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来自患有尤塞氏综合征、杜兴氏肌肉营养不良症和亨廷顿舞蹈症患者的成纤维细胞系的辐射敏感性。

Radiation sensitivity of fibroblast strains from patients with Usher's syndrome, Duchenne muscular dystrophy, and Huntington's disease.

作者信息

Nove J, Tarone R E, Little J B, Robbins J H

出版信息

Mutat Res. 1987 Jul;184(1):29-38. doi: 10.1016/0167-8817(87)90033-2.

DOI:10.1016/0167-8817(87)90033-2
PMID:2955220
Abstract

The colony-forming ability of 10 normal human fibroblast cell strains and of 10 strains representing 3 degenerative diseases of either nerve or muscle cells was determined after exposure of the cells to X-rays or beta-particles from tritiated water. Both methods of irradiation yielded similar comparative results. The fibroblast strains from the 5 Usher's syndrome patients and from 1 of the 2 Huntington's disease patients were hypersensitive to radiation, while those from the 3 Duchenne muscular dystrophy patients and the second Huntington's disease patient had normal sensitivity to radiation. These results indicate both disease-specific and strain-specific differences in the survival of fibroblasts after exposure to ionizing radiation.

摘要

在将10种正常人类成纤维细胞系以及代表神经或肌肉细胞3种退行性疾病的10种细胞系暴露于X射线或氚化水产生的β粒子后,测定了它们的集落形成能力。两种辐照方法产生了相似的比较结果。来自5名Usher综合征患者和2名亨廷顿舞蹈病患者中1人的成纤维细胞系对辐射高度敏感,而来自3名杜氏肌营养不良患者和另1名亨廷顿舞蹈病患者的成纤维细胞系对辐射具有正常敏感性。这些结果表明,暴露于电离辐射后,成纤维细胞的存活存在疾病特异性和细胞系特异性差异。

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Radiation sensitivity of fibroblast strains from patients with Usher's syndrome, Duchenne muscular dystrophy, and Huntington's disease.来自患有尤塞氏综合征、杜兴氏肌肉营养不良症和亨廷顿舞蹈症患者的成纤维细胞系的辐射敏感性。
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引用本文的文献

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Human Radiosensitivity and Radiosusceptibility: What Are the Differences?人类放射敏感性与放射易感性:有何不同?
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The Nucleoshuttling of the ATM Protein: A Unified Model to Describe the Individual Response to High- and Low-Dose of Radiation?
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Mutations of the Huntington's disease protein impact on the ATM-dependent signaling and repair pathways of the radiation-induced DNA double-strand breaks: corrective effect of statins and bisphosphonates.亨廷顿舞蹈病蛋白的突变对辐射诱导的DNA双链断裂的ATM依赖信号传导和修复途径产生影响:他汀类药物和双膦酸盐的纠正作用
Mol Neurobiol. 2014 Jun;49(3):1200-11. doi: 10.1007/s12035-013-8591-7. Epub 2013 Nov 26.
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[Genetic predisposition and radiation sensitivity of tumors].[肿瘤的遗传易感性与辐射敏感性]
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