Tarone R E, Otsuka F, Robbins J H
J Neurol Sci. 1984 Sep;65(3):367-81. doi: 10.1016/0022-510x(84)90099-6.
Hypersensitivity to the lethal effects of DNA-damaging agents is usually demonstrated using the classical colony-forming ability assay with cultured fibroblast lines. Based on the ability of viable cells in lymphoblastoid lines (Epstein-Barr virus-transformed B lymphocytes) to exclude the vital dye trypan blue, we have developed a more rapid survival assay which has been useful in detecting hypersensitivity to ionizing radiation in certain diseases characterized by primary degeneration of excitable tissue. We now present a complete description of this post-X-ray survival assay. We also demonstrate the suitability of both our assay and our method of data analysis for detecting hypersensitivity to ionizing radiation. This demonstration is based on a detailed analysis of assay results with lymphoblastoid lines from 28 normal donors, 3 ataxia telangiectasia (AT) patients, 2 obligate AT heterozygotes, 7 patients with diseases characterized by cellular hypersensitivity to ultraviolet radiation (UV), and 10 Duchenne muscular dystrophy (DMD) patients.
通常使用培养的成纤维细胞系的经典集落形成能力测定法来证明对DNA损伤剂致死效应的超敏反应。基于淋巴母细胞系（爱泼斯坦-巴尔病毒转化的B淋巴细胞）中活细胞排除活性染料台盼蓝的能力，我们开发了一种更快速的存活测定法，该方法在检测某些以可兴奋组织原发性变性为特征的疾病中对电离辐射的超敏反应方面很有用。我们现在给出这种X射线后存活测定法的完整描述。我们还证明了我们的测定法和数据分析方法对于检测对电离辐射的超敏反应的适用性。这一证明基于对来自28名正常供体、3名共济失调毛细血管扩张症（AT）患者、2名AT obligate杂合子、7名以细胞对紫外线（UV）超敏为特征的疾病患者以及10名杜兴氏肌营养不良症（DMD）患者的淋巴母细胞系的测定结果的详细分析。
