Ernst Sonja, Nonno Romolo, Langeveld Jan, Andreoletti Olivier, Acin Cristina, Papasavva-Stylianou Penelope, Sklaviadis Theodoros, Acutis Pier Luigi, van Keulen Lucien, Spiropoulos John, Keller Markus, Groschup Martin H, Fast Christine
Friedrich-Loeffler-Institut, 17493 Greifswald-Isle of Riems, Germany.
Department of Food Safety, Nutrition and Veterinary Public Health, Istituto Superiore di Sanità, 00161 Rome, Italy.
Pathogens. 2024 Jul 27;13(8):629. doi: 10.3390/pathogens13080629.
After the detection of bovine spongiform encephalopathy (BSE), and a zoonotic transmissible spongiform encephalopathy (TSE) caused by the pathological prion protein (PrP) in two goats, the investigation of goat prions became of greater interest. Therefore, a broad collection of European goat TSE isolates, including atypical scrapie, CH1641 and goat BSE as reference prion strains were biochemically characterised and subsequently inoculated into seven rodent models for further analysis (already published results of this comprehensive study are reviewed here for comparative reasons). We report here the histopathological and immunohistochemical data of this goat TSE panel, obtained after the first passage in Tgshp IX (tg-shARQ) mice, which overexpress the ovine prion protein. In addition to the clear-cut discrimination of all reference prion strains from the classical scrapie (CS) isolates, we were further able to determine three categories of CS strains. The investigation further indicates the occurrence of sub-strains that slightly resemble distant TSE strains, such as BSE or CH1641, reinforcing the theory that CS is not a single strain but a mixture of sub-strains, existing at varying extents in one isolate. This study further proved that Tgshp IX is a potent and reliable tool for the in-depth characterisation of prion strains.
在检测到牛海绵状脑病（BSE）以及两只山羊感染由病理性朊病毒蛋白（PrP）引起的人畜共患传染性海绵状脑病（TSE）后，山羊朊病毒的研究变得更受关注。因此，广泛收集了欧洲山羊TSE分离株，包括非典型瘙痒病、CH1641和山羊BSE作为参考朊病毒株，并对其进行了生化特性分析，随后接种到七种啮齿动物模型中进行进一步分析（出于比较目的，在此回顾了这项综合研究已发表的结果）。我们在此报告该山羊TSE组在过表达绵羊朊病毒蛋白的Tgshp IX（tg-shARQ）小鼠中首次传代后获得的组织病理学和免疫组织化学数据。除了能明确区分所有参考朊病毒株与经典瘙痒病（CS）分离株外，我们还进一步能够确定三类CS株。该研究进一步表明存在一些亚株，它们与如BSE或CH1641等远距离TSE株略有相似，这强化了CS不是单一毒株而是亚株混合物的理论，这些亚株在一个分离株中以不同程度存在。这项研究进一步证明Tgshp IX是深入表征朊病毒株的有效且可靠的工具。
