| 辐射敏感综合症--AT病研究进展 |
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| 引用本文： | 王明明,郑斯英,曹建平. 辐射敏感综合症--AT病研究进展[J]. 辐射防护, 2003, 23(6): 378-382 |
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| 作者姓名： | 王明明 郑斯英 曹建平 |
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| 作者单位： | 苏州大学放射医学与公共卫生学院,215007 |
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| 基金项目： | 国家自然科学基金，30170288， |
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| 摘 要： | 共济失调性毛细血管扩张综合症(AT)是一种单基因突变引起的遗传性疾病，表现为高辐射敏感性、进行性神经退变、免疫缺陷、早衰及易患癌症等。本文综述了AT病的研究概况，包括AT临床症状、AT基因的定位、结构与功能、辐射对AT细胞信号传导的影响、AT基因组不稳定性及基因治疗。
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| 关 键 词： | 辐射敏感综合症 AT 共济失调性毛细血管扩张综合症 基因突变 基因治疗 DNA |
PROGRESS IN THE RESEARCH OF ATAXIA TELANGIECTASIA AS A RADIATION SENSITIVE SYNDROME |
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| Wang Mingming Zheng Siying Cao Jianping. PROGRESS IN THE RESEARCH OF ATAXIA TELANGIECTASIA AS A RADIATION SENSITIVE SYNDROME[J]. Radiation Protection, 2003, 23(6): 378-382 |
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| Authors: | Wang Mingming Zheng Siying Cao Jianping |
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| Abstract: | Ataxia Telangiectasia (AT) as a genetic disorder results from single gene mutation, characterized by radiosensitivity, progressive nerve degeneration, immunodeficiency, premature aging and cancer predisposition. Progress in the research of AT is reviewed in this paper, including the clinical symptoms of this disorder, as well as the localization, structure and function of ATM (AT mutated) gene, the effects of ionizing radiation on the signal transduction of AT cells, AT genomic instability and AT gene therapy. |
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| Keywords: | Ataxia Telangiectasia Genomic Instability Gene Therapy |
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