Associated Papers
Integrated peripheral metabolic and inflammatory biomarker signatures are associated with clinical deterioration in Creutzfeldt-Jakob disease.
Chen ZY et al.
Neurobiology of Disease, 2026
Peripheral Signatures of Multidimensional Pathology in Symptomatic and Asymptomatic Creutzfeldt-Jakob Disease.
Chen ZY et al.
Cns Neuroscience & Therapeutics, 2026
The novel T107I Inherited prion disease can present as a clinical and biomarker mimic of familial Alzheimer's disease.
Holm-Mercer L et al.
Journal of Neurogenetics, 2025
Amyloid fibril structures and ferroptosis activation induced by ALS-causing SOD1 mutations.
Wang LQ et al.
Science Advances, 2024
Mutations of evolutionarily conserved aromatic residues suggest that misfolding of the mouse prion protein may commence in multiple ways.
Pal S et al.
Journal of Neurochemistry, 2023
Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms.
Denouel A et al.
Euro Surveillance : Bulletin Europeen Sur Les Maladies Transmissibles = European Communicable Disease Bulletin, 2023
Arg177 and Asp159 from dog prion protein slow liquid-liquid phase separation and inhibit amyloid formation of human prion protein.
Li XN et al.
The Journal of Biological Chemistry, 2023
Creutzfeldt-Jakob disease presenting as Korsakoff syndrome caused by E196A mutation in PRNP gene: A case report.
Zhang YK et al.
World Journal of Clinical Cases, 2023
High-Depth PRNP Sequencing in Brains With Sporadic Creutzfeldt-Jakob Disease.
Murley AG et al.
Neurology. Genetics, 2023
Prion Mutations in Republic of Republic of Korea, China, and Japan.
Kim DY et al.
International Journal of Molecular Sciences, 2022
Estimation of the number of inherited prion disease mutation carriers in the UK.
Corbie R et al.
European Journal of Human Genetics : Ejhg, 2022
Cryo-EM structure of disease-related prion fibrils provides insights into seeding barriers.
Li Q et al.
Nature Structural & Molecular Biology, 2022
Prevalence and Treatments of Movement Disorders in Prion Diseases: A Longitudinal Cohort Study.
Sequeira D et al.
Movement Disorders : Official Journal of the Movement Disorder Society, 2022
The First Evaluation of Proteinase K-Resistant Prion Protein (PrPSc) in Korean Appendix Specimens.
Won SY et al.
Medicina (kaunas, Lithuania), 2022
Genetic prion diseases presenting as frontotemporal dementia: clinical features and diagnostic challenge.
Chen Z et al.
Alzheimer's Research & Therapy, 2022
Blood β-Synuclein and Neurofilament Light Chain During the Course of Prion Disease.
Halbgebauer S et al.
Neurology, 2022
Regional variability and genotypic and pharmacodynamic effects on PrP concentration in the CNS.
Mortberg MA et al.
Jci Insight, 2022
Human prion protein: exploring the thermodynamic stability and structural dynamics of its pathogenic mutants.
Halder P et al.
Journal of Biomolecular Structure & Dynamics, 2022
Characteristics of Chinese patients with genetic CJD who have E196A or E196K mutation in PRNP: comparative analysis of patients identified in the Chinese National CJD Surveillance System.
Shi Q et al.
Bmj Open, 2021
Genetic Prion Disease: Insight from the Features and Experience of China National Surveillance for Creutzfeldt-Jakob Disease.
Shi Q et al.
Neuroscience Bulletin, 2021
Evaluation of plasma tau and neurofilament light chain biomarkers in a 12-year clinical cohort of human prion diseases.
Thompson AGB et al.
Molecular Psychiatry, 2021
Phenotypic diversity of genetic Creutzfeldt-Jakob disease: a histo-molecular-based classification.
Baiardi S et al.
Acta Neuropathologica, 2021
Genetic prion disease-related mutation E196K displays a novel amyloid fibril structure revealed by cryo-EM.
Wang LQ et al.
Science Advances, 2021
Probable genetic Creutzfeldt-Jakob Disease with rare E196K mutation.
Orlewska K et al.
Neurologia I Neurochirurgia Polska, 2021
Identification of Prion Disease-Related Somatic Mutations in the Prion Protein Gene (PRNP) in Cancer Patients.
Kim YC et al.
Cells, 2020
First Report of the Potential Bovine Spongiform Encephalopathy (BSE)-Related Somatic Mutation E211K of the Prion Protein Gene (PRNP) in Cattle.
Won SY et al.
International Journal of Molecular Sciences, 2020
Genetic Factors in Mammalian Prion Diseases.
Mead S et al.
Annual Review of Genetics, 2019
Mutations in Prion Protein Gene: Pathogenic Mechanisms in C-Terminal vs. N-Terminal Domain, a Review.
Bernardi L et al.
International Journal of Molecular Sciences, 2019
Cerebrospinal Fluid Total Prion Protein in the Spectrum of Prion Diseases.
Villar-Piqué A et al.
Molecular Neurobiology, 2019
Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans.
Rossi M et al.
Viruses, 2019
Clinical and neuropathological phenotype associated with the novel V189I mutation in the prion protein gene.
Di Fede G et al.
Acta Neuropathologica Communications, 2019
Novel prion mutation (p.Tyr225Cys) in a Korean patient with atypical Creutzfeldt-Jakob disease.
Bagyinszky E et al.
Clinical Interventions in Aging, 2019
Prion disease diagnosis using subject-specific imaging biomarkers within a multi-kernel Gaussian process.
Canas LS et al.
Neuroimage. Clinical, 2019
Movement Disorders in Prionopathies: A Systematic Review.
Rodriguez-Porcel F et al.
Tremor and Other Hyperkinetic Movements (new York, N.y.), 2019
A valine-to-lysine substitution at position 210 induces structural conversion of prion protein into a β-sheet rich oligomer.
Kakuda K et al.
Biochemical and Biophysical Research Communications, 2018
Glycosylation Significantly Inhibits the Aggregation of Human Prion Protein and Decreases Its Cytotoxicity.
Yi CW et al.
Scientific Reports, 2018
The clinical features in Chinese patients with PRNP D178N mutation.
Chen S et al.
Acta Neurologica Scandinavica, 2018
Genetic PrP Prion Diseases.
Kim MO et al.
Cold Spring Harbor Perspectives in Biology, 2018
iPS Cell Cultures from a Gerstmann-Sträussler-Scheinker Patient with the Y218N PRNP Mutation Recapitulate tau Pathology.
Matamoros-Angles A et al.
Molecular Neurobiology, 2018
The language disorder of prion disease is characteristic of a dynamic aphasia and is rarely an isolated clinical feature.
Caine D et al.
Plos One, 2018
Genetic Creutzfeldt-Jakob disease.
Ladogana A et al.
Handbook of Clinical Neurology, 2018
Characterization of mutations in PRNP (prion) gene and their possible roles in neurodegenerative diseases.
Bagyinszky E et al.
Neuropsychiatric Disease and Treatment, 2018