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Insights into Mechanisms of Chronic Neurodegeneration

The Roslin Institute and R(D)SVS, University of Edinburgh, Easter Bush, Midlothian EH25 9RG, UK
National CJD Research and Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH8 9JU, UK
Author to whom correspondence should be addressed.
Academic Editor: Kurt A. Jellinger
Int. J. Mol. Sci. 2016, 17(1), 82;
Received: 30 November 2015 / Revised: 22 December 2015 / Accepted: 23 December 2015 / Published: 12 January 2016
(This article belongs to the Special Issue Mechanisms of Neurodegeneration)
Chronic neurodegenerative diseases such as Alzheimer’s disease (AD), Parkinson’s disease (PD), and prion diseases are characterised by the accumulation of abnormal conformers of a host encoded protein in the central nervous system. The process leading to neurodegeneration is still poorly defined and thus development of early intervention strategies is challenging. Unique amongst these diseases are Transmissible Spongiform Encephalopathies (TSEs) or prion diseases, which have the ability to transmit between individuals. The infectious nature of these diseases has permitted in vivo and in vitro modelling of the time course of the disease process in a highly reproducible manner, thus early events can be defined. Recent evidence has demonstrated that the cell-to-cell spread of protein aggregates by a “prion-like mechanism” is common among the protein misfolding diseases. Thus, the TSE models may provide insights into disease mechanisms and testable hypotheses for disease intervention, applicable to a number of these chronic neurodegenerative diseases. View Full-Text
Keywords: neurodegeneration; prion; transmissible spongiform encephalopathies (TSE); protein misfolding; proteinopathies neurodegeneration; prion; transmissible spongiform encephalopathies (TSE); protein misfolding; proteinopathies
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Diack, A.B.; Alibhai, J.D.; Barron, R.; Bradford, B.; Piccardo, P.; Manson, J.C. Insights into Mechanisms of Chronic Neurodegeneration. Int. J. Mol. Sci. 2016, 17, 82.

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