Results 121 to 130 of about 78,455 (315)
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 +11 more
wiley +1 more source
Prions, prion-like prionoids, and neurodegenerative disordersVacancy
Annals of Indian Academy of Neurology, 2016 Prion diseases or transmissible spongiform encephalopathies are fatal neurodegenerative diseases characterized by the aggregation and deposition of the misfolded prion protein in the brain.
Ashok Verma
doaj +1 more source
On the statistical mechanics of prion diseases
, 2001 We simulate a two-dimensional, lattice based, protein-level statistical mechanical model for prion diseases (e.g., Mad Cow disease) with concommitant prion protein misfolding and aggregation.
A. Coghlan +25 more
core +1 more source
Liquid–Liquid Phase Separation in Major Hallmarks of Cancer
Cell Proliferation, EarlyView.Aberrant condensates formed through phase separation are involved in the dysregulation of various critical cellular processes, including genome stability, transcriptional regulation and signal transduction, thereby promoting malignant transformation and the acquisition of multiple cancer hallmarks.
Chen‐chen Xie +10 more
wiley +1 more source
A survey and a molecular dynamics study on the (central) hydrophobic region of prion proteins
, 2014 Prion diseases are invariably fatal neurodegenerative diseases that affect humans and animals. Unlike most other amyloid forming neurodegenerative diseases, these can be highly infectious. Prion diseases occur in a variety of species.
Wang, Feng, Zhang, Jiapu
core +1 more source
Prions and Prion Diseases [PDF]
Laboratory Medicine, 1999 Prion diseases (transmissible encephalopathies) are progressive neurodegenerative diseases of animals and humans caused by a unique agent, the prion. These diseases have recently been in the news because of the emergence of bovine spongiform encephalopathy (BSE; mad cow disease) and variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom.
openaire +1 more source
Epidemiology of progressive intellectual and neurological deterioration in UK children
Developmental Medicine &Child Neurology, EarlyView.This study of PIND in UK children was carried out via the British Paediatric Surveillance Unit from 1997 to 2024. It identified six cases of vCJD. 2367 children had other diagnoses explain their deterioration. There were 259 other diseases in the diagnosed group.
Christopher M. Verity +3 more
wiley +1 more source
, 2009 In the template-assistance model, normal prion protein (PrPC), the pathogenic cause of prion diseases such as Creutzfeldt-Jakob (CJD) in human, Bovine Spongiform Encephalopathy (BSE) in cow, and scrapie in sheep, converts to infectious prion (PrPSc ...
A Aguzzi +40 more
core +1 more source
Protein tandem repeats that produce frameshifts can generate new structural states and functions
The FEBS Journal, EarlyView.We explored an alternative protein structure landscape by analyzing amino acid sequences from frameshifted tandem repeats—regions prone to frameshifts. These frameshifts, especially in short repeats, lead to more drastic changes than in non‐repetitive regions, often altering structure, function, localization, and potentially contributing to disease ...
Zarifa Osmanli +8 more
wiley +1 more source