Michel B
Service de Neurologie, Hôpital Sainte-Marguerite, Marseille.
Rev Neurol (Paris). 1990;146(1):1-11.
The concept of prion encephalopathy has emerged from such previous notions as slow virus infections, spongiform encephalopathies or transmissible dementias. The term prion (Prusiner, 1982) is now used in preference to unconventional agents. Proteins and genes of prions have recently been identified by molecular biology. Exactly how prion proteins are amplified in cells is still unknown. It has been demonstrated that amyloid deposits in scrapie-infected brain, Creutzfeldt-Jakob, Gerstmann-Straüssler and Kuru diseases are composed of prion proteins. Prion encephalopathies are good models to study some immunopathological mechanisms observed in the central nervous system in degenerative diseases or ageing.
朊病毒脑病的概念源自先前的一些观念，如慢病毒感染、海绵状脑病或传染性痴呆。现在更倾向于使用术语“朊病毒”（普鲁西纳，1982年）而非非常规病原体。朊病毒的蛋白质和基因最近已通过分子生物学鉴定出来。朊病毒蛋白在细胞中究竟如何扩增仍不清楚。已经证明，在羊瘙痒病感染的大脑、克雅氏病、格斯特曼-施特劳斯勒病和库鲁病中的淀粉样沉积物是由朊病毒蛋白组成的。朊病毒脑病是研究在退行性疾病或衰老过程中中枢神经系统观察到的一些免疫病理机制的良好模型。
