Prions, prionoids and protein misfolding disorders

Nat Rev Genet. 2018 Jul;19(7):405-418. doi: 10.1038/s41576-018-0011-4.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Humans
  • PrPC Proteins* / genetics
  • PrPC Proteins* / metabolism
  • PrPSc Proteins* / genetics
  • PrPSc Proteins* / metabolism
  • Prion Diseases* / genetics
  • Prion Diseases* / metabolism
  • Prion Diseases* / pathology
  • Proteostasis Deficiencies* / genetics
  • Proteostasis Deficiencies* / metabolism
  • Proteostasis Deficiencies* / pathology


  • PrPC Proteins
  • PrPSc Proteins