Using NMR spectroscopy to investigate the role played by copper in prion diseases
Sign Up to like & getrecommendations! Published in 2020 at "Neurological Sciences"
DOI: 10.1007/s10072-020-04321-9
Abstract: Prion diseases are a group of rare neurodegenerative disorders that develop as a result of the conformational conversion of normal prion protein (PrPC) to the disease-associated isoform (PrPSc). The mechanism that actually causes disease remains… read more here.
Keywords: copper ions; prion; role; spectroscopy ... See more keywords
Ultra-efficient Amplification of Abnormal Prion Protein by Modified Protein Misfolding Cyclic Amplification with Electric Current
Sign Up to like & getrecommendations! Published in 2017 at "Molecular Neurobiology"
DOI: 10.1007/s12035-017-0431-8
Abstract: Prion diseases are clinically diagnosed and confirmed upon post-mortem histopathological examination of brain tissue. The only reliable molecular marker for prion diseases is abnormal prion protein (PrPSc), a pathologically conformed prion protein that primarily accumulates… read more here.
Keywords: prion; prion protein; prion diseases; abnormal prion ... See more keywords
Decrease in Skin Prion-Seeding Activity of Prion-Infected Mice Treated with a Compound Against Human and Animal Prions: a First Possible Biomarker for Prion Therapeutics
Sign Up to like & getrecommendations! Published in 2021 at "Molecular Neurobiology"
DOI: 10.1007/s12035-021-02418-6
Abstract: Previous studies have revealed that the infectious scrapie isoform of prion protein (PrPSc) harbored in the skin tissue of patients or animals with prion diseases can be amplified and detected through the serial protein misfolding… read more here.
Keywords: prion; seeding activity; prion diseases; prion seeding ... See more keywords
The risk of Creutzfeldt–Jakob disease infection in cadaveric surgical training
Sign Up to like & getrecommendations! Published in 2022 at "Anatomical Science International"
DOI: 10.1007/s12565-022-00662-x
Abstract: The usefulness of cadaver surgical training in the clinical field is already well known. In Japan, the number of universities introducing cadaver surgical training is increasing. In addition to formalin fixation, various fixation methods are… read more here.
Keywords: creutzfeldt jakob; cadaver surgical; prion diseases; surgical training ... See more keywords
Autophagy pathways in the treatment of prion diseases.
Sign Up to like & getrecommendations! Published in 2019 at "Current opinion in pharmacology"
DOI: 10.1016/j.coph.2019.04.013
Abstract: Prions use cellular machineries for autocatalytic propagation by conformational conversion of the cellular prion protein into the pathological isoform PrPSc. Autophagy is a basic cellular degradation and recycling machinery that delivers cargo to lysosomes. Increase… read more here.
Keywords: prion; treatment prion; pharmacology; prion diseases ... See more keywords
How can we increase the number of autopsies for prion diseases? A model system in Japan
Sign Up to like & getrecommendations! Published in 2017 at "Journal of the Neurological Sciences"
DOI: 10.1016/j.jns.2016.11.073
Abstract: • The autopsy of prion disease patients is often difficult because of the fear of infectivity. read more here.
Keywords: diseases model; model system; prion diseases; number autopsies ... See more keywords
Prion diseases reported in the “Annual of the Pathological Autopsy Cases in Japan”
Sign Up to like & getrecommendations! Published in 2021 at "Journal of the Neurological Sciences"
DOI: 10.1016/j.jns.2020.117215
Abstract: BACKGROUND For surveillance projects to be successful, it is important to accurately diagnose all patients, without overlooking any cases. Here, we investigated the present clinical diagnostic accuracy for prion diseases in Japan. METHODS We analyzed… read more here.
Keywords: annual pathological; pathological autopsy; prion; autopsy ... See more keywords
Aberrant Decrease of the Endogenous SIRT3 and Increases of Acetylated Proteins in Scrapie Infected Cell Line SMB-S15 and in the Brains of Experimental Mice.
Sign Up to like & getrecommendations! Published in 2019 at "ACS chemical neuroscience"
DOI: 10.1021/acschemneuro.9b00341
Abstract: Mitochondrial dysfunction is closely linked to the pathogenesis of neurodegenerative disorders, including prion diseases. SIRT3 is the major mitochondria NAD+ dependent deacetylase that acts as a regulator of mitochondrial protein function. Although SIRT3 was reported… read more here.
Keywords: aberrant decrease; increases acetylated; prion; decrease endogenous ... See more keywords
A designer chaperone against prion diseases
Sign Up to like & getrecommendations! Published in 2019 at "Nature Biomedical Engineering"
DOI: 10.1038/s41551-019-0367-6
Abstract: A computationally designed small molecule inhibits the replication of prion-protein aggregates and ameliorates neurological symptoms in mouse and non-human-primate models of transmissible spongiform encephalopathy. read more here.
Keywords: prion diseases; designer chaperone; prion; chaperone prion ... See more keywords
Differential overexpression of SERPINA3 in human prion diseases
Sign Up to like & getrecommendations! Published in 2017 at "Scientific Reports"
DOI: 10.1038/s41598-017-15778-8
Abstract: Prion diseases are fatal neurodegenerative disorders with sporadic, genetic or acquired etiologies. The molecular alterations leading to the onset and the spreading of these diseases are still unknown. In a previous work we identified a… read more here.
Keywords: prion; overexpression serpina3; prion diseases; differential overexpression ... See more keywords
Bank vole prion protein extends the use of RT-QuIC assays to detect prions in a range of inherited prion diseases
Sign Up to like & getrecommendations! Published in 2021 at "Scientific Reports"
DOI: 10.1038/s41598-021-84527-9
Abstract: The cerebrospinal fluid (CSF) real-time quaking-induced conversion assay (RT-QuIC) is an ultrasensitive prion amyloid seeding assay for diagnosis of sporadic Creutzfeldt–Jakob disease (CJD) but several prion strains remain unexplored or resistant to conversion with commonly… read more here.
Keywords: inherited prion; prion; prion protein; bank vole ... See more keywords