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Prion degradation pathways: Potential for therapeutic intervention

Goold, R; McKinnon, C; Tabrizi, SJ; (2015) Prion degradation pathways: Potential for therapeutic intervention. Molecular and Cellular Neuroscience , 66 (Part A) pp. 12-20. 10.1016/j.mcn.2014.12.009. Green open access

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Abstract

Prion diseases are fatal neurodegenerative disorders. Pathology is closely linked to the misfolding of native cellular PrP(C) into the disease-associated form PrP(Sc) that accumulates in the brain as disease progresses. Although treatments have yet to be developed, strategies aimed at stimulating the degradation of PrP(Sc) have shown efficacy in experimental models of prion disease. Here, we describe the cellular pathways that mediate PrP(Sc) degradation and review possible targets for therapeutic intervention. This article is part of a Special Issue entitled 'Neuronal Protein'.

Type: Article
Title: Prion degradation pathways: Potential for therapeutic intervention
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.mcn.2014.12.009
Publisher version: http://dx.doi.org/10.1016/j.mcn.2014.12.009
Language: English
Additional information: © 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
Keywords: Autophagy, Lysosomal degradation, PrP(Sc), Prion disease, Proteasome, Therapeutics
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/1465361
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