Prusiner S B
Department of Neurology, University of California, San Francisco 94143-0518, USA.
Trends Biochem Sci. 1996 Dec;21(12):482-7. doi: 10.1016/s0968-0004(96)10063-3.
Prions cause a group of human and animal neurodegenerative diseases, which are now classified together because their etiology and pathogenesis, involve modification of the prion protein (PrP). Prion diseases are manifest as infectious, genetic and sporadic disorders. These diseases can be transmitted among mammals by the infectious particle designated 'prion'. Despite intensive searches over the past three decades, no nucleic acid has been found within prions, yet a modified isoform of the host-encoded PrP designated PrPSc is essential for infectivity. In fact, considerable experimental data argue that prions are composed exclusively of PrPSc. Earlier terms used to describe the prion diseases include transmissible encephalopathies, spongiform encephalopathies and slow virus diseases. The human prion disorders include kuru, Creutzfeldt-Jackob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI).
朊病毒会引发一系列人类和动物神经退行性疾病，由于它们的病因和发病机制都涉及朊病毒蛋白（PrP）的修饰，所以现在被归为一类。朊病毒疾病表现为传染性、遗传性和散发性疾病。这些疾病可通过名为“朊病毒”的感染性颗粒在哺乳动物之间传播。尽管在过去三十年里进行了深入研究，但在朊病毒中尚未发现核酸，然而宿主编码的PrP的一种修饰异构体PrPSc对于感染性至关重要。事实上，大量实验数据表明朊病毒仅由PrPSc组成。早期用于描述朊病毒疾病的术语包括传染性脑病、海绵状脑病和慢病毒疾病。人类朊病毒疾病包括库鲁病、克雅氏病（CJD）、格斯特曼-施特劳斯勒-谢inker综合征（GSS）和致死性家族性失眠症（FFI）。
