Overexpression of bank vole PrP(I109) in mice induces a spontaneous atypical prion disease with sex-dependent onset, early NfL elevation, and universal prion strain permissiveness [PDF]
Acta Neuropathologica CommunicationsTransgenic mice overexpressing bank vole prion protein with the isoleucine 109 polymorphism, TgVole(I109)4x, develop spontaneous neurodegenerative disease with sex-dependent onset, averaging 170 days in females and 200 days in males at terminal stage ...
Hasier Eraña +31 more
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Large-scale lipidomic profiling identifies novel potential biomarkers for prion diseases and highlights lipid raft-related pathways [PDF]
Veterinary Research, 2021 Prion diseases are transmissible spongiform encephalopathies induced by the abnormally-folded prion protein (PrPSc), which is derived from the normal prion protein (PrPC).
Yong-Chan Kim +3 more
doaj +4 more sources
Detection of Chronic Wasting Disease Prions in Raw, Processed, and Cooked Elk Meat, Texas, USA [PDF]
Emerging Infectious DiseasesWe describe chronic wasting disease (CWD) prion detection in raw and cooked meat from a CWD-positive elk. We found limited zoonotic potential in CWD prions from those meat products.
Rebeca Benavente +8 more
doaj +2 more sources
Detection of Prions in Wild Pigs (Sus scrofa) from Areas with Reported Chronic Wasting Disease Cases, United States [PDF]
Emerging Infectious DiseasesUsing a prion amplification assay, we identified prions in tissues from wild pigs (Sus scrofa) living in areas of the United States with variable chronic wasting disease (CWD) epidemiology.
Paulina Soto +14 more
doaj +2 more sources
Creutzfeldt-Jakob Disease Incidence, South Korea, 2001–2019
Emerging Infectious Diseases, 2022 We found increasing trends of Creutzfeldt-Jakob disease (CJD) cases and annual incidence in South Korea during 2001–2019. We noted relatively low (5.7%) distribution of familial CJD.
Yong-Chan Kim, Byung-Hoon Jeong
doaj +1 more source
Immunomodulation for prion and prion-related diseases [PDF]
Expert Review of Vaccines, 2010 Prion diseases are a unique category of illness, affecting both animals and humans, where the underlying pathogenesis is related to a conformational change of a normal self protein called cellular prion protein to a pathological and infectious conformer known as scrapie prion protein (PrP(Sc)). Currently, all prion diseases lack effective treatment and
Thomas, Wisniewski, Fernando, Goñi
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Human J-Domain Protein DnaJB6 Protects Yeast from [PSI+] Prion Toxicity
Biology, 2022 Human J-domain protein (JDP) DnaJB6 has a broad and potent activity that prevents formation of amyloid by polypeptides such as polyglutamine, A-beta, and alpha-synuclein, related to Huntington’s, Alzheimer’s, and Parkinson’s diseases, respectively.
Richard E. Dolder +3 more
doaj +1 more source
Stability and Cu(II) Binding of Prion Protein Variants Related to Inherited Human Prion Diseases [PDF]
Biophysical Journal, 2003 All inherited forms of human prion diseases are linked with mutations in the prion protein (PrP) gene. Here we have investigated the stability and Cu(II) binding properties of three recombinant variants of murine full-length PrP(23-231)-containing destabilizing point mutations that are associated with human Gerstmann-Sträussler-Scheinker disease (F198S)
Cereghetti, Grazia M. +3 more
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Uptake, Retention, and Excretion of Infectious Prions by Experimentally Exposed Earthworms
Emerging Infectious Diseases, 2021 Prions are proteinaceous infectious agents that can be transmitted through various components of the environment, including soil particles. We found that earthworms exposed to prion-contaminated soil can bind, retain, and excrete prions, which remain ...
Sandra Pritzkow +3 more
doaj +1 more source
THERPA: A small molecule database related to prion protein regulation and prion diseases progression [PDF]
Prion, 2018 Prion diseases are fatal neurodegenerative disorders that affect humans and animals. Although various small molecules have been evaluated for application in the treatment of prion diseases, none have been shown to be efficacious. Expanding our knowledge of these molecules is important for understanding of the complex mechanisms of prion diseases.
Sol Moe Lee +6 more
openaire +2 more sources