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How do PrPSc Prions Spread between Host Species, and within Hosts?
Open AccessReview

What Is Our Current Understanding of PrPSc-Associated Neurotoxicity and Its Molecular Underpinnings?

MRC Toxicology Unit, Hodgkin Building, University of Leicester, Lancaster Road, Leicester LE1 9HN, UK
Department of Clinical Neurosciences, University of Cambridge, Cambridge Biomedical Campus, Cambridge CB2 0AH, UK
Author to whom correspondence should be addressed.
Pathogens 2017, 6(4), 63;
Received: 29 September 2017 / Revised: 21 November 2017 / Accepted: 27 November 2017 / Published: 1 December 2017
(This article belongs to the Special Issue PrPSc prions: state of the art)
The prion diseases are a collection of fatal, transmissible neurodegenerative diseases that cause rapid onset dementia and ultimately death. Uniquely, the infectious agent is a misfolded form of the endogenous cellular prion protein, termed PrPSc. Despite the identity of the molecular agent remaining the same, PrPSc can cause a range of diseases with hereditary, spontaneous or iatrogenic aetiologies. However, the link between PrPSc and toxicity is complex, with subclinical cases of prion disease discovered, and prion neurodegeneration without obvious PrPSc deposition. The toxic mechanisms by which PrPSc causes the extensive neuropathology are still poorly understood, although recent advances are beginning to unravel the molecular underpinnings, including oxidative stress, disruption of proteostasis and induction of the unfolded protein response. This review will discuss the diseases caused by PrPSc toxicity, the nature of the toxicity of PrPSc, and our current understanding of the downstream toxic signaling events triggered by the presence of PrPSc. View Full-Text
Keywords: prion disease; neurodegeneration; neurotoxicity; proteostasis; PrPSc prion disease; neurodegeneration; neurotoxicity; proteostasis; PrPSc
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Hughes, D.; Halliday, M. What Is Our Current Understanding of PrPSc-Associated Neurotoxicity and Its Molecular Underpinnings? Pathogens 2017, 6, 63.

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