Boyle Aileen, Hogan Kris, Manson Jean C, Diack Abigail B
The Roslin Institute & R(D)SVS, University of Edinburgh, Easter Bush, Edinburgh, EH25 9RG, UK.
Methods Mol Biol. 2017;1658:263-283. doi: 10.1007/978-1-4939-7244-9_18.
Transmissible spongiform encephalopathies (TSE) or prion diseases exhibit strain variation, a phenomenon that has been studied extensively in mouse bioassays. Despite the introduction of many rapid in vitro systems, bioassays remain a key tool in defining prion strains and their ability to transmit disease in vivo. Prion strains can be characterized by a range of phenotypic characteristics such as incubation period, vacuolar pathology, and distribution of the abnormal form of PrP following experimental transmission of the agent into a panel of mice (transgenic or wild type). Interpretation of these characteristics requires considerable experience and an understanding of the procedures used to define them. This chapter reviews the techniques used in strain typing of prion diseases from inoculum preparation and pathological studies to data interpretation alongside an extensive troubleshooting guide.
传染性海绵状脑病（TSE）或朊病毒疾病表现出毒株变异，这一现象已在小鼠生物测定中得到广泛研究。尽管引入了许多快速体外系统，但生物测定仍然是定义朊病毒毒株及其在体内传播疾病能力的关键工具。朊病毒毒株可通过一系列表型特征来表征，如潜伏期、空泡病变以及将病原体实验性接种到一组小鼠（转基因或野生型）后异常形式的朊蛋白（PrP）的分布。对这些特征的解释需要相当多的经验以及对用于定义它们的程序的理解。本章回顾了朊病毒疾病毒株分型中使用的技术，从接种物制备、病理研究到数据解释，并附带一份详尽的故障排除指南。
