Barrett S F, Tarone R E, Moshell A N, Ganges M B, Robbins J H
J Invest Dermatol. 1981 Jan;76(1):59-62. doi: 10.1111/1523-1747.ep12524886.
In xeroderma pigmentosum, an inherited disorder of defective DNA repair, post-UV colony-forming ability of fibroblasts from patients in complementation groups A through F correlates with the patients' neurological status. The first xeroderma pigmentosum patient assigned to the recently discovered group G had the neurological abnormalities of XP. We have determined the post-UV colony-forming ability of cultured fibroblasts from this patient and from 5 more control donors. Log-phase fibroblasts were irradiated with 254 nm UV light from a germicidal lamp, trypsinized, and replated at known densities. After 2 to 4 weeks' incubation the cells were fixed, stained and scored for colony formation. The strains' post-UV colony-forming ability curves were obtained by plotting the log of the percent remaining post-UV colony-forming ability as a function of the UV dose. The post-UV colony-forming ability of 2 of the 5 new normal strains was in the previously defined control donor zone, but that of the other 3 extended down to the level of the most resistant xeroderma pigmentosum strain. The post-UV colony-forming ability curve of the group G fibroblasts was not significantly different from the curves of the group D fibroblast strains from patients with clinical histories similar to that of the group G patient.
在着色性干皮病（一种遗传性DNA修复缺陷疾病）中，A至F互补组患者的成纤维细胞经紫外线照射后的集落形成能力与患者的神经学状态相关。首例被归入最近发现的G组的着色性干皮病患者有XP的神经学异常表现。我们测定了该患者以及另外5名对照供体培养的成纤维细胞经紫外线照射后的集落形成能力。对数期的成纤维细胞用杀菌灯发出的254nm紫外线照射，用胰蛋白酶消化后，以已知密度重新接种。培养2至4周后，将细胞固定、染色并对集落形成进行计数。通过绘制经紫外线照射后剩余集落形成能力百分比的对数作为紫外线剂量的函数，获得各菌株经紫外线照射后的集落形成能力曲线。5株新的正常菌株中有2株经紫外线照射后的集落形成能力处于先前定义的对照供体范围内，但其他3株的该能力下降到了最具抗性的着色性干皮病菌株的水平。G组成纤维细胞经紫外线照射后的集落形成能力曲线与D组成纤维细胞菌株的曲线没有显著差异，D组患者的临床病史与G组患者相似。
