Results 81 to 90 of about 22,988 (222)

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

Brain Pathology, Volume 36, Issue 4, July 2026.
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

Creutzfeldt–Jakob disease: A case report and differential diagnoses

Clinical Case Reports, 2022
Although sporadic Creutzfeldt–Jakob disease is a rare neurodegenerative disease and often difficult to diagnose at the earliest onset, meticulous clinical examination, electroencephalography, and neuroimaging findings will help in diagnosis.
Akash Raut, Anjila Thapa, Ashish Shrestha, Kamal Saud, Reema Rajbhandari, Shailendra Katwal   +5 more
doaj   +1 more source

Probing the Influence of Sulfur–Aromatic Interactions on the Electronic Structure of Gas‐Phase Peptides

Chemistry – A European Journal, Volume 32, Issue 23, 16 June 2026.
Just as fingerprints reveal identity, spectroscopic fingerprints reveal molecular secrets. In this article, we show parts of the unique fingerprint of the sulfur–aromatic interaction. By measuring how this force alters the electronic structure of gas‐phase peptides, we provide a fundamental understanding of interactions that give insights into the ...
Laura Pille, Carlos Ortiz‐Mahecha, Bart Oostenrijk, Juliette Leroux, Marion Girod, Konstantin Hirsch, Luke MacAleese, Robert Horst Meißner, Debora Scuderi, Isaak Unger, Vicente Zamudio‐Bayer, Sadia Bari, Lucas Schwob   +12 more
wiley   +1 more source

HaloTag Fusion Enables Dynamic Analysis of Prion Protein Biosynthesis, Turnover, and Misfolding

ChemBioChem, Volume 27, Issue 11, 15 June 2026.
Prion protein (PrP) misfolding underlies fatal neurodegenerative diseases. We developed a HaloTag‐based PrP fusion enabling spatiotemporal labeling of distinct PrP populations in living cells. This system recapitulates native PrP biology, reveals early misfolding events in disease‐associated mutants, and allows mechanistic interrogation of PrP‐lowering
Antonio Masone, Eleonora Busani, Roberto Chiesa   +2 more
wiley   +1 more source

Patterns of Postictal Abnormalities in Relation to Status Epilepticus in Adults

Annals of Clinical and Translational Neurology, Volume 13, Issue 6, Page 1118-1128, June 2026.
ABSTRACT Objective Abnormalities on peri‐ictal diffusion‐weighted magnetic resonance imaging (DWI‐PMAs) are well‐established for patients with status epilepticus (SE), but knowledge on patterns of DWI‐PMAs and their prognostic impact is sparse. Methods This systematic review and individual participant data meta‐analysis included observational studies ...
Andrea Enerstad Bolle, Silvana Sarria‐Estrada, Thomas Bonduelle, Bertram Daniel Dynesen, Manuel Toledo, Zhu Chung Che, Kjell Heuser, Anastasios Chatzikonstantinou, Jérôme Aupy, Udaya Seneviratne, Estevo Santamarina Perez, Christoph Patrick Beier   +11 more
wiley   +1 more source

PrPCWD lymphoid cell targets in early and advanced chronic wasting disease of mule deer

, 2002
Up to 15% of free-ranging mule deer in northeastern Colorado and southeastern Wyoming, USA, are afflicted with a prion disease, or transmissible spongiform encephalopathy (TSE), known as chronic wasting disease (CWD).
Keulen, L.J.M., van, van Keulen, L.J.M., Miller, M.W., Oesch, B., Hoover, E.A., Langeveld, J.P.M., Sigurdson, C.J., Barillas-Mury, C.   +7 more
core   +1 more source

A Systematic Comparison of Alpha‐Synuclein Seed Amplification Assays for Increasing Reproducibility

Annals of Clinical and Translational Neurology, Volume 13, Issue 6, Page 1088-1105, June 2026.
ABSTRACT Seed amplification assays (SAAs) enable ultrasensitive detection of misfolded α‐synuclein across biofluids and tissues. Yet, heterogeneity in protocols limits cross‐study comparability and clinical translation. Here, we review α‐synuclein SAA methods and their performance across various biological matrices.
Manuela Amaral‐do‐Nascimento, Daniela F. Santos, Tuane C. R. G. Vieira, Tiago F. Outeiro   +3 more
wiley   +1 more source

The Molecular Pathology of Prion Diseases [PDF]

, 2004
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal ...
Vassallo, Neville, Herms, Jochen, Kretzschmar, Hans A.   +2 more
core  

Case series of Creutzfeldt-Jakob disease in a third-level hospital in Quito

, 2018
Background: Creutzfeldt-Jakob disease is a rare and fatal neurodegenerative disorder that affects mammals and humans. The prevalence of this disease in the United States is 0.5 to 1 per million inhabitants.
Serrano Dueñas, Marcos
core   +1 more source

Application of real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance

, 2023
Background Evaluation of the application of CSF real-time quaking-induced conversion in Creutzfeldt–Jakob disease surveillance to investigate test accuracy, influencing factors, and associations with disease incidence.
Hermann, Peter, Goebel, Stefan, Schmitz, Matthias, Cramm, Maria, Zerr, Inga, Schütte-Schmidt, Julia, Bunck, Timothy, Glatzel, Markus, Matschke, Jakob, Stadelmann, Christine, Schulz-Schaeffer, Walter   +10 more
core   +1 more source