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Viruses 2019, 11(4), 309; https://doi.org/10.3390/v11040309

Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans

1
Department of Experimental, Diagnostic and Specialty Medicine (DIMES), University of Bologna, 40138 Bologna, Italy
2
IRCCS Istituto delle Scienze Neurologiche di Bologna, 40139 Bologna, Italy
3
Department of Biomedical and Neuromotor Sciences, University of Bologna, 40123 Bologna, Italy
*
Author to whom correspondence should be addressed.
Received: 17 February 2019 / Revised: 25 March 2019 / Accepted: 26 March 2019 / Published: 29 March 2019
(This article belongs to the Special Issue Deciphering the Molecular Targets of Prion and Prion-Like Strains)
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Abstract

Prion diseases are a unique group of rare neurodegenerative disorders characterized by tissue deposition of heterogeneous aggregates of abnormally folded protease-resistant prion protein (PrPSc), a broad spectrum of disease phenotypes and a variable efficiency of disease propagation in vivo. The dominant clinicopathological phenotypes of human prion disease include Creutzfeldt–Jakob disease, fatal insomnia, variably protease-sensitive prionopathy, and Gerstmann–Sträussler–Scheinker disease. Prion disease propagation into susceptible hosts led to the isolation and characterization of prion strains, initially operatively defined as “isolates” causing diseases with distinctive characteristics, such as the incubation period, the pattern of PrPSc distribution, and the regional severity of neuropathological changes after injection into syngeneic hosts. More recently, the structural basis of prion strains has been linked to amyloid polymorphs (i.e., variant amyloid protein conformations) and the concept extended to all protein amyloids showing polymorphic structures and some evidence of in vivo or in vitro propagation by seeding. Despite the significant advances, however, the link between amyloid structure and disease is not understood in many instances. Here we reviewed the most significant contributions of human prion disease studies to current knowledge of the molecular basis of phenotypic variability and the prion strain phenomenon and underlined the unsolved issues from the human disease perspective. View Full-Text
Keywords: prion; strain; CreutzfeldtJakob disease; human prion disease; VPSPr; GSS; fatal insomnia; experimental transmission prion; strain; CreutzfeldtJakob disease; human prion disease; VPSPr; GSS; fatal insomnia; experimental transmission
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Rossi, M.; Baiardi, S.; Parchi, P. Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans. Viruses 2019, 11, 309.

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