Results 41 to 50 of about 6,958 (184)

Trapping Prion Protein in the Endoplasmic Reticulum Impairs PrPC Maturation and Prevents PrPSc Accumulation [PDF]

Journal of Biological Chemistry, 2005
The conversion of the normal cellular prion protein (PrP(C)) into the abnormal scrapie isoform (PrP(Sc)) is a key feature of prion diseases. The pathogenic mechanisms and the subcellular sites of the conversion are complex and not completely understood.
Cardinale, A, Filesi, I, Vetrugno, V, Pocchiari, M, Sy, MS, BIOCCA, SILVIA   +5 more
openaire   +4 more sources

Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice

Brain Pathology, EarlyView.
We developed a rapid AAV‐based system to generate prion disease models in weeks rather than months. Following systemic AAV9P31 delivery of modified PrP to knockout mice, we achieved brain‐wide expression and successful propagation of both classical (RML) and atypical (GSS‐A117V) prion strains.
Maitena San‐Juan‐Ansoleaga, Eva Fernández‐Muñoz, Jorge M. Charco, Enric Vidal, Diego Herrero‐Martínez, Josu Galarza‐Ahumada, Cristina Sampedro‐Torres‐Quevedo, Samanta Giler, Mariví Geijo, Gloria González‐Aseguinolaza, Hasier Eraña, Joaquín Castilla   +11 more
wiley   +1 more source

A receptor for infectious and cellular prion protein

Brazilian Journal of Medical and Biological Research, 1999
Prions are an unconventional form of infectious agents composed only of protein and involved in transmissible spongiform encephalopathies in humans and animals.
V.R. Martins
doaj   +1 more source

Immunological role of cellular prion protein (PrPC) during cytomegaloviral infection

, 2022
Aims: Human cytomegalovirus (HCMV) infection is the most common congenital viral infection and the leading cause of lasting perinatal brain damage, with the inflammatory response being the primary cause of pathogenic manifestations. Accordingly, it has been shown that anti-inflammatory drugs can reduce abnormalities in newborn mice infected with mouse ...
Karner, Dubravka, Kveštak, Daria, Kučan Brlić, Paola, Cokarić Brdovčak, Maja, Lisnić, Berislav, Brizić, Ilija, Juranić Lisnić, Vanda, Golemac, Mijo, Tomac, Jelena, Legname, Giuseppe, Altmeppen, Hermann C, Hasan, Milena, Jonjić, Stipan, Lenac Roviš, Tihana   +13 more
openaire   +3 more sources

Expression and knockdown of cellular prion protein (PrPC) in differentiating mouse embryonic stem cells [PDF]

Differentiation, 2011
The mammalian cellular prion protein (PrP(C)) is a highly conserved glycoprotein that may undergo conversion into a conformationally altered isoform (scrapie prion protein or PrP(Sc)), widely believed to be the pathogenic agent of transmissible spongiform encephalopathies (TSEs).
Oscar A, Peralta, William R, Huckle, Willard H, Eyestone   +2 more
openaire   +2 more sources

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

Brain Pathology, EarlyView.
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 ...
Hasier Eraña, Enric Vidal, Natalia Fernández‐Borges, Jorge M. Charco, Carlos M. Díaz‐Domínguez, Cristina Sampedro‐Torres‐Quevedo, Josu Galarza‐Ahumada, Eva Fernández‐Muñoz, Maitena San‐Juan‐Ansoleaga, Miguel Ángel Pérez‐Castro, Nuno Gonçalves‐Anjo, Patricia Piñeiro, Samanta Giler, Nora González‐Martín, Nuria L. Lorenzo, Africa Manero‐Azua, Guiomar Perez de Nanclares, Mariví Geijo, Manuel A. Sánchez‐Martín, Jesús R. Requena, Joaquín Castilla   +20 more
wiley   +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

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

Cellular prion protein (PrPc) and hypoxia: true to each other in good times and in bad, in sickness and in health

Frontiers in Cellular Neuroscience, 2016
The cellular prion protein (PrPc) and hypoxia appear to be tightly intertwined. Beneficial effects of PrPc on neuronal survival under hypoxic conditions such as focal cerebral ischemia are strongly supported.
Sanja Ramljak, Holger Herlyn, Inga Zerr
doaj   +1 more source

LPS-induced systemic inflammation reveals an immunomodulatory role for the prion protein at the blood-brain interface

Journal of Neuroinflammation, 2017
Background The cellular prion protein (PrPC) is an evolutionary conserved protein abundantly expressed not only in the central nervous system but also peripherally including the immune system.
Ø. Salvesen, M. R. Reiten, A. Espenes, M. K. Bakkebø, M. A. Tranulis, C. Ersdal   +5 more
doaj   +1 more source