Results 71 to 80 of about 461,407 (167)

Proteinase K-Resistant Material in ARR/VRQ Sheep Brain Affected with Classical Scrapie Is Composed Mainly of VRQ Prion Protein [PDF]

, 2011
Classical scrapie is a prion disease in sheep and goats. In sheep, susceptibility to disease is genetically influenced by single amino acid substitutions.
Bossers, A., A. Bossers, T. Sklaviadis, I. Lantier, F. Lantier, L. J. M. van Keulen, Van Keulen, L., S. McCutcheon, Berthon, P., Lantier, Isabelle, van Keulen, L.J.M., van Zijderveld, F.G., P. Berthon, Lantier, Frédéric, F., Zijderveld, F.G., van, Jacobs, J.G., Keulen, L., Van, Langeveld, J.P.M., Lantier, I., H. Rezaei, J. P. M. Langeveld, Rezaei, Human, Sklaviadis, T., Berthon, Patricia, J. G. Jacobs, F. G. van Zijderveld, Lantier, F., McCutcheon, S., Rezaei, H.   +28 more
core   +1 more source

Effect of incineration, co‐incineration and combustion on TSE hazards in category 1 animal by‐products

EFSA Journal, Volume 23, Issue 5, May 2025.
Abstract The European Commission requested EFSA to assess the effect of incineration, co‐incineration and combustion of Category 1 animal by‐products (ABP) on the BSE/TSE hazards in ash resulting from these treatments. The presence of residual TSE hazards is assessed by detection of prion infectivity or seeding activity.
EFSA Panel on Biological Hazards (BIOHAZ), Ana Allende, Avelino Alvarez‐Ordóñez, Valeria Bortolaia, Sara Bover‐Cid, Alessandra De Cesare, Wietske Dohmen, Laurent Guillier, Lieve Maria Herman, Liesbeth Jacxsens, Lapo Mughini‐Gras, Maarten Nauta, Jakob Ottoson, Luisa Peixe, Fernando Perez‐Rodriguez, Panagiotis Skandamis, Elisabetta Suffredini, Olivier Andreoletti, Vincent Béringue, John Griffin, Marion Simmons, Kamela Kryemadhi, Barbara Lanfranchi, Angel Ortiz‐Pelaez, Romolo Nonno   +24 more
wiley   +1 more source

Canine D163-PrP polymorphic variant does not provide complete protection against prion infection in small ruminant PrP context

Scientific Reports, 2021
E/D163 polymorphism of dog prion protein (PrP) has been recently proposed as the variant responsible for canid prion resistance. To further investigate the protective role of this variant against prion replication, the transgenic mouse model OvPrP-Tg532 ...
Alba Marín-Moreno, Juan Carlos Espinosa, Patricia Aguilar-Calvo, Natalia Fernández-Borges, José Luis Pitarch, Lorenzo González, Juan María Torres   +6 more
doaj   +1 more source

The role of host PrP in Transmissible Spongiform Encephalopathies [PDF]

, 2007
PrP has a central role in the Transmissible Spongiform Encephalopathies (TSEs), and mutations and polymorphisms in host PrP can profoundly alter the host's susceptibility to a TSE agent.
Barron, Rona M, Wiseman, Frances, Barron, Rona, Hart, Patricia, Manson, Jean C., Wiseman, Patricia, Cancellotti, Enrico, Bishop, Matthew T., Barron, Rona M., Bishop, Matthew T, Manson, Jean C   +10 more
core   +1 more source

Prion Protein Endoproteolysis: Cleavage Sites, Mechanisms and Connections to Prion Disease

Journal of Neurochemistry, Volume 169, Issue 1, January 2025.
Endoproteolytic cleavage events affect the ability of the cellular prion protein (PrPC) to serve as a substrate for template‐mediated conversion into a misfolded isoform denoted PrPSc, a process integral to the pathogenesis of the fatal neurodegenerative disorders either known as transmissible spongiform encephalopathies or as prion diseases.
Andrew R. Castle, David Westaway
wiley   +1 more source

Species barrier as molecular basis for adaptation of synthetic prions with N‐terminally truncated PrP

The FEBS Journal, Volume 291, Issue 22, Page 5051-5076, November 2024.
Recombinant prion protein (PrP) assemblies from different primary mammalian structures can transmit and exhibit divergent adaptation processes and strain properties over passaging in transgenic mice expressing hamster PrP, mimicking the genuine evolutionary properties of prions across species barriers.
Human Rezaei, Davy Martin, Laetitia Herzog, Fabienne Reine, Alba Marín Moreno, Mohammed Moudjou, Naima Aron, Angélique Igel, Hannah Klute, Stella Youssafi, Jean‐Baptiste Moog, Pierre Sibille, Olivier Andréoletti, Joan Torrent, Vincent Béringue   +14 more
wiley   +1 more source

Atypical BSE (BASE) transmitted from asymptomatic aging cattle to a primate. [PDF]

PLoS ONE, 2008
BACKGROUND: Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform Encephalopathy (cBSE).
Emmanuel E Comoy, Cristina Casalone, Nathalie Lescoutra-Etchegaray, Gianluigi Zanusso, Sophie Freire, Dominique Marcé, Frédéric Auvré, Marie-Magdeleine Ruchoux, Sergio Ferrari, Salvatore Monaco, Nicole Salès, Maria Caramelli, Philippe Leboulch, Paul Brown, Corinne I Lasmézas, Jean-Philippe Deslys   +15 more
doaj   +1 more source

A study of the incubation period, or age at onset, of the transmissible spongiform encephalopathies/prion diseases. [PDF]

In order to model epidemics of infectious diseases, particularly to estimate probable numbers of cases with onset at any particular time, it is necessaiy to incorporate a term for the incubation period frequency distribution. Sartwell's hypothesis states
Wooldridge, Marion Joan Anstee
core   +1 more source

Variability in disease phenotypes within a single PRNP genotype suggests the existence of multiple natural sheep scarpie strains within Europe [PDF]

, 2010
Variability of pathological phenotypes within classical sheep scrapie cases has been reported for some time, but in many instances it has been attributed to differences in the PRNP genotype of the host.
González, L., E. Monleon, L. J. M. van Keulen, Jeffrey, M., Balkema-Buschmann, A., C. Hoffmann, Badiola, J.J., Lulini, B., Keulen, L.J.M., van, van Keulen, L.J.M., A. Balkema-Buschmann, Monleón, E., S. Siso, A. Ortiz-Pelaez, Langeveld, J.P.M., Hoffmann, C., J. J. Badiola, Sisó, S., Acín, C., Ortiz-Peláez, A., J. P. M. Langeveld, B. Iulini, M. Jeffrey, L. Gonzalez, C. Casalone, Casalone, C., C. Acin   +26 more
core   +1 more source

Evaluation of the application of Slovenia to be recognised as having a negligible risk of classical scrapie

EFSA Journal, Volume 22, Issue 10, October 2024.
Abstract Slovenia submitted a request to the European Commission to be recognised as a Member State with negligible risk of classical scrapie. EFSA has been asked to assess if Slovenia has demonstrated that, between 2016 and 2022, a sufficient number of ovine and caprine animals over 18 months old, representative of those slaughtered, culled or found ...
European Food Safety Authority (EFSA), Giulio Di Piazza, Giuseppe Ru, Marion Simmons, Barbara Lanfranchi, Angel Ortiz‐Peláez   +5 more
wiley   +1 more source