Results 11 to 20 of about 8,300 (285)

Yeast Models for Amyloids and Prions: Environmental Modulation and Drug Discovery

Molecules, 2019
Amyloids are self-perpetuating protein aggregates causing neurodegenerative diseases in mammals. Prions are transmissible protein isoforms (usually of amyloid nature).
Tatiana A. Chernova, Yury O. Chernoff, Keith D. Wilkinson   +2 more
doaj   +2 more sources

Silent Propagation of Classical Scrapie Prions in Homozygous K222 Transgenic Mice [PDF]

Emerging Infectious Diseases
Classical scrapie affects sheep and goats. To control prevalence in sheep, the European Union initiated breeding programs targeting resilient genotypes.
Natalia Fernández-Borges, Alba Marín-Moreno, Juan Carlos Espinosa, Sara Canoyra, Olivier Andréoletti, Juan María Torres   +5 more
doaj   +2 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   +2 more sources

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   +2 more sources

Overexpression of bank vole PrP(I109) in mice induces a spontaneous atypical prion disease with sex-dependent onset, early NfL elevation, and universal prion strain permissiveness [PDF]

Acta Neuropathologica Communications
Transgenic mice overexpressing bank vole prion protein with the isoleucine 109 polymorphism, TgVole(I109)4x, develop spontaneous neurodegenerative disease with sex-dependent onset, averaging 170 days in females and 200 days in males at terminal stage ...
Hasier Eraña, Enric Vidal, Natalia Fernández-Borges, Jorge M. Charco, Carlos M. Díaz‑Domínguez, Cristina Sampedro-Torres-Quevedo, Maitena San-Juan-Ansoleaga, Eva Fernández-Muñoz, Josu Galarza-Ahumada, Miguel Ángel Pérez-Castro, Nuno Gonçalves-Anjo, Patricia Piñeiro, Laura Pirisinu, Michele Angelo Di Bari, Samanta Giler, Ilaria Raimondi, Juan Carlos Espinosa, Ilaria Vanni, Claudia D’Agostino, Juan Rodríguez-Cuesta, Laura Pasetto, Valentina Bonetto, Nora González-Martín, Susana Teijeira, Wen-Quan Zou, Mariví Geijo, Juan María Torres, Roberto Chiesa, Manuel A. Sánchez‑Martín, Romolo Nonno, Jesús R. Requena, Joaquín Castilla   +31 more
doaj   +2 more sources

The L108I polymorphism in mouse prion protein drives spontaneous disease and enhances transmission of atypical and classical prion strains. [PDF]

Brain Pathol
A single amino acid change (L108I) combined with PrP overexpression drives spontaneous atypical prion formation in mice, enabling also efficient propagation of diverse prion strains. This model allows studying how spontaneous prion diseases arise and provides powerful tools for investigating strain emergence, transmission barriers, and mechanisms ...
Eraña H, Vidal E, Fernández-Borges N, Charco JM, Díaz-Domínguez CM, Sampedro-Torres-Quevedo C, Galarza-Ahumada J, Fernández-Muñoz E, San-Juan-Ansoleaga M, Pérez-Castro MÁ, Gonçalves-Anjo N, Piñeiro P, Giler S, González-Martín N, Lorenzo NL, Manero-Azua A, Perez de Nanclares G, Geijo M, Sánchez-Martín MA, Requena JR, Castilla J.   +20 more
europepmc   +2 more sources

DL-3-n-Butylphthalide Protects Against PrP¹⁰⁶ ^-126-Induced Neurotoxicity Through NRF2 Signaling and OPA1/DRP1-Mediated Mitochondrial Dynamics. [PDF]

CNS Neurosci Ther
NBP counteracts PrP106‐126‐induced neurotoxicity by activating NRF2 and restoring OPA1/DRP1‐mediated mitochondrial dynamics. It suppresses oxidative stress and preserves mitochondrial function and bioenergetics. These actions support NBP as a promising therapeutic candidate for prion‐related neurodegeneration.
Wu W, Zhang X, Jiang M, Ma N.
europepmc   +2 more sources

Interaction networks of prion, prionogenic and prion-like proteins in budding yeast, and their role in gene regulation. [PDF]

PLoS ONE, 2014
Prions are transmissible, propagating alternative states of proteins. Prions in budding yeast propagate heritable phenotypes and can function in large-scale gene regulation, or in some cases occur as diseases of yeast.
Djamel Harbi, Paul M Harrison
doaj   +1 more source

Glycosylation-related Gene Expression in Prion Diseases [PDF]

Journal of Biological Chemistry, 2005
International ...
Barret, Agnès, Forestier, Lionel, Deslys, Jean-Philippe, Julien, Raymond, Gallet, Paul François   +4 more
openaire   +3 more sources

Molecular Nanobiotechnological Approaches for the Detection and Therapy of Prion Related Diseases [PDF]

Nano Biomedicine and Engineering, 2012
Prion diseases are associated with the accumulation in the brain of an abnormal, protease resistant isoform of a host encoded glycoprotein known as prion protein (PrP). Nanotechnology in combination with biotechniques promises a broad spectrum of highly innovative approaches for overcoming the challenges posed by the prions.
PK Praseetha, Anand S Thampy, P Venugopalan, Murthy S Chavali   +3 more
openaire   +2 more sources