Transmissible spongiform encephalopathies or prion diseases describe a number of different human disorders that differ in their clinical phenotypes, which are nonetheless united by their transmissible nature and common pathology. Clinical variation in the absence of a conventional infectious agent is believed to be encoded by different conformations of the misfolded prion protein. This misfolded protein is the target of methods designed to prevent disease transmission in a surgical setting and reduction of the misfolded seed or preventing its continued propagation have been the focus of therapeutic strategies. It is therefore possible that strain variation may influence the efficacy of prevention and treatment approaches.

Historically, an understanding of prion disease transmission and pathogenesis has been focused on research tools developed using agriculturally relevant strains of prion disease. However, an increased understanding of the molecular biology of human prion disorders has highlighted differences not only between different forms of the disease affecting humans and animals but also within diseases such as Creutzfeldt-Jakob Disease (CJD), which is represented by several sporadic CJD specific conformations and an additional conformation associated with variant CJD. In this chapter we will discuss whether prion strain variation can affect the efficacy of methods used to decontaminate prions and whether strain variation in pre-clinical models of prion disease can be used to identify therapeutic strategies that have the best possible chance of success in the clinic.

可传播的海绵状脑病或病毒疾病描述了许多不同的人类疾病，它们的临床表型不同，但由于其可传播的性质和共同的病理学而结合在一起。据信在缺乏常规感染剂的情况下临床变异是由错折叠的ion病毒蛋白的不同构象编码的。这种错误折叠的蛋白质是设计用于在外科手术环境中防止疾病传播的方法的目标，减少错误折叠的种子或防止其继续繁殖一直是治疗策略的重点。因此，应变变化可能会影响预防和治疗方法的功效。

历史上，对病毒疾病传播和发病机理的理解一直集中在使用与农业相关的relevant病毒疾病菌株开发的研究工具上。但是，对人类pr病毒疾病分子生物学的深入了解不仅突显了影响人类和动物的不同形式疾病之间的差异，而且还突出了诸如克雅氏病（Creutzfeldt-Jakob Disease）（CJD）之类的疾病，该疾病以几种零星的CJD特异性代表构象以及与变体CJD相关的其他构象。