Results 61 to 70 of about 21,599 (190)

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

Risk Ranking of Microbiological Hazards in Food (2015–2019)

Food Risk Assess Europe, Volume 4, Issue 3, July 2026.
Abstract Microbiological food safety risks arise from pathogens that can contaminate food at different points along the food chain. Exposure to these hazards can result in illnesses that can vary widely in severity and duration, depending on factors such as the specific pathogen involved, the amount of the pathogen ingested, and the susceptibility of ...
Ms Chloe Glennon, Lisa O’Connor, Kevin Burke, Lauren Russell, Shaun Smith, Declan Bolton, Theo de Waal, Patricia Garvey, Montserrat Gutierrez, Noel McCarthy, Lois O’Connor, Helen O’Shea, Geraldine Duffy   +12 more
wiley   +1 more source

Sporadic Creutzfeldt-Jakob Disease: A Case Report and Literature Review

Cureus
Prion disease is an uncommon entity characterized by exceptionally rapid neurodegenerative deterioration. There are three categories of prion disease: (1) sporadic: sporadic Creutzfeldt-Jakob disease (sCJD), sporadic fatal insomnia, and protease ...
María José Sánchez Pérez, Ana S Vargas, Benito G Ceballos Vazquez Tagle, Cesar A Nieves Perez   +3 more
semanticscholar   +1 more source

Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia

Behavioural Neurology, 1994
Aphasia is a common manifestation of Creutzfeldt-Jakob disease (CJD), and investigation of the linguistic disorders of CJD patients may provide insights into the neurobiological mechanisms of language and aphasia.
S. E. McPherson, J. D. Kuratani, J. L. Cummings, J. Shih, P. S. Mischel, H. V. Vinters   +5 more
doaj   +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

Defining the Prion Type of Fatal Familial Insomnia

Pathogens, 2021
Fatal familial insomnia (FFI) belongs to the genetic human transmissible spongiform encephalopathies (TSE), such as genetic Creutzfeldt-Jakob disease (CJD) or Gerstmann-Straeussler-Scheinker syndrome (GSS).
Wiebke Jürgens-Wemheuer, Arne Wrede, Walter Schulz-Schaeffer   +2 more
doaj   +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

Revisão sistemática do manejo farmacológico na doença de Creutzfeldt-Jakob: ainda sem opções?

Journal Archives of Health
Introdução: A Doença de Creutzfeldt-Jakob (DCJ) é uma doença neurodegenerativa, caracterizada por provocar uma desordem cerebral com perda de memória e tremores. É de rápida evolução, e de forma inevitável, leva à morte do paciente.
Lucas Alves Pedrada, Camille Pettene Dantas, Robson Waldeck Silva Júnior, Jassiara Soares da Silva   +3 more
semanticscholar   +1 more source

Enfermedad de Creutzfeldt-Jakob en un paciente con infartos cerebrales múltiples

Revista Cubana de Medicina, 1999
Se presentó el caso de un paciente con enfermedad de Creutzfeldt-Jakob, con antecedentes de enfermedad cerebrovascular isquémica previa y un cuadro típico de demencia, ataxia, mioclonías y cambios típicos en el electroencefalograma con imagenología ...
Edmundo Rivero Arias, María Teresa Cuesta Mejías, René Zamora Marín, Leopoldo José Araujo Pradere   +3 more
doaj  

Human prion disease with a G114V mutation and epidemiological studies in a Chinese family: a case series

Journal of Medical Case Reports, 2008
Introduction Transmissible spongiform encephalopathies are a group of neurodegenerative diseases of humans and animals. Genetic Creutzfeldt-Jakob diseases, in which mutations in the PRNP gene predispose to disease by causing the expression of abnormal ...
Ye Jing, Han Jun, Shi Qi, Zhang Bao-Yun, Wang Gui-Rong, Tian Chan, Gao Chen, Chen Jian-Min, Li Cun-Jiang, Liu Zheng, Li Xian-Zhang, Zhang Lai-Zhong, Dong Xiao-Ping   +12 more
doaj   +1 more source