Phenotypic variability of sporadic human prion disease and its molecular basis: past, present, and future

P Parchi, R Strammiello, A Giese, H Kretzschmar - Acta neuropathologica, 2011 - Springer
Human prion diseases are rare neurodegenerative disorders related to prion protein
misfolding that can occur as sporadic, familial or acquired forms. In comparison to other …

Zoonotic risks from small ruminants

M Ganter - Veterinary microbiology, 2015 - Elsevier
Zoonoses are infections that spread naturally between species (sometimes by a vector) from
animals to other animal species or to humans or from humans to animals. Most of the …

Distinct molecular phenotypes in bovine prion diseases

AG Biacabe, JL Laplanche, S Ryder, T Baron - EMBO reports, 2004 - embopress.org
Bovine spongiform encephalopathy (BSE) in cattle, the most likely cause of variant
Creutzfeldt–Jakob disease in humans, is thought to be caused by a unique infectious agent …

Differentiation of prion protein glycoforms from naturally occurring sheep scrapie, sheep-passaged scrapie strains (CH1641 and SSBP1), bovine spongiform …

M Stack, M Chaplin, J Clark - Acta neuropathologica, 2002 - Springer
A panel of ruminant brain tissues were subjected to a Western immunoblotting technique
using two monoclonal antibodies (mAbs). The resultant prion protein (PrP) glycoforms …

Sheep and goat BSE propagate more efficiently than cattle BSE in human PrP transgenic mice

D Padilla, V Béringue, JC Espinosa… - PLoS …, 2011 - journals.plos.org
A new variant of Creutzfeldt Jacob Disease (vCJD) was identified in humans and linked to
the consumption of Bovine Spongiform Encephalopathy (BSE)-infected meat products …

Atypical scrapie cases in Germany and France are identified by discrepant reaction patterns in BSE rapid tests

A Buschmann, AG Biacabe, U Ziegler, A Bencsik… - Journal of virological …, 2004 - Elsevier
The intensified surveillance of scrapie in small ruminants in the European Union (EU) has
resulted in a substantial increase of the number of diagnosed cases. Four rapid tests which …

Dominant-negative inhibition of prion replication in transgenic mice

V Perrier, K Kaneko, J Safar… - Proceedings of the …, 2002 - National Acad Sciences
Our discovery of dominant-negative inhibition of prion formation in cultured cells provided an
explanation for the resistance of some sheep to scrapie and humans to Creutzfeldt–Jakob …

Prion strains: shining new light on old concepts

AJ Block, JC Bartz - Cell and Tissue Research, 2023 - Springer
Prion diseases are a group of inevitably fatal neurodegenerative disorders affecting
numerous mammalian species, including humans. The existence of heritable phenotypes of …

Effects of different experimental conditions on the PrPSc core generated by protease digestion: implications for strain typing and molecular classification of CJD

S Notari, S Capellari, A Giese, I Westner… - Journal of Biological …, 2004 - ASBMB
The discovery of molecular subtypes of the pathological prion protein PrP Sc has provided
the basis for a novel classification of human transmissible spongiform encephalopathies …

Rapid and discriminatory diagnosis of scrapie and BSE in retro-pharyngeal lymph nodes of sheep

JPM Langeveld, JG Jacobs, JHF Erkens… - BMC veterinary …, 2006 - Springer
Background Diagnosis based on prion detection in lymph nodes of sheep and goats can
improve active surveillance for scrapie and, if it were circulating, for bovine spongiform …