title: Prion degradation pathways: Potential for therapeutic intervention.
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'.
authors.affiliation: Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, United Kingdom.
authors.forename: Rob
authors.initials: R
authors.lastname: Goold
authors.affiliation: Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, United Kingdom.
authors.forename: Chris
authors.initials: C
authors.lastname: McKinnon
authors.affiliation: Department of Neurodegenerative Disease, UCL Institute of Neurology, University College London, United Kingdom. Electronic address: s.tabrizi@prion.ucl.ac.uk.
authors.forename: Sarah J
authors.initials: SJ
authors.lastname: Tabrizi
mesh_terms: D000818:Animals; D006801:Humans; D017096:Prion Diseases / Q000378:metabolism* / Q000628:therapy*; D011328:Prions / Q000378:metabolism*; D015398:Signal Transduction / Q000235:genetics / Q000502:physiology*
chemical_list: D011328:Prions
keywords: Autophagy; Lysosomal degradation; PrP(Sc); Prion disease; Proteasome; Therapeutics