Results 81 to 90 of about 1,549,882 (213)

Copper and Zinc Interactions with Cellular Prion Proteins Change Solubility of Full-Length Glycosylated Isoforms and Induce the Occurrence of Heterogeneous Phenotypes. [PDF]

PLoS ONE, 2016
Prion diseases are characterized biochemically by protein aggregation of infectious prion isoforms (PrPSc), which result from the conformational conversion of physiological prion proteins (PrPC).
Svetlana Brim, Martin H Groschup, Thorsten Kuczius   +2 more
doaj   +1 more source

Human Brain Contusions Contain Pathogenic Transmissible Species that Induce Progressive Cognitive Decline and Tau Pathology in Mice

Annals of Neurology, Volume 99, Issue 4, Page 897-911, April 2026.
Objective Traumatic brain injury (TBI) is an established risk factor for dementia, although the underlying mechanisms remain unclear. Our previous research demonstrated that a single severe TBI in wild‐type (WT) mice induces a prion‐like form of tau (tauTBI) that spreads throughout the brain, leading to memory deficits.
Gloria Vegliante, Francesca Pischiutta, Elena Restelli, Federico Moro, Maria Antonietta Chiaravalloti, Ilaria Raimondi, Ilaria Bertani, Ilaria Lisi, Eliana Sammali, Rosaria Pascente, Serena Scozzari, Laura Pasetto, Carly Douglas, Marco Locatelli, Fabrizio Ortolano, Valentina Bonetto, David J. Loane, Nino Stocchetti, Roberto Chiesa, Elisa R. Zanier   +19 more
wiley   +1 more source

Accumulation of Pathological Prion Protein PrPSc in the Skin of Animals with Experimental and Natural Scrapie

PLoS Pathogens, 2007
Prion infectivity and its molecular marker, the pathological prion protein PrP(Sc), accumulate in the central nervous system and often also in lymphoid tissue of animals or humans affected by transmissible spongiform encephalopathies. Recently, PrP(Sc) was found in tissues previously considered not to be invaded by prions (e.g., skeletal muscles). Here,
Thomzig, Achim, Schulz-Schaeffer, Walter, Wrede, Arne, Wemheuer, Wilhelm, Brenig, Bertram, Kratzel, Christine, Lemmer, Karin, Beekes, Michael   +7 more
openaire   +6 more sources

Toward the Atomic Structure of PrPSc [PDF]

, 2017
In this review, we detail our current knowledge of PrPSc structure on the basis of structural and computational studies. We discuss the progress toward an atomic resolution description of PrPSc and results from the broader field of amyloid studies that ...
Rodriguez, Jose A, Jiang, Lin, Eisenberg, David S   +2 more
core   +1 more source

Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein

Acta Neuropathologica Communications, 2018
Human familial prion diseases are associated with mutations at 34 different prion protein (PrP) amino acid residues. However, it is unclear whether infectious prions are found in all cases.
Brent Race, Katie Williams, Andrew G. Hughson, Casper Jansen, Piero Parchi, Annemieke J. M. Rozemuller, Bruce Chesebro   +6 more
doaj   +1 more source

Toxic mechanisms of amyloid oligomers and therapeutic strategies

Protein Science, Volume 35, Issue 4, April 2026.
Abstract Amyloid oligomers are increasingly recognized as the major toxic contributors across protein‐misfolding disorders. In this review, we cover mechanistic evidence showing how these transient and structurally heterogeneous oligomers disrupt cellular homeostasis by: (i) permeabilizing lipid membranes and forming ion‐conducting pores; (ii ...
Magdalena I. Ivanova, Carmelo La Rosa, Ayyalusamy Ramamoorthy   +2 more
wiley   +1 more source

Characterization of intracellular dynamics of inoculated PrP-res and newly generated PrPSc during early stage prion infection in Neuro2a cells [PDF]

, 2014
To clarify the cellular mechanisms for the establishment of prion infection, we analyzed the intracellular dynamics of inoculated and newly generated abnormal isoform of prion protein (PrPSc) in Neuro2a cells.
Gerald S. Baron, Akio Suzuki, Baron, Gerald S., Horiuchi, Motohiro, Motohiro Horiuchi, Suzuki, Akio, Yamasaki, Takeshi, Hasebe, Rie, Takeshi Yamasaki, Rie Hasebe   +9 more
core   +1 more source

Possible alignment of the EU BSE surveillance with the new WOAH provisions

EFSA Journal, Volume 24, Issue 4, April 2026.
Abstract The European Commission requested the assessment of the capacity of the surveillance provisions of the World Organization for Animal Health (WOAH) to detect bovine spongiform encephalopathy (BSE) cases (C‐, H‐ and L‐type) in the European Union (EU) and to propose if any current EU surveillance provisions should be kept.
EFSA Panel on Animal Health and Welfare (AHAW), Søren Saxmose Nielsen, Julio Alvarez, Anette Boklund, Sabine Dippel, Fernanda Dórea, Jordi Figuerola, Mette S. Herskin, Virginie Michel, Miguel Angel Miranda Chueca, Eleonora Nannoni, Anja B. Riber, Karl Stahl, Jan Arend Stegeman, Hans‐Hermann Thulke, Frank Tuyttens, Christoph Winckler, Mark Arnold, Christian Ducrot, John Griffin, Giuseppe Ru, Marion Simmons, Sean Ashe, Angel Ortiz‐ Pelaez, Kamela Kryemadhi, Romolo Nonno   +25 more
wiley   +1 more source

Retrograde Transport by Clathrin-Coated Vesicles is Involved in Intracellular Transport of PrPSc in Persistently Prion-Infected Cells [PDF]

, 2018
Intracellular dynamics of an abnormal isoform of prion protein (PrPSc) are tightly associated with prion propagation. However, the machineries involved in the intracellular trafficking of PrPSc are not fully understood.
Horiuchi, Motohiro, Suzuki, Akio, Yamasaki, Takeshi, Hasebe, Rie   +3 more
core   +1 more source

Plasmonics‐Enhanced Characterization of Cervid PrP (87–114) Fragment Aggregates in Solution

Journal of Biophotonics, Volume 19, Issue 3, March 2026.
Multimodal nanophotonics platform combining plasmon‐enhanced imaging, label‐free SERS, and optical staining reveals new insights into the aggregation of the cervid PrP (87–114) fragment, an amyloidogenic sequence linked to chronic wasting disease susceptibility.ABSTRACTWe performed a multimodal characterisation of self‐assembled fibrillar aggregates ...
Shinki Midha, Aishwarya Sriraman, Lyudmyla Dorosh, Holger Wille, Maria Stepanova   +4 more
wiley   +1 more source