Synthetic Infectious Prions

Conference paper
Part of the IFMBE Proceedings book series (IFMBE, volume 49)

Abstract

Prion diseases are fatal and incurable neurodegenerative maladies of unique etiology. These disorders have attracted considerable attention for their unique infectivity pathogenic mechanism. Here we describe the milestone studies that have lead to the demonstration that prions are solely constituted of proteins. The formal demonstration has been achieved through several papers describing unconvertible evidence that recombinant prion protein refolded into amyloid fibrils can contain infectious species that lead to disease in animal models of prion coversion and replication. These molecules have been named as synthetic infectious prions.

Keywords

prion protein recombinant proteins prions transgenic mice synthetic prions 

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Copyright information

© IFMBE 2013

Authors and Affiliations

  1. 1.Department of NeurobiologyScuola Internazionale Superiore di Studi Avanzati (SISSA)TriesteItaly

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