Bell J E, Gentleman S M, Ironside J W, McCardle L, Lantos P L, Doey L, Lowe J, Fergusson J, Luthert P, McQuaid S, Allen I V
Creuzfeldt-Jakob Disease Surveillance Unit, Edinburgh, UK.
Neuropathol Appl Neurobiol. 1997 Feb;23(1):26-35.
Creutzfeldt-Jakob disease (CJD) and other prion diseases are associated with the deposition of insoluble prion protein (PrPCJD) in the central nervous system (CNS). Antibodies raised against PrPCJD also react with its precursor protein, a soluble form of PrP (PrPC), which is widely distributed in the normal CNS. This cross-reactivity has in the past raised doubts as to the specificity and diagnostic reliability of PrP immunolocalization, especially in familial cases which are atypical clinically and which lack characteristic pathology findings. Following an MRC-funded workshop which focused on this problem, a multicentre prospective study was set up to identify a reliable protocol for PrPCJD immunocytochemistry. Five UK centres took part in this study and demonstrated consistent staining of plaques, vacuolar deposits in severe spongiform change, and perineuronal deposits using a variety of antibodies and enhancement procedures. A protocol using formic acid, guanidine thiocyanate, and hydrated autoclaving pre-treatment in conjunction with a monoclonal PrPCJD antibody produced the clearest immunochemical results and is presented as the consensus UK recommendation for PrPCJD immunocytochemical procedures.
克雅氏病（CJD）及其他朊病毒病与中枢神经系统（CNS）中不溶性朊病毒蛋白（PrPCJD）的沉积有关。针对PrPCJD产生的抗体也会与其前体蛋白（一种可溶性PrP形式，即PrPC）发生反应，PrPC在正常中枢神经系统中广泛分布。这种交叉反应过去曾引发对PrP免疫定位的特异性和诊断可靠性的质疑，尤其是在临床非典型且缺乏特征性病理表现的家族性病例中。在一个由医学研究委员会资助、聚焦该问题的研讨会后，开展了一项多中心前瞻性研究，以确定PrPCJD免疫细胞化学的可靠方案。英国的五个中心参与了这项研究，使用多种抗体和增强程序，均显示出斑块、严重海绵状改变中的空泡沉积物以及神经元周围沉积物的一致染色。一种使用甲酸、硫氰酸胍和高压湿热预处理联合单克隆PrPCJD抗体的方案产生了最清晰的免疫化学结果，现作为英国对PrPCJD免疫细胞化学程序的共识推荐呈现。
