Department of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena, Rome 299-00164, Italy.
Pathogens. 2013 Jul 2;2(3):446-56. doi: 10.3390/pathogens2030446.
Prions exist as different strains exhibiting distinct disease phenotypes. Currently, the identification of prion strains is still based on biological strain typing in rodents. However, it has been shown that prion strains may be associated with distinct PrPSc biochemical types. Taking advantage of the availability of several prion strains adapted to a novel rodent model, the bank vole, we investigated if any prion strain was actually associated with distinctive PrPSc biochemical characteristics and if it was possible to univocally identify strains through PrPSc biochemical phenotypes. We selected six different vole-adapted strains (three human-derived and three animal-derived) and analyzed PrPSc from individual voles by epitope mapping of protease resistant core of PrPSc (PrPres) and by conformational stability and solubility assay. Overall, we discriminated five out of six prion strains, while two different scrapie strains showed identical PrPSc types. Our results suggest that the biochemical strain typing approach here proposed was highly discriminative, although by itself it did not allow us to identify all prion strains analyzed.
朊病毒存在不同的株，表现出不同的疾病表型。目前，朊病毒株的鉴定仍然基于啮齿动物的生物学株型鉴定。然而，已经表明朊病毒株可能与不同的 PrPSc 生化类型有关。利用几种适应新型啮齿动物模型——田鼠的朊病毒株的可用性，我们研究了是否存在与独特的 PrPSc 生化特征相关的朊病毒株，以及是否可以通过 PrPSc 的生化表型来明确地鉴定株型。我们选择了六种不同的田鼠适应株（三种来源于人类，三种来源于动物），并通过蛋白酶抗性核心的表位作图（PrPres）和构象稳定性和溶解度测定分析来自个体田鼠的 PrPSc。总的来说，我们区分了六种中的五种，而两种不同的瘙痒病株显示出相同的 PrPSc 类型。我们的研究结果表明，这里提出的生化株型鉴定方法具有高度的区分能力，尽管它本身并不能识别所有分析的朊病毒株。
