Results 101 to 110 of about 36,884 (188)

Mutant prion proteins increase calcium permeability of AMPA receptors, exacerbating excitotoxicity. [PDF]

PLoS Pathog, 2020
Ghirardini E, Restelli E, Morini R, Bertani I, Ortolan D, Perrucci F, Pozzi D, Matteoli M, Chiesa R.   +8 more
europepmc   +1 more source

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

Brain Pathology, Volume 36, Issue 4, July 2026.
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 unifying model for the propagation of prion proteins in yeast brings insight into the [PSI+] prion. [PDF]

PLoS Comput Biol, 2020
Lemarre P, Pujo-Menjouet L, Sindi SS.
europepmc   +1 more source

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

Multiaxial Biophysical Control of Oncogenic Phase Separation by Indoleamines: A Proof‐of‐Concept Synthesis of Landscape‐Level Regulation

Journal of Pineal Research, Volume 78, Issue 4, July 2026.
ABSTRACT Oncogenic condensates act as biophysical sanctuaries that stabilize malignant survival programs. However, a universal regulator capable of orchestrating the integrated biophysical axes governing cellular phase behavior has remained elusive.
Doris Loh, Luiz Gustavo de Almeida Chuffa, Fábio Rodrigues Ferreira Seiva, Russel J. Reiter   +3 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

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

Small Extracellular Vesicles from Neural Cells: Physiological and Pathological Roles, and Potential in Neurodegenerative Therapy

Advanced Healthcare Materials, Volume 15, Issue 21, 5 June 2026.
Neural cell–derived small extracellular vesicles (sEVs) are emerging as pivotal mediators in neurodegenerative diseases, exerting both pathogenic and therapeutic functions. This review synthesizes current evidence on how sEVs from distinct neural cell types regulate neurodegeneration, neuroprotection, biomarker discovery, and targeted drug delivery ...
Muhammad Waqas Salim, Wei Zhang, Lyndsey Collins‐Praino, Yuling Wang, Andrew Care   +4 more
wiley   +1 more source

RNF213 Is an Interferon‐Stimulated Gene That Targets Influenza A Virus NP and Activates MDA5 to Restrict Infection

Advanced Science, Volume 13, Issue 31, 4 June 2026.
RNF213 is characterized as a dual‐functional antiviral effector. It directly mediates the degradation of the influenza A virus nucleoprotein (NP) while simultaneously activating the MDA5‐mediated innate immune signaling pathway. This coordinated response establishes a powerful host defense system against viral infection. ABSTRACT Influenza A virus (IAV)
Haoning Li, Yuying Zhang, Jingjing Wang, Qianqian Zhang, Hailiang Sun, Fanhua Wei   +5 more
wiley   +1 more source

The Polymers of Life: Exploring Cellular Function Through Polymer Concepts

Advanced Science, Volume 13, Issue 33, 15 June 2026.
Biomolecular phase separation reveals that a hidden layer of cellular organization is governed by the principles of polymer science. This review bridges polymer physics and cell biology, offering a primer on fundamental concepts, proposing a framework for interrogating cellular function, and synthesizing biophysical methods for decoding macromolecular ...
Mark Chen, Ashutosh Chilkoti
wiley   +1 more source