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Prion protein oligomer and its neurotoxicity
  • ISSN号:1672-9145
  • 期刊名称:Acta Biochimica et Biophysica Sinica
  • 时间:2013.6
  • 页码:442-451
  • 分类:S852.659.7[农业科学—基础兽医学;农业科学—兽医学;农业科学—畜牧兽医] X503.1[环境科学与工程—环境工程]
  • 作者机构:[1]School of Life Science and Technology, China Pharmaceutical University, Nanjing 21009, China, [2]Shanghai Institute of Materia Medica, Chinese Academy of Sciences, Shanghai 201203, China, [3]Key Laboratory of Chemical Biology of Fujian Province, College of Chemistry and Chemical Engineering, Xiamen University, Xiamen 361005 China
  • 相关基金:Funding This work was supported by the grants from the National Natural Science Foundation of China (Nos. 31170717, 91129713, and 30900233).
  • 相关项目:兔和人朊病毒蛋白PrPC与铜离子结合特性的差异研究
中文摘要:

prion 疾病,也作为能递送的海绵状的 encephalopathies 知道,是致命的 neurodegenerative 混乱。根据蛋白质仅仅假设,在 prion 疾病的致病的关键分子的事件是导出主人的细胞的 prion 蛋白质(PrPC ) 的 conformational 变换进一种错误褶层形式(scrapie PrP, PrPSc ) 。增加的证据证明了最传染的因素是 prion 蛋白质形成的更小的 subfibrillar oligomers。prion oligomer 和 PrPSc 富于表结构并且对朊酶 K 的解朊作用抵抗。而 PrPSc 是不可溶解的, prion oligomer 在生理的环境是可溶的。各种各样的 prion oligomers 处于不同条件被形成。Prion oligomers 比 PrPSc 的纤丝状的形式在 vitro 并且在 vivo 展出了更多的 neurotoxicity,暗示 prion oligomers 能是为攻击 prion 疾病的潜在的药目标。在这篇文章,我们关于 prion oligomers 描述最近的试验性的证据,与 prion oligomer 形成和它的 neurotoxicity 的一个特殊焦点。

英文摘要:

The prion diseases, also known as transmissible spongiform encephalopathies, are fatal neurodegenerative disorders. According to the 'protein only' hypothesis, the key molecular event in the pathogenesis of priori disease is the conformational conversion of the host-derived cellular prion protein (PrPc) into a misfolded form (scrapie PrP, prpSC). Increasing evidence has shown that the most infectious factor is the smaller subfibrillar oligomers formed by prion proteins. Both the prion oligomer and PrPsc are rich in β-sheet structure and resistant to the proteolysis of pro- teinase K. The prion oligomer is soluble in physiologic environments whereas PrPsc is insoluble. Various prion oligomers are formed in different conditions. Prion oligomers exhibited more neurotoxicity both in vitro and in vivo than the fibrillar forms of PrPsc, implying that prion oligo- mers could be potential drug targets for attacking prion diseases. In this article, we describe recent experimental evidence regarding prion oligomers, with a special focus on prion oligomer formation and its neurotoxicity.

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期刊信息
  • 《生物化学与生物物理学报:英文版》
  • 北大核心期刊(2004版)
  • 主管单位:
  • 主办单位:中国科学院上海生物化学研究所
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  • 地址:上海岳阳路319号
  • 邮编:200031
  • 邮箱:abbs@sibs.ac.cn
  • 电话:021-54920956 54920955
  • 国际标准刊号:ISSN:1672-9145
  • 国内统一刊号:ISSN:31-1940/Q
  • 邮发代号:4-210
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  • 被引量:5851