Novel properties and biology of scrapie prions.
Novel properties and biology of scrapie prions.
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DOI：
10.1007/978-3-642-76540-7_14
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发表时间：
1991
影响因子：
--
通讯作者：
S. Prusiner
中科院分区：
文献类型：
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作者：
S. Prusiner
在过去的40年里，已经积累了一系列令人印象深刻的数据，表明羊瘙痒症，库鲁病，克雅氏病（CJD）和Gerstmann-Sträussler综合征（GSS）的许多特征不是典型的传染病。此外，在过去的二十年里，已经收集了大量的实验结果，这些结果强烈地表明羊瘙痒病病原体是一种新的病原体。为了将羊瘙痒病和CJD病原体与类病毒和病毒区分开来，引入了术语“朊病毒”（Prusiner 1982）。尽管羊瘙痒病可通过接种患病组织提取物传播给动物，但感染是否是羊瘙痒病自然传播的特征尚不清楚（Parry 1962，1983; M 'Gowan 1914;Westawayet al. 1989; Hourrigan等1979; Gordon 1966; Dickinson等1965; Dickinson 1976）。一些研究者提出自然的羊瘙痒症是一种遗传性疾病，其传染性是偶然的（Parry 1962，1983），而另一些人则强烈地认为自然的羊瘙痒症是一种传染病（Dickinsonet al. 1965; Dickinson 1976）。
Over the past 40 years, an impressive array of data has been accumulated indicating that many features of scrapie, kuru, Creutzfeldt-Jakob disease (CJD), and Gerstmann-Sträussler syndrome (GSS) are not typical of infectious diseases. Furthermore, the past two decades have witnessed the collection of numerous experimental results which argue strongly that the scrapie agent is a novel pathogen. To distinguish the scrapie and CJD agents from viroids and viruses, the term “prion” was introduced (Prusiner 1982). Although scrapie can be transmitted to animals by inoculation with extracts of diseased tissue, whether infection features in the spread of natural scrapie is unclear (Parry1962, 1983;M’Gowan1914;Westawayet al. 1989;Hourriganet al. 1979;Gordon1966;Dickinsonet al. 1965;Dickinson1976). Some investigators have proposed that natural scrapie is a genetic disease and that its transmissibility is incidental (Parry1962, 1983) while others have argued vehemently that natural scrapie is an infectious disease (Dickinsonet al. 1965;Dickinson1976).