Scrapie in goats has been known since 1942, the archetype of prion diseases in which only prion protein (PrP) in misfolded state (PrPSc) acts as infectious agent with fatal consequence. Emergence of bovine spongiform encephalopathy (BSE) with its zoonotic behaviour and detection in goats enhanced fears that its source was located in small ruminants. However, in goats knowledge on prion strain typing is limited. A European-wide study is presented concerning the biochemical phenotypes of the protease resistant fraction of PrPSc (PrPres) in over thirty brain isolates from transmissible spongiform encephalopathy (TSE) affected goats collected in seven countries. Three different scrapie forms were found: classical scrapie (CS), Nor98/atypical scrapie and one case of CH1641 scrapie. In addition, CS was found in two variants—CS-1 and CS-2 (mainly Italy)—which differed in proteolytic resistance of the PrPres N-terminus. Suitable PrPres markers for discriminating CH1641 from BSE (C-type) appeared to be glycoprofile pattern, presence of two triplets instead of one, and structural (in)stability of its core amino acid region. None of the samples exhibited BSE like features. BSE and these four scrapie types, of which CS-2 is new, can be recognized in goats with combinations of a set of nine biochemical parameters.

中文翻译：

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生化方法区分山羊的四种瘙痒病和牛海绵状脑病

自从1942年以来就已经知道山羊痒症是，病毒疾病的原型，其中只有处于错误折叠状态的病毒蛋白（PrPc）（PrPSc）才是导致致命后果的传染原。牛海绵状脑病（BSE）的出现与人畜共患病行为以及在山羊中的发现都加剧了人们对其来源位于反刍动物中的担忧。但是，在山羊中，关于病毒毒株类型的知识是有限的。欧洲范围内的一项研究提出了关于在七个国家/地区从超过30例来自传染性海绵状脑病（TSE）感染山羊的脑分离物中PrPSc（PrPres）的蛋白酶抗性部分的生化表型的研究。发现了三种不同的刮擦形式：经典刮擦（CS），Nor98 /非典型刮擦和一箱CH1641刮擦。此外，在两个变体CS-1和CS-2（主要是意大利）中发现了CS，它们在PrPres N末端的蛋白水解抗性方面有所不同。区分CH1641与BSE（C型）的合适PrPres标记似乎是糖谱图，两个三联体而不是一个三联体以及其核心氨基酸区域的结构（不稳定）稳定性。所有样品均未表现出类似疯牛病的特征。在山羊中，通过结合九种生化参数的组合，可以识别出疯牛病和这四种新的痒痒病类型，其中CS-2是新的。