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      Prions, prionoids and protein misfolding disorders

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      Nature Reviews Genetics
      Springer Nature

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          Abstract

          Prion diseases are progressive, incurable and fatal neurodegenerative conditions. The term 'prion' was first nominated to express the revolutionary concept that a protein could be infectious. We now know that prions consist of PrPSc, the pathological aggregated form of the cellular prion protein PrPC. Over the years, the term has been semantically broadened to describe aggregates irrespective of their infectivity, and the prion concept is now being applied, perhaps overenthusiastically, to all neurodegenerative diseases that involve protein aggregation. Indeed, recent studies suggest that prion diseases (PrDs) and protein misfolding disorders (PMDs) share some common disease mechanisms, which could have implications for potential treatments. Nevertheless, the transmissibility of bona fide prions is unique, and PrDs should be considered as distinct from other PMDs.

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          Author and article information

          Journal
          Nature Reviews Genetics
          Nat Rev Genet
          Springer Nature
          1471-0056
          1471-0064
          April 30 2018
          Article
          10.1038/s41576-018-0011-4
          29713012
          d5cfe57f-239c-40c9-ada5-84a1323d7e21
          © 2018

          http://www.springer.com/tdm

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