弗里德赖希共济失调中细胞对电离辐射的超敏反应研究。
Studies of cellular hypersensitivity to ionising radiation in Friedreich's ataxia.
作者信息
Chamberlain S, Lewis P D
出版信息
J Neurol Neurosurg Psychiatry. 1982 Dec;45(12):1136-8. doi: 10.1136/jnnp.45.12.1136.
Abstract
Skin fibroblasts from seven patients with Friedreich's ataxia showed a small but significant increase in sensitivity to ionising radiation, as measured by post-irradiation clonal growth, when compared with cells from ten age-matched control subjects and from eight patients with motor neuron disease. Fibroblasts from three patients with Friedreich's ataxia also showed impairment of their ability to repair potentially lethal damage after ionising radiation. These findings are consistent with the view that defective DNA reparative mechanisms may be involved in the pathogenesis of Friedreich's ataxia.
摘要
与来自10名年龄匹配的对照受试者以及8名运动神经元疾病患者的细胞相比，通过照射后克隆生长测定，7名弗里德赖希共济失调患者的皮肤成纤维细胞对电离辐射的敏感性有小幅但显著的增加。3名弗里德赖希共济失调患者的成纤维细胞在电离辐射后修复潜在致死性损伤的能力也受损。这些发现与以下观点一致，即有缺陷的DNA修复机制可能参与了弗里德赖希共济失调的发病机制。
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