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Title: The Molecular Pathology of Prion Diseases
Authors: Vassallo, Neville
Herms, Jochen
Kretzschmar, Hans A.
Keywords: Prion diseases -- Pathogenesis
Prions -- Research
Creutzfeldt-Jakob syndrome
Prion diseases -- Treatment
Issue Date: 2004
Publisher: Malta Medical Journal
Citation: Malta Medical Journal. 2004, Vol.16(4), p. 15-19
Abstract: Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal cellular prion protein (PrPC) into a pathogenic likeness of itself (PrPSc). Formation of neurotoxic PrPSc and/or loss of the normal function of native PrPC result in activation of cellular pathways ultimately leading to neuronal death. Prion diseases can affect both humans and animals, with scrapie of sheep, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease being the most notable. This review is intended to provide an overview of the salient scientific discoveries in prion research, mainly from a molecular perspective. Further, some of the major outstanding questions in prion science are highlighted. Prion research is having a profound impact on modern medicine, and strategies for prevention and treatment of these disorders may also find application in the more common neurodegenerative diseases.
Appears in Collections:MMJ, Volume 16, Issue 4
MMJ, Volume 16, Issue 4
Scholarly Works - FacM&SPB

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