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Toxic Protein Spread in Neurodegeneration: Reality versus Fantasy


Kara, Eleanna; Marks, Jordan D; Aguzzi, Adriano (2018). Toxic Protein Spread in Neurodegeneration: Reality versus Fantasy. Trends in Molecular Medicine, 24(12):1007-1020.

Abstract

Over the past decade, the importance of the propagation of amyloidogenic proteins such as α-synuclein and tau in the pathogenesis of neurodegenerative diseases has been supported by numerous neuropathological and experimental studies. While these proteins behave similarly to prions, recent evidence suggests the existence of fundamental differences, as they can propagate in the absence of endogenous template, they do not exhibit a strict 'strain' behavior, and they may not be transmissible between individuals. We therefore propose to name these proteins 'prionoids'. In this review we critically assess the extent of the overlap between these two entities and evaluate how the propagation of prionoids can fit into the wider system dysfunction seen in the brains of patients with Alzheimer's and Parkinson's diseases.

Abstract

Over the past decade, the importance of the propagation of amyloidogenic proteins such as α-synuclein and tau in the pathogenesis of neurodegenerative diseases has been supported by numerous neuropathological and experimental studies. While these proteins behave similarly to prions, recent evidence suggests the existence of fundamental differences, as they can propagate in the absence of endogenous template, they do not exhibit a strict 'strain' behavior, and they may not be transmissible between individuals. We therefore propose to name these proteins 'prionoids'. In this review we critically assess the extent of the overlap between these two entities and evaluate how the propagation of prionoids can fit into the wider system dysfunction seen in the brains of patients with Alzheimer's and Parkinson's diseases.

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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Scopus Subject Areas:Life Sciences > Molecular Medicine
Life Sciences > Molecular Biology
Uncontrolled Keywords:Molecular Medicine, Molecular Biology
Language:English
Date:1 December 2018
Deposited On:10 Jan 2019 13:04
Last Modified:29 Jul 2020 08:52
Publisher:Elsevier
ISSN:1471-4914
OA Status:Green
Publisher DOI:https://doi.org/10.1016/j.molmed.2018.09.004
PubMed ID:30442495
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