Llorens Franc, Ferrer Isidre, del Río José Antonio
Institute of Neuropathology, University Hospital Bellvitge-Bellvitge Biomedical Research Institute (IDIBELL), L'Hospitalet de Llobregat, Spain,
Mol Neurobiol. 2014 Feb;49(1):413-23. doi: 10.1007/s12035-013-8529-0. Epub 2013 Aug 16.
The cellular prion protein (PrP(C)) plays a key role in prion diseases when it converts to the pathogenic form scrapie prion protein. Increasing knowledge of its participation in prion infection contrasts with the elusive and controversial data regarding its physiological role probably related to its pleiotropy, cell-specific functions, and cellular-specific milieu. Multiple approaches have been made to the increasing understanding of the molecular mechanisms and cellular functions modulated by PrP(C) at the transcriptomic and proteomic levels. Gene expression analyses have been made in several mouse and cellular models with regulated expression of PrP(C) resulting in PrP(C) ablation or PrP(C) overexpression. These analyses support previous functional data and have yielded clues about new potential functions. However, experiments on animal models have shown moderate and varied results which are difficult to interpret. Moreover, studies in cell cultures correlate little with in vivo counterparts. Yet, both animal and cell models have provided some insights on how to proceed in the future by using more refined methods and selected functional experiments.
细胞朊蛋白（PrP(C)）在转化为致病形式的瘙痒病朊蛋白时，在朊病毒疾病中起关键作用。人们对其参与朊病毒感染的认识不断增加，但关于其生理作用的数据却难以捉摸且存在争议，这可能与其多效性、细胞特异性功能和细胞特异性环境有关。人们已采用多种方法，以在转录组学和蛋白质组学水平上增进对由PrP(C)调节的分子机制和细胞功能的理解。在几种小鼠和细胞模型中进行了基因表达分析，这些模型中PrP(C)的表达受到调控，导致PrP(C)缺失或PrP(C)过表达。这些分析支持了先前的功能数据，并产生了关于新潜在功能的线索。然而，在动物模型上进行的实验显示出中度且多样的结果，难以解释。此外，细胞培养中的研究与体内情况相关性不大。不过，动物模型和细胞模型都为未来如何通过使用更精细的方法和选定的功能实验提供了一些见解。
