Fornace A J, Little J B, Weichselbaum R R
Biochim Biophys Acta. 1979 Jan 26;561(1):99-109. doi: 10.1016/0005-2787(79)90494-5.
DNA repair and colony survival were measured in fibroblasts from a patient with Fanconi's anemia, HG 261, and from normal human donors after exposure of these cells to the cross-linking agent mitomycin C, X-rays or ultraviolet light. Survival was similar in HG 261 and normal cells after X-ray or ultraviolet radiation, but was reduced in the Fanconi's anemia cells after treatment with mitomycin C. The level of DNA cross-linking, as measured by the method of alkaline elution, was the same in both cell strains after exposure to various doses of mitomycin C. With incubation after drug treatment, a gradual decrease in the amount of cross-linking was observed; the rate of this apparent repair of cross-link damage was the same in both normal and HG 261 cells. The rejoining of DNA single strand breaks after X-irradiation and the production of excision breaks after ultraviolet radiation were also normal in HG 261 cells as determined by alkaline elution.
在将来自一名范可尼贫血患者（HG 261）和成正常人供体的成纤维细胞暴露于交联剂丝裂霉素C、X射线或紫外线后，对DNA修复和集落存活情况进行了检测。在接受X射线或紫外线辐射后，HG 261细胞和正常细胞的存活情况相似，但在用丝裂霉素C处理后，范可尼贫血细胞的存活率降低。通过碱性洗脱法测定，在暴露于不同剂量的丝裂霉素C后，两种细胞系中的DNA交联水平相同。药物处理后进行孵育，观察到交联量逐渐减少；在正常细胞和HG 261细胞中，这种交联损伤的明显修复速率相同。通过碱性洗脱法测定，在HG 261细胞中，X射线照射后DNA单链断裂的重新连接以及紫外线辐射后切除断裂的产生也均正常。
