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Prion propagation, toxicity and degradation
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Journal Article
Prion propagation, toxicity and degradation
2012
Overview
In this perspective, the authors review new developments that suggest that many diseases share features with prion infections. They also highlight some of the critical open questions in prion biology, including how prions damage their hosts and how hosts attempt to neutralize invading prions.
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?
Publisher
Nature Publishing Group US,Nature Publishing Group
Subject
