The Prion Concept and Synthetic Prions

Giuseppe Legname, Fabio Moda

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

© 2017 Elsevier Inc. Transmissible spongiform encephalopathies or prion diseases are a group of fatal neurodegenerative diseases caused by unconventional infectious agents, known as prions (PrP Sc ). Prions derive from a conformational conversion of the normally folded prion protein (PrP C ), which acquires pathological and infectious features. Moreover, PrP Sc is able to transmit the pathological conformation to PrP C through a mechanism that is still not well understood. The generation of synthetic prions, which behave like natural prions, is of fundamental importance to study the process of PrP C conversion and to assess the efficacy of therapeutic strategies to interfere with this process. Moreover, the ability of synthetic prions to induce pathology in animals confirms that the pathological properties of the prion strains are all enciphered in abnormal conformations, characterizing these infectious agents.
Original languageEnglish
Title of host publicationProgress in Molecular Biology and Translational Science
Pages147-156
Number of pages10
Volume150
DOIs
Publication statusPublished - Jan 1 2017

Keywords

  • Amyloids
  • Prion protein
  • Protein aggregation
  • Synthetic prions

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