Acta Neuropathologica, Journal Year: 2024, Volume and Issue: 148(1)
Published: July 24, 2024
Language: Английский
Journal of the Neurological Sciences, Journal Year: 2025, Volume and Issue: 469, P. 123380 - 123380
Published: Jan. 4, 2025
Language: Английский
Citations
1
Molecular Neurobiology, Journal Year: 2025, Volume and Issue: unknown
Published: Feb. 7, 2025
Language: Английский
Citations
1
Neurology International, Journal Year: 2024, Volume and Issue: 16(5), P. 1039 - 1065
Published: Sept. 20, 2024
Creutzfeldt-Jakob disease is a rare neurodegenerative and invariably fatal with fulminant course once the first clinical symptoms emerge. Its incidence appears to be rising, although increasing figures may related improved diagnostic tools. Due highly variable picture at onset, many specialty physicians should aware of this refer patient neurologist for complete evaluation. The criteria have been changed based on considerable progress made in research pathogenesis identification reliable biomarkers. Moreover, accumulated knowledge led series possible therapeutic targets, although, given low very rapid course, evaluation safety efficacy these strategies challenging.
Language: Английский
Citations
4
Neural Regeneration Research, Journal Year: 2024, Volume and Issue: 20(4), P. 1009 - 1014
Published: June 3, 2024
PrP Sc , a misfolded, aggregation-prone isoform of the cellular prion protein (PrP C ), is infectious agent responsible for fatal neurodegenerative diseases humans and other mammals. can adopt different pathogenic conformations (prion strains), which be resistant to potential drugs, or acquire drug resistance, posing challenges development effective therapies. Since obligate precursor any strain serves as mediator neurotoxicity, it represents an attractive therapeutic target diseases. In this minireview, we briefly outline approaches discuss our recent identification Zn(II)-BnPyP, -targeting porphyrin with unprecedented bimodal mechanism action. We argue that in-depth understanding molecular by Zn(II)-BnPyP targets may lead toward new class dual anti-prion compounds.
Language: Английский
Citations
3
Acta Neuropathologica Communications, Journal Year: 2025, Volume and Issue: 13(1)
Published: Jan. 27, 2025
Deposition of abnormally phosphorylated tau aggregates is a central event leading to neuronal dysfunction and death in Alzheimer's disease (AD) other tauopathies. Among aggregates, oligomers (TauOs) are considered the most toxic. AD brains show significant increase TauOs compared healthy controls, their concentration correlating with severity cognitive deficits progression. In vitro vivo TauO exposure leads synaptic dysfunction, but mechanisms action unclear. Evidence suggests that cellular prion protein (PrPC) may act as mediator neurotoxicity, previously proposed for β-amyloid α-synuclein oligomers. To investigate whether PrPC mediates detrimental activities, we effects on memory plasticity wild type (WT) knockout (Prnp0/0) mice. Intracerebroventricular injection significantly impaired recognition WT not Prnp0/0 Similarly, inhibited long-term potentiation acute hippocampal slices from Surface plasmon resonance indicated high-affinity binding between KD 20–50 nM. Immunofluorescence analysis naïve PrPC-overexpressing HEK293 cells exposed showed dose-dependent association over time, co-localization plasma membrane intracellular compartments, suggesting PrPC-may play role internalization. These findings support concept activities through direct interaction, targeting this interaction might be promising therapeutic strategy
Language: Английский
Citations
0
Cureus, Journal Year: 2025, Volume and Issue: unknown
Published: Jan. 29, 2025
Prion diseases are fatal neurodegenerative disorders. Previous studies have indicated the presence of "prion carriers" who remain asymptomatic, but scrapie prion protein (PrPSc) has begun to accumulate in brain. Indeed, we identified an undiagnosed case disease a cadaver used for anatomical practice medical students. These findings suggest that cadavers autopsy may occasionally include carriers. In forensic autopsy, staff cannot sometimes obtain background information dead bodies, and risks exposure prions can be higher than other autopsies. To ensure safety staff, conducted screening tests on brains cadavers. One demonstrated positive results real-time quaking-induced conversion (RT-QuIC) assay, which amplifies abnormal proteins vitro. This result indicates asymptomatic cases exist not only also living individuals. The risk infection via procedures including is high, lethal pathogens difficult decontaminate. Medical should consider or patient carrier regardless whether they symptomatic asymptomatic. type investigation allows us pre-symptomatic disease, could contribute enhanced provide new insights into human diseases.
Language: Английский
Citations
0
Deleted Journal, Journal Year: 2025, Volume and Issue: 7(7), P. 253 - 260
Published: Jan. 1, 2025
The clinical durations of sporadic Creutzfeldt-Jacob disease (sCJD) patients typically do not exceed 2 years, though considerable variation exists. factors influencing survival among Chinese sCJD remain incompletely characterized. A comprehensive evaluation 31 across 7 categories was conducted in a retrospective cohort 300 probable patients. analysis revealed that over 65 years age at onset, those presenting with pyramidal or extrapyramidal dysfunction, showing high signal intensity caudate/putamen on magnetic resonance imaging, and receiving nasal feeding demonstrated significantly shorter times. This study provides the first systematic documentation potential risk affecting times patients, establishing an evidence base for developing implementing targeted intervention strategies.
Language: Английский
Citations
0
Pharmaceutical Patent Analyst, Journal Year: 2025, Volume and Issue: unknown, P. 1 - 5
Published: March 7, 2025
A snapshot of noteworthy recent developments in the patent literature relevance to pharmaceutical and medical research development.
Language: Английский
Citations
0
Movement Disorders Clinical Practice, Journal Year: 2025, Volume and Issue: unknown
Published: April 4, 2025
Language: Английский
Citations
0
Journal of Patient-Reported Outcomes, Journal Year: 2025, Volume and Issue: 9(1)
Published: May 6, 2025
Abstract Background The Medical Research Council-Prion Disease Rating Scale (MRC-PDRS) is a 20-point clinician-reported outcome scale to assess disease progression in patients with prion disease, an invariably fatal neurodegenerative caused by misfolded protein. This study aims evaluate the measurement properties and interpretability of MRC-PDRS support measure’s use for effective management research evaluating effectiveness treatment options diseases. Methodology Utilizing patient data from Telemedicine Assessment Program CJD (TAPCJD), statistical assessment was conducted internal consistency, construct validity (including convergent, divergent validity, known-groups discriminant validity), responsiveness, interpretation guidelines using distribution-based approaches estimate thresholds indicating minimal important change (MIC) scores. Criterion measures used responsiveness included Telephone Interview Cognitive Status (TICS) Neuropsychiatric Inventory–Questionnaire (NPI-Q). Results/Conclusions These findings provide strong preliminary evidence that reliable, valid, responsive as tool measuring MIC estimates ranging 1 3 points. supports potential benefits clinical trials, potentially practice settings.
Language: Английский
Citations
0