Results 71 to 80 of about 1,549,882 (213)

Adaptive selection of a prion strain conformer corresponding to established North American CWD during propagation of novel emergent Norwegian strains in mice expressing elk or deer prion protein.

PLoS Pathogens, 2021
Prions are infectious proteins causing fatal, transmissible neurodegenerative diseases of animals and humans. Replication involves template-directed refolding of host encoded prion protein, PrPC, by its infectious conformation, PrPSc.
Jifeng Bian, Sehun Kim, Sarah J Kane, Jenna Crowell, Julianna L Sun, Jeffrey Christiansen, Eri Saijo, Julie A Moreno, James DiLisio, Emily Burnett, Sandra Pritzkow, Damian Gorski, Claudio Soto, Terry J Kreeger, Aru Balachandran, Gordon Mitchell, Michael W Miller, Romolo Nonno, Turid Vikøren, Jørn Våge, Knut Madslien, Linh Tran, Tram Thu Vuong, Sylvie L Benestad, Glenn C Telling   +24 more
doaj   +1 more source

Exploring and Targeting the Connection of Iron and Copper Homeostasis to Neurodegenerative Diseases

MedComm, Volume 7, Issue 6, June 2026.
Iron and copper dyshomeostasis, along with their interactions with key intrinsically disordered proteins (e.g., Aβ, tau, α‐synuclein) have a strong implication in the onset and progression of neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), Prion diseases (PrDs), Huntington's disease (HD), Wilson's disease (WD),
Xin Liu, Lin Jia, Kerong Wu, Mo Chen, Jichao Sun, Chuanbin Yang, Chengchao Xu, Jie Sun, Jigang Wang, Lingyun Dai   +9 more
wiley   +1 more source

Prion protein gene polymorphisms and accumulation of pathogenic prion protein (PrPSc) in a herd with previously confirmed scrapie cases

Bioscience Journal, 2015
Scrapie in sheep is associated with at least three polymorphisms in the prion protein gene (PRNP) on codons 136, 154, and 171. Countries where scrapie is endemic have been using breeding programs based on selection for the most resistant alleles. There are some PRNP genotyping data on sheep in Brazil, and scrapie has sporadically been observed since ...
Sotomaior, Cristina Santos, Driemeier, David, Leal, Juliano, Ollhoff, Rüdiger Daniel, Thomaz-Soccol, Vanete   +4 more
openaire   +5 more sources

Enhanced Sensitivity of a Modified Quaking‐Induced Conversion Diagnostic Test for the Broad Detection of Sporadic and Inherited Prion Diseases: A Retrospective Study

Annals of Neurology, Volume 99, Issue 5, Page 1303-1314, May 2026.
Objective Quaking‐induced conversion (QuIC) tests, which detect prion‐seeding activity in cerebrospinal fluid (CSF), have markedly advanced the antemortem diagnosis of prion diseases such as Creutzfeldt‐Jakob disease (CJD). These tests provide high diagnostic accuracy and enable timely differentiation from other rapidly progressive neurodegenerative ...
Jennifer Myskiw, Rebecca Fox, Dominic M.S. Kielich, Lise Lamoureux, Melanie Leonhardt, Olivia Nykvist, Jessy A. Slota, Kristen Avery, Clark Phillipson, Kathy Frost, Sharon Simon, Brian S. Appleby, Ben A. Bailey‐Elkin, Stephanie A. Booth   +13 more
wiley   +1 more source

Variably Protease‐Sensitive Prionopathy: Two New Cases With Motor Neuron‐Dementia Syndrome

Annals of Clinical and Translational Neurology, Volume 13, Issue 4, Page 827-833, April 2026.
ABSTRACT We describe two patients with variably protease‐sensitive prionopathy (VPSPr) who developed progressive upper motor neuron symptoms, insomnia, behavioral and cognitive decline, compatible with primary lateral sclerosis associated with frontotemporal dementia (FTD).
María Elena Erro, María Victoria Zelaya, Hasier Eraña, Javier Sánchez Ruiz de Gordoa, Fermín García‐Amigot, Anika Simonovska‐Serra, María Cristina Caballero, Isidre Ferrer, Ellen Gelpi, Ivonne Jericó, Joaquín Castilla   +10 more
wiley   +1 more source

Prions in Milk from Ewes Incubating Natural Scrapie [PDF]

, 2008
Since prion infectivity had never been reported in milk, dairy products originating from transmissible spongiform encephalopathy (TSE)-affected ruminant flocks currently enter unrestricted into the animal and human food chain.
Bergonier Dominique, Fabien Corbière, Lacroux, Caroline, Cassard Hervé, Grassi, Jacques, Andréoletti Olivier, Torffin Moldal, Sylvie L. Benestad, Schelcher, Francois, F., Lugan, Séverine, S., Costes, Pierrette, Benestad Sylvie L., Simmons Hugh, Sylvie L Benestad, Nathalie Morel, Lantier, Frédéric, F., Simon Stéphanie, Lacroux Caroline, Weisbecker, Jean Louis, J. L., Benestad, Sylvie, Hervé Cassard, Bergonier, Dominique, Schelcher, François, Weisbecker, Jean-Louis, Morel, Nathalie, Weisbecker Jean-Louis, Lantier Frederic, Moldal, Torffin, François Schelcher, Stéphanie Simon, Simmons, Hugh, Dominique Bergonier, Morell, Nathalie, Jacques Grassi, Olivier Andréoletti, Schelcher François, Cécile Feraudet-Tarisse, Andréoletti, Olivier, Pierrette Costes, Cassard, Hervé, H., Corbière Fabien, Caroline Lacroux, Maillet, Séverinne, Mathey, Jacinthe, Jean-Louis Weisbecker, Mathey Jacinthe, Hugh Simmons, Cassard, Hervé, Corbière, Fabien, Féraudet-Tarisse, Cécile, Jacinthe Mathey, Feraudet-Tarisse Cécile, Frederic Lantier, Simon, Stéphanie, Séverine Lugan, Séverine Maillet, Morel Nathalie, Costes Pierrette, Costes, Pierrette, P., Lantier, Frédéric, Grassi Jacques, Benestad, Sylvie L, Lugan Séverine, Maillet, Séverine, Moldal Torffin, Lugan, Sévérine, Féraudet, Cécile, Lacroux, Caroline, C., Maillet Séverine   +68 more
core   +2 more sources

Identification of Candidate Proteins Binding to Prion Protein

Neurobiology of Disease, 1997
Prion diseases are disorders of protein conformation that produce neurodegeneration in humans and animals. Studies of transgenic (Tg) mice indicate that a factor designated protein X is involved in the conversion of the normal cellular prion protein ...
Fruma Yehiely, Paul Bamborough, Maria Da Costa, Billie J. Perry, Gopal Thinakaran, Fred E. Cohen, George A. Carlson, Stanley B. Prusiner   +7 more
doaj   +1 more source

The Prion Basis of Progressive Neurodegenerative Disorders

Interdisciplinary Perspectives on Infectious Diseases, 2023
The discovery of proteinaceous infectious agents by Prusiner in 1982 was sensational. All previously known pathogens contained nucleic acids, the code of life, that enabled them to reproduce. In contrast, the proteinaceous agents of disease, called prion
Tvisha Joshi, Nidhi Ahuja
doaj   +1 more source

The Phenotypic Spectrum of Sporadic Creutzfeldt‐Jakob Disease Cortical Subtype

Annals of Neurology, Volume 99, Issue 4, Page 883-896, April 2026.
Objective The objective of this study was to characterize the phenotypic spectrum of the rare sporadic Creutzfeldt‐Jakob disease cortical subtype (sCJDMM/MV2C) in a large multicentric autopsy cohort. Methods We evaluated clinical histories, biofluid markers, brain diffusion‐weighted (DW)‐magnetic resonance imaging (MRI), and electroencephalogram (EEG ...
Simone Baiardi, Claudia Marina Vargiu, Brian S. Appleby, Marcelo Barria, Giuseppe Mario Bentivenga, Ignazio Calì, Benedetta Carlà, Mark Cohen, Armin Giese, Jochen Herms, Aino‐Minerva Kortelainen, Anna Ladogana, Angela Mammana, Diane Ritchie, Otto Windl, Sabina Capellari, Piero Parchi   +16 more
wiley   +1 more source

Recombinant PrP and Its Contribution to Research on Transmissible Spongiform Encephalopathies

Pathogens, 2017
The misfolding of the cellular prion protein (PrPC) into the disease-associated isoform (PrPSc) and its accumulation as amyloid fibrils in the central nervous system is one of the central events in transmissible spongiform encephalopathies (TSEs). Due to
Jorge M. Charco, Hasier Eraña, Vanessa Venegas, Sandra García-Martínez, Rafael López-Moreno, Ezequiel González-Miranda, Miguel Ángel Pérez-Castro, Joaquín Castilla   +7 more
doaj   +1 more source