Aguzzi Adriano, Polymenidou Magdalini
Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstr. 12, CH-8091 Zürich, Switzerland.
Cell. 2004 Jan 23;116(2):313-27. doi: 10.1016/s0092-8674(03)01031-6.
Prions have been responsible for an entire century of tragic episodes. Fifty years ago, kuru decimated the population of Papua New Guinea. Then, iatrogenic transmission of prions caused more than 250 cases of Creutzfeldt-Jakob disease. More recently, transmission of bovine spongiform encephalopathy to humans caused a widespread health scare. On the other hand, the biology of prions represents a fascinating and poorly understood phenomenon, which may account for more than just diseases and may represent a fundamental mechanism of crosstalk between proteins. The two decades since Stanley Prusiner's formulation of the protein-only hypothesis have witnessed spectacular advances, and yet some of the most basic questions in prion science have remained unanswered.
朊病毒已经导致了整整一个世纪的悲剧事件。五十年前，库鲁病致使巴布亚新几内亚的人口大量死亡。随后，朊病毒的医源性传播导致了250多例克雅氏病。最近，牛海绵状脑病传播给人类引发了广泛的健康恐慌。另一方面，朊病毒的生物学特性是一个引人入胜但却知之甚少的现象，它可能不仅仅导致疾病，还可能代表了蛋白质之间相互作用的一种基本机制。自斯坦利·普鲁西纳提出仅蛋白质假说以来的二十年里，虽然取得了惊人的进展，但朊病毒科学中一些最基本的问题仍然没有答案。
