Kübler Eric, Oesch Bruno, Raeber Alex J
Prionics AG, Schlieren, Switzerland.
Br Med Bull. 2003;66:267-79. doi: 10.1093/bmb/66.1.267.
Prion diseases are usually diagnosed clinically and confirmed by post-mortem histopathological examination of brain tissue. The only reliable molecular marker for prion diseases is PrP(Sc), the pathological conformer of the prion protein that accumulates in the central nervous system and, to a lesser extent, in lymphoreticular tissues. For BSE, several commercial diagnostic kits based on the post-mortem immunochemical detection of PrP(Sc) in brain tissue are now available. These rapid screening tests have been used in active surveillance of BSE and have greatly improved the detection of infected cattle before their entry into the human food chain. At present, no diagnostic test exists for the detection of prion diseases in live animals or humans. New diagnostic techniques aimed at increasing sensitivity and specificity of PrP(Sc) detection in body fluids and at identifying novel surrogate markers are under development. In this report, we review the classical diagnostic methods as well as present and future tools for the diagnosis of prion diseases.
朊病毒疾病通常通过临床诊断，并通过对脑组织进行尸检组织病理学检查来确诊。朊病毒疾病唯一可靠的分子标志物是PrP(Sc)，它是朊病毒蛋白的病理构象异构体，在中枢神经系统中积累，并在较小程度上在淋巴网状组织中积累。对于牛海绵状脑病（BSE），现在有几种基于对脑组织中PrP(Sc)进行死后免疫化学检测的商业诊断试剂盒。这些快速筛查试验已用于BSE的主动监测，并大大提高了在受感染牛进入人类食物链之前对其的检测能力。目前，尚无用于检测活体动物或人类朊病毒疾病的诊断试验。旨在提高体液中PrP(Sc)检测的灵敏度和特异性以及识别新型替代标志物的新诊断技术正在开发中。在本报告中，我们回顾了经典诊断方法以及朊病毒疾病诊断的现有和未来工具。
