Liberski P P
Department of Oncology, School of Medicine Lodz.
Folia Neuropathol. 1994;32(2):65-73.
Basic hypotheses concerning nature of an infectious agent of the transmissible spongiform encephalopathies are reported here. The agent may consist of a protein only (PrPsc), and for such a molecular structure the term "prion" has been coined. It may consist of a yet-to-be-discovered small oligonucleotide and a shell protein and this is the "virino" concept. Or, the agent may be a virus which has still eluded detection. The experiments with transgenic and knock-out mice proved that PrPsc is crucial for pathogenesis of spongiform encephalopathies. However, presence of strains of scrapie agent strongly favour a nucleic acid as a template of genetic information, which is difficult to reconcile with the "protein only" hypothesis. On the other hand, the development of spontaneous neurodegenerative disorder in Tg(GSS MoPrP) mice, if independently confirmed, strongly supports the "prion" hypothesis. Furthermore, the linkage of mutations within the PRNP gene with phenotypic appearance of Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker syndrome points to importance of the PrP gene. In conclusion, it must be said that the molecular structure of the agent is still uncertain.
本文报道了关于传染性海绵状脑病致病因子本质的基本假说。该致病因子可能仅由一种蛋白质（PrPsc）组成，针对这种分子结构创造了“朊病毒”一词。它可能由一种尚未被发现的小寡核苷酸和一种外壳蛋白组成，这就是“病毒样颗粒”的概念。或者，该致病因子可能是一种尚未被检测到的病毒。转基因和基因敲除小鼠实验证明，PrPsc对海绵状脑病的发病机制至关重要。然而，瘙痒病病原体毒株的存在强烈支持核酸作为遗传信息的模板，这很难与“仅蛋白质”假说相协调。另一方面，如果能独立证实，Tg(GSS MoPrP)小鼠中自发性神经退行性疾病的发展将有力支持“朊病毒”假说。此外，PRNP基因内的突变与克雅氏病和格斯特曼-施特劳斯勒-谢inker综合征的表型出现之间的联系表明了PrP基因的重要性。总之，必须指出的是，该致病因子的分子结构仍然不确定。
