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- W2017025451 abstract "Prions are infectious self-replicating proteins that can cause neurodegenerative disorders such as bovine spongiform encephalopathy. Here, Colby and Prusiner discuss recent advances in the generation of synthetic prion strains that may lead to new insights into the structure of prions and the mechanisms by which they originate and propagate. Prions are self-replicating proteins that can cause neurodegenerative disorders such as bovine spongiform encephalopathy (also known as mad cow disease). Aberrant conformations of prion proteins accumulate in the central nervous system, causing spongiform changes in the brain and eventually death. Since the inception of the prion hypothesis — which states that misfolded proteins are the infectious agents that cause these diseases — researchers have sought to generate infectious proteins from defined components in the laboratory with varying degrees of success. Here, we discuss several recent studies that have produced an array of novel prion strains in vitro that exhibit increasingly high titres of infectivity. These advances promise unprecedented insight into the structure of prions and the mechanisms by which they originate and propagate." @default.
- W2017025451 created "2016-06-24" @default.
- W2017025451 creator A5032183531 @default.
- W2017025451 creator A5066991343 @default.
- W2017025451 date "2011-09-26" @default.
- W2017025451 modified "2023-09-23" @default.
- W2017025451 title "De novo generation of prion strains" @default.
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- W2017025451 doi "https://doi.org/10.1038/nrmicro2650" @default.
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