Prion Diseases pp 209-228 | Cite as

Synthetic Mammalian Prions

  • Fabio Moda
  • Edoardo Bistaffa
  • Joanna Narkiewicz
  • Giulia Salzano
  • Giuseppe LegnameEmail author
Part of the Neuromethods book series (NM, volume 129)


We describe a detailed method to generate different synthetic prions characterized by defined abnormal structures, which confer to each isolate specific infectious properties. When challenged in vitro or in vivo some of these isolates were able to cause illness and produced pathological features similar to that observed in animals or human with naturally occurring prion diseases, including the sporadic form of the Creutzfeldt–Jakob disease. Thus, synthetic prions are of fundamental importance to shed light on the intricate molecular events leading to the misfolding of the normal prion protein. Understanding prion conversion mechanism could allow designing therapeutic strategies aimed at blocking this process.

Key words

Synthetic prions Prion protein Fibrillization Aggregation Amyloid 


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Copyright information

© Springer Science+Business Media LLC 2017

Authors and Affiliations

  • Fabio Moda
    • 1
  • Edoardo Bistaffa
    • 1
    • 2
  • Joanna Narkiewicz
    • 2
  • Giulia Salzano
    • 2
  • Giuseppe Legname
    • 2
    Email author
  1. 1.IRCCS Foundation Carlo Besta Neurological InstituteMilanItaly
  2. 2.Scuola Internazionale Superiore di Studi Avanzati (SISSA)TriesteItaly

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