Results 31 to 40 of about 16,662 (259)

Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin [PDF]

Biochemical Journal, 2008
Disease-related PrPSc [pathogenic PrP (prion protein)] is classically distinguished from its normal cellular precursor, PrPC(cellular PrP) by its detergent insolubility and partial resistance to proteolysis. Although molecular diagnosis of prion disease has historically relied upon detection of protease-resistant fragments of PrPSc using PK (proteinase
Cronier, Sabrina, Gros, N, Tattum, Mh, Jackson, Gs, Clarke, Ar, Collinge, J, Wadsworth, Jdf   +6 more
openaire   +4 more sources

Anti-prion drug mPPIg5 inhibits PrP(C) conversion to PrP(Sc). [PDF]

, 2013
Prion diseases, also known as transmissible spongiform encephalopathies, are a group of fatal neurodegenerative diseases that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans.
A Ertmer, A Janaszewska, A Taraboulos, AP Perez, AY Yam, B Caughey, B Caughey, B Caughey, B Klajnert, B Klajnert, B Klajnert, B Klajnert, B Klajnert, B Klajnert, DD Pless, Dietmar Appelhans, DR Borchelt, Ina Maja Vorberg, J Masel, J Solassol, James M. McCarthy, Jeremy C. Simpson, Jörg Tatzelt, K Doh-Ura, KA Nishina, KF Winklhofer, KF Winklhofer, M Fischer, M Miesbauer, Mark S. Rogers, Markus Franke, MP McKinley, N Nishida, NR Deleault, P Heegaard, PC Klohn, PJ Bosque, PMH Heegaard, R Klebe, RJ Kascsak, S Ghaemmaghami, S Gilch, S Supattapone, S Supattapone, SA Priola, SB Prusiner, SB Prusiner, Sibeal Waldron, Ulrike K. Resenberger, Y Lim   +49 more
core   +3 more sources

C. elegans Models to Study the Propagation of Prions and Prion-Like Proteins

Biomolecules, 2020
A hallmark common to many age-related neurodegenerative diseases, such as Alzheimer’s disease (AD), Parkinson’s disease (PD), and amyotrophic lateral sclerosis (ALS), is that patients develop proteinaceous deposits in their central nervous system (CNS ...
Carl Alexander Sandhof, Simon Oliver Hoppe, Jessica Tittelmeier, Carmen Nussbaum-Krammer   +3 more
doaj   +1 more source

Antihypertensive drug guanabenz is active in vivo against both yeast and mammalian prions. [PDF]

PLoS ONE, 2008
BackgroundPrion-based diseases are incurable transmissible neurodegenerative disorders affecting animals and humans.Methodology/principal findingsHere we report the discovery of the in vivo antiprion activity of Guanabenz (GA), an agonist of alpha2 ...
Déborah Tribouillard-Tanvier, Vincent Béringue, Nathalie Desban, Fabienne Gug, Stéphane Bach, Cécile Voisset, Hervé Galons, Hubert Laude, Didier Vilette, Marc Blondel   +9 more
doaj   +1 more source

Computational Studies of the Structural Stability of Rabbit Prion Protein Compared to Human and Mouse Prion Proteins [PDF]

, 2011
Prion diseases are invariably fatal and highly infectious neurodegenerative diseases affecting humans and animals. The neurodegenerative diseases such as Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob diseases, Gerstmann-Str$\ddot{a}$ussler ...
Zhang, Jiapu
core   +2 more sources

A cationic tetrapyrrole inhibits toxic activities of the cellular prion protein [PDF]

, 2016
Prion diseases are rare neurodegenerative conditions associated with the conformational conversion of the cellular prion protein (PrPC) into PrPSc, a self-replicating isoform (prion) that accumulates in the central nervous system of affected individuals.
Biasini, Emiliano, Borsello, Tiziana, Castilla, Joaqu\uedn, Cerovic, Milica, Cimini, Sara, Elezgarai, Saioa R., Gobbi, Marco, Massignan, Tania, Moreno, Jorge, Negro, Alessandro, Nonno, Romolo, Restelli, Elena, Riccardi, Geraldina, Sangiovanni, Valeria, Stincardini, Claudia, Stravalaci, Matteo, Vanni, Ilaria   +16 more
core   +2 more sources

Human Keratinocytes Express Cellular Prion-Related Proteinin Vitro and during Inflammatory Skin Diseases [PDF]

The American Journal of Pathology, 1998
Prion diseases are transmissible spongiform encephalopathies of humans and animals characterized by the accumulation of a proteinase-resistant isoform of the cellular prion-related protein (PrPc) within the central nervous system. In the present report we demonstrate for the first time the presence of PrPc on squamous epithelia of normal and diseased ...
J, Pammer, W, Weninger, E, Tschachler
openaire   +2 more sources

Increased Attack Rates and Decreased Incubation Periods in Raccoons with Chronic Wasting Disease Passaged through Meadow Voles

Emerging Infectious Diseases, 2022
Chronic wasting disease (CWD) is a naturally-occurring neurodegenerative disease of cervids. Raccoons (Procyon lotor) and meadow voles (Microtus pennsylvanicus) have previously been shown to be susceptible to the CWD agent.
S. Jo Moore, Christina M. Carlson, Jay R. Schneider, Christopher J. Johnson, Justin J. Greenlee   +4 more
doaj   +1 more source

Regulation of Synaptic Pumilio Function by an Aggregation-Prone Domain [PDF]

, 2010
We identified Pumilio (Pum), a Drosophila translational repressor, in a computational search for metazoan proteins whose activities might be regulated by assembly into ordered aggregates.
Menon, Kaushiki P., Salazar, Anna M., Silverman, Edward J., Zinn, Kai   +3 more
core   +2 more sources

Transgenic Mice Expressing Porcine Prion Protein Resistant to Classical Scrapie but Susceptible to Sheep Bovine Spongiform Encephalopathy and Atypical Scrapie

Emerging Infectious Diseases, 2009
How susceptible pigs are to infection with sheep prions is unknown. We show, through transmission experiments in transgenic mice expressing porcine prion protein (PrP), that the susceptibility of this mouse model to bovine spongiform encephalopathy (BSE)
Juan-Carlos Espinosa, María-Eugenia Herva, Olivier Andréoletti, Danielle Padilla, Caroline Lacroux, Hervé Cassard, Isabelle Lantier, Joaquin Castilla, Juan-María Torres   +8 more
doaj   +1 more source