Immortalization of prion protein deficient cell lines and establishment of prion-susceptible susceptible cell lines by introduction of prion protein genes.

朊病毒感染后，朊病毒蛋白的异常同种型（PrP（Sc））将朊病毒蛋白的细胞同种型（PrP（C））转化为PrP（Sc）。PrP（C）向PrP（Sc）的转化导致PrP（Sc）积累和PrP（C）缺乏，在病因学上有助于诱导朊病毒疾病。目前，朊病毒病的诊断方法大多依赖于PrP（Sc）的检测。PrP（Sc）在许多不同样品中的高灵敏度/准确度特异性检测是尝试开发可靠检测方法的先决条件。为了实现这一目标，最近已经开发了几种方法来促进PrP（Sc）的灵敏和精确检测，即蛋白质错误折叠循环扩增、构象依赖免疫测定、解离增强镧系元素荧光免疫测定、毛细管凝胶电泳、荧光相关光谱、流动微珠免疫测定等。认识到朊病毒感染机制的复杂性的功能相关的朊病毒易感细胞培养模型也一直在研究，不仅与诊断有关，而且与朊病毒生物学有关。朊蛋白（PrP）基因缺陷的神经元细胞系能够清楚地阐明PrP（C）的功能，这将有助于理解朊病毒感染的机制。本文综述了朊病毒疾病诊断方法和细胞培养模型的发展趋势及其在朊病毒生物学中的应用前景。

After prion infection, an abnormal isoform of prion protein (PrP (Sc)) converts the cellular isoform of prion protein (PrP (C)) into PrP(Sc). PrP (C)-to-PrP (Sc) conversion leads to PrP (Sc) accumulation and PrP (C) deficiency, contributing etiologically to induction of prion diseases. Presently, most of the diagnostic methods for prion diseases are dependent on PrP (Sc) detection. Highly sensitive/accurate specific detection of PrP (Sc) in many different samples is a prerequisite for attempts to develop reliable detection methods. Towards this goal, several methods have recently been developed to facilitate sensitive and precise detection of PrP (Sc), namely, protein misfolding cyclic amplification, conformation-dependent immunoassay, dissociation-enhanced lanthanide fluorescent immunoassay, capillary gel electrophoresis, fluorescence correlation spectroscopy, flow microbead immunoassay, etc. Additionally, functionally relevant prion-susceptible cell culture models that recognize the complexity of the mechanisms of prion infection have also been pursued, not only in relation to diagnosis, but also in relation to prion biology. Prion protein (PrP) gene-deficient neuronal cell lines that can clearly elucidate PrP (C) functions would contribute to understanding of the prion infection mechanism. In this review, we describe the trend in recent development of diagnostic methods and cell culture models for prion diseases and their potential applications in prion biology.