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Prion degradation pathways: Potential for therapeutic intervention [PDF]
, 2015 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
Goold, R, McKinnon, C, Tabrizi, SJ
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Expression Pattern of a Mini Human PrP Gene Promoter in Transgenic Mice
Neurobiology of Disease, 2002 The prion protein is central to the pathogenesis of prion diseases, although its exact function remains unclear. Although transgenic mice have been widely utilised in prion research, their PrP expression patterns have not been characterised in detail. We
E.A. Asante +4 more
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Genome-wide association study of behavioural and psychiatric features in human prion disease. [PDF]
, 2015 Prion diseases are rare neurodegenerative conditions causing highly variable clinical syndromes, which often include prominent neuropsychiatric symptoms.
Carswell, C +9 more
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Seminars in Neurology, 2012 Prion diseases are a group of diseases caused by abnormally conformed infectious proteins, called prions. They can be sporadic (Jakob-Creutzfeldt disease [JCD]), genetic (genetic JCD, Gerstmann-Sträussler-Scheinker, and familial fatal insomnia), or acquired (kuru, variant JCD, and iatrogenic JCD).
Takada, Leonel, Geschwind, Michael
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BMC Bioinformatics, 2018 Background Influenza A Virus (IAV) causes respiratory disease in swine and is a zoonotic pathogen. Uncontrolled IAV in swine herds not only affects animal health, it also impacts production through increased costs associated with treatment and prevention
Michael A Zeller +4 more
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Veterinary Research, 2018 In animal prion diseases, including bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease in cervids, and scrapie in sheep and goats, a disease-associated isoform of prion protein (PrPd) accumulates in the brains of affected animals ...
Kohtaro Miyazawa +5 more
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Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments [PDF]
, 2019 Despite their phenotypic heterogeneity, most human prion diseases belong to two broadly defined groups: Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker disease (GSS).
Cali, Ignazio +8 more
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PrPCWD lymphoid cell targets in early and advanced chronic wasting disease of mule deer [PDF]
, 2002 Up to 15% of free-ranging mule deer in northeastern Colorado and southeastern Wyoming, USA, are afflicted with a prion disease, or transmissible spongiform encephalopathy (TSE), known as chronic wasting disease (CWD).
Barillas-Mury, C. +6 more
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Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding [PDF]
, 2010 Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP. These diseases can be hereditary in humans and four of the many disease-associated missense mutants of PrP are in the ...
Daggett, Valerie, van der Kamp, Marc W
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A Retrospective Cohort Study of a Newly Proposed Criteria for Sporadic Creutzfeldt–Jakob Disease
DiagnosticsBackground/Objectives: Sporadic Creutzfeldt–Jakob disease (sCJD) is a fatal neurodegenerative disorder traditionally diagnosed based on the World Health Organization (WHO) criteria in 1998. Recently, Hermann et al.
Toshiaki Nonaka +29 more
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