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The utility of bank voles for studying prion disease.
Progress in Molecular Biology and Translational Science ( IF 4.025 ) Pub Date : 2020-09-08 , DOI: 10.1016/bs.pmbts.2020.08.009
Hamza Arshad 1 , Matthew E C Bourkas 1 , Joel C Watts 1
Affiliation  

The transmission of prions between species is typically an inefficient process due to the species barrier, which represents incompatibility between prion seed and substrate molecules. Bank voles (Myodes glareolus) are an exception to this rule, as they are susceptible to a diverse range of prion strains from many different animal species. In particular, bank voles can be efficiently infected with most types of human prions and have played a critical role in validating variably protease-sensitive prionopathy (VPSPr) and certain forms of Gerstmann-Sträussler-Scheinker (GSS) disease as bona fide prion disorders rather than non-transmissible proteinopathies. The bank vole prion protein (BVPrP) confers a “universal prion acceptor” phenotype when expressed in mice and when used as a substrate for in vitro prion amplification assays, indicating that the unique prion transmission properties of bank voles are mediated by BVPrP. Over-expression of BVPrP in mice can also promote the spontaneous development of prion disease, indicating that BVPrP is intrinsically prone to both spontaneous and template-directed misfolding. Here, we discuss the utility of bank voles and BVPrP for prion research and how they have provided new tools for establishing rapid animal bioassays, modeling spontaneous prion disease, standardizing prion diagnostics, and understanding the molecular basis of the species barrier.



中文翻译:

银行田鼠在研究病毒疾病中的用途。

由于物种之间的屏障,species病毒在物种之间的传播通常是效率低下的过程,这代表了ion病毒种子与底物分子之间的不相容性。岸田鼠(Myodes glareolus)是该规则的例外,因为它们容易受到来自许多不同动物物种的多种diverse病毒株的影响。特别是,银行田鼠可以有效地感染大多数类型的人类朊病毒的,并已在验证可变蛋白酶敏感prionopathy(VPSPr)和格-施-沙(GSS)的一些形式的疾病的发挥了关键作用善意ion病毒疾病,而不是不可传播的蛋白病。当在小鼠中表达并且用作体外病毒扩增测定的底物时,库田vol病毒ion蛋白(BVPrP)赋予“通用病毒受体”表型,表明库田鼠的独特病毒传递特性是由BVPrP介导的。BVPrP在小鼠中的过表达还可以促进of病毒疾病的自发发展,表明BVPrP本质上倾向于自发和模板定向错误折叠。在这里,我们讨论了银行田鼠和BVPrP在病毒研究中的实用性,以及它们如何为建立快速动物生物测定,模拟自发性病毒疾病,标准化病毒诊断以及理解物种屏障的分子基础提供了新工具。

更新日期:2020-09-20
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