Prion diseases or transmissible spongiform encephalopathies, including Creutzfeldt-Jakob disease (CJD) and variant CJD (vCJD), are chronic neurodegenerative diseases characterized by accumulation of abnormal infectious prions known as PrPSc. Infectious prion is emerging as a blood transfusion-transmissible pathogen and is present at extremely low levels in the blood of asymptomatic patients, which is not detectable by current standard methodologies. As such, prion-related diseases impose a huge challenge to blood transfusion services around the world. Rapid, sensitive, and specific assays are thus urgently needed to detect trace amounts of infectious prions in the blood or tissues of asymptomatic patients, as early diagnostic blood transfusion and screening tools. Recent advances in the in vitro assay technologies including protein misfolding cyclic amplification and immunoassays have made it possible to detect infectious prions in the blood of animals with scrapie and even of asymptomatic animals infected with prions. These assays thus bring some hope for the possibility of detecting infectious prions in human blood and tissues for both early diagnosis for blood transfusion and transplantation screening purposes. This review focuses on the development of the various in vitro bioassay systems for the rapid amplification and detection of infectious prions that might be present in blood and tissues for blood transfusion and transplantation recipients.

中文翻译：

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用于在血液和组织中扩增和检测异常病毒PrP（Sc）的体外生物测定系统。

on病毒疾病或可传播的海绵状脑病，包括Creutzfeldt-Jakob病（CJD）和变异CJD（vCJD），是慢性神经退行性疾病，其特征是积累了称为PrPSc的异常感染性ions病毒。感染性ion病毒正在作为一种可以通过输血传播的病原体出现，并且在无症状患者的血液中的含量极低，这是当前标准方法无法检测到的。因此，与病毒有关的疾病对全世界的输血服务提出了巨大的挑战。因此，作为早期诊断输血和筛查工具，迫切需要快速，灵敏和特异的检测方法来检测无症状患者血液或组织中的痕量感染性ions病毒。包括蛋白质错误折叠循环扩增和免疫测定在内的体外测定技术的最新进展使得检测带有瘙痒病的动物甚至感染病毒的无症状动物血液中的感染性ions病毒成为可能。因此，这些测定法为检测人血和组织中的感染性ions病毒以用于输血和移植筛查的早期诊断提供了希望。这篇综述着重于各种体外生物测定系统的开发，以快速扩增和检测血液和组织中可能存在的，用于输血和移植接受者的感染性pr病毒。