Molecular mechanisms of prion pathogenesis

A Aguzzi, C Sigurdson… - Annu. Rev. Pathol. Mech …, 2008 - annualreviews.org
Prion diseases are infectious neurodegenerative diseases occurring in humans and animals
with an invariably lethal outcome. One fundamental mechanistic event in prion diseases is …

Atypical/Nor98 scrapie: properties of the agent, genetics, and epidemiology

S Benestad, JN Arsac, W Goldmann, M Nöremark - Veterinary research, 2008 - hal.science
Atypical/Nor98 scrapie cases in sheep were diagnosed for the first time in Norway in 1998.
They are now identified in small ruminants in most European countries and represent an …

Packaging of prions into exosomes is associated with a novel pathway of PrP processing

LJ Vella, RA Sharples, VA Lawson… - The Journal of …, 2007 - Wiley Online Library
Prion diseases are fatal, transmissible neurodegenerative disorders associated with
conversion of the host‐encoded prion protein (PrPC) into an abnormal pathogenic isoform …

A novel human disease with abnormal prion protein sensitive to protease

P Gambetti, Z Dong, J Yuan, X Xiao… - Annals of Neurology …, 2008 - Wiley Online Library
Objective To report a novel prion disease characterized by distinct histopathological and
immunostaining features, and associated with an abnormal isoform of the prion protein (PrP) …

The physical relationship between infectivity and prion protein aggregates is strain-dependent

P Tixador, L Herzog, F Reine, E Jaumain… - PLoS …, 2010 - journals.plos.org
Prions are unconventional infectious agents thought to be primarily composed of PrPSc, a
multimeric misfolded conformer of the ubiquitously expressed host-encoded prion protein …

Protease-sensitive synthetic prions

DW Colby, R Wain, IV Baskakov, G Legname… - PLoS …, 2010 - journals.plos.org
Prions arise when the cellular prion protein (PrPC) undergoes a self-propagating
conformational change; the resulting infectious conformer is designated PrPSc. Frequently …

Update on classical and atypical scrapie in sheep and goats

JJ Greenlee - Veterinary pathology, 2019 - journals.sagepub.com
Scrapie is a naturally occurring transmissible spongiform encephalopathy (TSE) or prion
disease of sheep and goats. Scrapie is a protein misfolding disease where the normal prion …

[HTML][HTML] Similar biochemical signatures and prion protein genotypes in atypical scrapie and Nor98 cases, France and Norway

JN Arsac, O Andreoletti, JM Bilheude… - Emerging infectious …, 2007 - ncbi.nlm.nih.gov
Isolates of atypical scrapie recently identified in sheep and goats in France were compared
with Nor98 isolates reported in Norway. Western blot methods for characterization of the …

Prion strain diversity

JC Bartz - Cold Spring Harbor perspectives in …, 2016 - perspectivesinmedicine.cshlp.org
Prion diseases affect a wide range of mammal species and are caused by a misfolded self-
propagating isoform (PrPSc) of the normal prion protein (PrPC). Distinct strains of prions …

Conventional and state-of-the-art detection methods of bovine spongiform encephalopathy (BSE)

M Olech - International Journal of Molecular Sciences, 2023 - mdpi.com
Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease that belongs
to a group of diseases known as transmissible spongiform encephalopathies (TSEs). It is …