Results 41 to 50 of about 7,256 (169)

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

Wernicke-Korsakoff syndrome as a rare phenotype of sporadic Creutzfeldt-Jakob disease [PDF]

Prion, 2018
We reported the case of a patient with Wernicke-Korsakoff syndrome (WKs) as an early clinical manifestation of sporadic Creutzfeld-Jakob disease (sCJD). The 66-year-old female complained of dizziness and imbalance which mostly occurred while walking.
Joanna, Bielewicz, Anna, Szczepańska-Szerej, Magdalena, Ogórek, Piotr, Dropko, Katarzyna, Wojtal, Konrad, Rejdak   +5 more
openaire   +3 more sources

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

Chronological Diagnostic Algorithm Predicting Neuropathology in Parkinsonism

Annals of Neurology, Volume 99, Issue 6, Page 1405-1414, June 2026.
Objective Pre‐mortem diagnosis of parkinsonism is often challenging due to atypical presentations, overlapping syndromes, and co‐pathologies. This study aimed to develop a machine learning‐based algorithm predicting neuropathology in parkinsonism using chronological clinical presentations, which has previously been underexplored.
Daisuke Ono, Hiroaki Sekiya, Alexia R. Maier, Neill R. Graff‐Radford, Zbigniew K. Wszolek, Dennis W. Dickson   +5 more
wiley   +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

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

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

Exploring and Targeting the Connection of Iron and Copper Homeostasis to Neurodegenerative Diseases

MedComm, Volume 7, Issue 6, June 2026.
Iron and copper dyshomeostasis, along with their interactions with key intrinsically disordered proteins (e.g., Aβ, tau, α‐synuclein) have a strong implication in the onset and progression of neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), Prion diseases (PrDs), Huntington's disease (HD), Wilson's disease (WD),
Xin Liu, Lin Jia, Kerong Wu, Mo Chen, Jichao Sun, Chuanbin Yang, Chengchao Xu, Jie Sun, Jigang Wang, Lingyun Dai   +9 more
wiley   +1 more source