The history of prion disease

Francesco M. Galassi, Maciej Henneberg, Frank J. Rühli

Research output: Contribution to journalComment/debate


Prion diseases are fatal spongiform neurodegenerative pathologies caused by the mutant protein PrPSc, that propagates by changing the conformation of its physiological counterpart PrPC. Since Gajdusek's first description of kuru in 1957 and Prusiner's 1982 prion hypothesis, evidence has accumulated on the pathogenesis of prion diseases. However, the role of PrPC was only described in 2016: long-term preservation of neuronal integrity through an interaction with a yet unidentified Schwann cell receptor...
Original languageEnglish
Pages (from-to)1211
Number of pages1
JournalThe Lancet Neurology
Issue number12
Publication statusPublished - 1 Nov 2016


  • Prion diseases
  • neurodegenerative pathologies
  • mutant protein


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