PERSPECTIVE Prion propagation, toxicity and degradation
PERSPECTIVE Prion propagation, toxicity and degradation
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DOI：
10.1038/nn.3120
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发表时间：
2012-07-01
影响因子：
25
通讯作者：
Falsig, Jeppe
中科院分区：
文献类型：
--
作者：
Aguzzi, Adriano;Falsig, Jeppe
二十年来，普里恩科学就像坐过山车一样。20世纪90年代中期，疯牛病(牛海绵状脑病，BSE)的幽灵引发了一场前所未有的公众恐慌，最初是因为人们意识到这种动物的普里恩疾病可以传播给人类，然后又因为有证据表明BSE感染的人可以通过输血传播感染而重新点燃。随着疯牛病的逐渐消失，公众、资助机构和未来的博士生对普恩病毒的兴趣也在减弱。然而，在过去的几年里，人们发现了一系列令人困惑的疾病与普恩病毒感染的共同特征，包括细胞间传播。在这里，我们回顾这些进展，并总结那些我们目前认为在普恩生物学中最有趣的开放问题：普恩如何损害它们的宿主，以及宿主如何试图中和入侵的普恩？
Prion science has been on a rollercoaster for two decades. In the mid 1990s, the specter of mad cow disease (bovine spongiform encephalopathy, BSE) provoked an unprecedented public scare that was first precipitated by the realization that this animal prion disease could be transmitted to humans and then rekindled by the evidence that BSE-infected humans could pass on the infection through blood transfusions. Along with the gradual disappearance of BSE, the interest in prions has waned with the general public, funding agencies and prospective PhD students. In the past few years, however, a bewildering variety of diseases have been found to share features with prion infections, including cell-to-cell transmission. Here we review these developments and summarize those open questions that we currently deem most interesting in prion biology: how do prions damage their hosts, and how do hosts attempt to neutralize invading prions?