Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein

Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein
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DOI:
10.1099/vir.0.042507-0
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发表时间:
2012-07-01
影响因子:
3.8
通讯作者:
Barron, Rona M.
Barron, Rona M.
中科院分区:
医学3区
文献类型:
--
作者:
Wilson, Rona;Plinston, Chris;Barron, Rona M.

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牛海绵状脑病(BSE)和变异型克雅氏病(vCJD)之间的关联已证明,牛传染性海绵状脑病(TSE)可对人类健康构成风险,并提高了其他反刍动物TSE可能传染给人类的可能性。近年来,已描述了绵羊、牛和鹿中的几种新型TSE,目前尚不清楚这些病原体对人类造成的风险。在这项研究中,我们接种了两种形式的非典型BSE(基础和H型BSE),慢性消耗性疾病(CWD)分离株和七个分离株的非典型羊瘙痒症基因靶向转基因(Tg)小鼠表达人朊蛋白(PrP)。在用这些反刍动物TSE激发后,表达人PrP的基因靶向Tg小鼠未显示任何疾病病理学体征。这些数据强烈表明,这些最近发现的反刍动物TSE和人类之间存在实质性传播障碍。
The association between bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) has demonstrated that cattle transmissible spongiform encephalopathies (TSEs) can pose a risk to human health and raises the possibility that other ruminant TSEs may be transmissible to humans. In recent years, several novel TSEs in sheep, cattle and deer have been described and the risk posed to humans by these agents is currently unknown. In this study, we inoculated two forms of atypical BSE (BASE and H-type BSE), a chronic wasting disease (CWD) isolate and seven isolates of atypical scrapie into gene-targeted transgenic (Tg) mice expressing the human prion protein (PrP). Upon challenge with these ruminant TSEs, gene-targeted Tg mice expressing human PrP did not show any signs of disease pathology. These data strongly suggest the presence of a substantial transmission barrier between these recently identified ruminant TSEs and humans.