帕金森病和阿尔茨海默病：培养细胞系对X射线的超敏反应。
Parkinson's disease and Alzheimer's disease: hypersensitivity to X rays in cultured cell lines.
作者信息
Robbins J H, Otsuka F, Tarone R E, Polinsky R J, Brumback R A, Nee L E
出版信息
J Neurol Neurosurg Psychiatry. 1985 Sep;48(9):916-23. doi: 10.1136/jnnp.48.9.916.
Abstract
Fibroblast and/or lymphoblastoid lines from patients with several inherited primary neuronal degenerations are hypersensitive to DNA-damaging agents. Therefore, lymphoblastoid lines were irradiated from patients with sporadic Parkinson's disease (PD), Alzheimer's disease, and amyotrophic lateral sclerosis. The mean survival values of the eight Parkinson's disease and of the six Alzheimer's disease lines, but not of the five amyotrophic lateral sclerosis lines, were less than that of the 28 normal lines. Our results with Parkinson's disease and Alzheimer's disease cells can be explained by a genetic defect arising as a somatic mutation during embryogenesis, causing defective repair of the X-ray type of DNA damage. Such a DNA repair defect could cause an abnormal accumulation of spontaneously occurring DNA damage in Parkinson's disease and Alzheimer's disease neurons in vivo, resulting in their premature death.
摘要
来自患有几种遗传性原发性神经元退行性疾病患者的成纤维细胞系和/或淋巴母细胞系对DNA损伤剂高度敏感。因此，对散发性帕金森病（PD）、阿尔茨海默病和肌萎缩侧索硬化症患者的淋巴母细胞系进行了辐射。8个帕金森病细胞系和6个阿尔茨海默病细胞系的平均存活值低于28个正常细胞系，但5个肌萎缩侧索硬化症细胞系的平均存活值并不低于正常细胞系。我们对帕金森病和阿尔茨海默病细胞的研究结果可以用胚胎发生过程中作为体细胞突变出现的基因缺陷来解释，这种缺陷导致对X射线类型的DNA损伤修复缺陷。这种DNA修复缺陷可能导致帕金森病和阿尔茨海默病神经元在体内自发发生的DNA损伤异常积累，从而导致它们过早死亡。
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