromosome aberrations compared with normals. This increase is, however, small, and, taken together with the survival data, suggests that increased cell killing as a measure of the unusual clinical radiosensitivity is not the major effect of the BCNS gene.
在将成纤维细胞暴露于电离辐射后,未观察到基底细胞痣综合征(BCNS)患者的培养细胞与正常对照之间在存活率上存在差异。通过维持实验测量,BCNS细胞中的潜在致死性损伤修复也与正常情况无异。与正常人相比,发现BCNS患者经G0期照射的淋巴细胞具有显著更高水平的X射线诱导染色体畸变。然而,这种增加幅度较小,结合存活率数据表明,作为衡量异常临床放射敏感性指标的细胞杀伤增加并非BCNS基因的主要作用。