Human Prion Disease Surveillance in Washington State, 2006-2017
Sign Up to like & getrecommendations! Published in 2020 at "JAMA Network Open"
DOI: 10.1001/jamanetworkopen.2020.20690
Abstract: Key Points Question What are the results of human prion disease surveillance in Washington state? Findings In this cross-sectional study using state surveillance data from 2006 to 2017, 143 human prion disease cases were detected,… read more here.
Keywords: state; surveillance; prion; disease ... See more keywords
Is multiple system atrophy an infectious disease?
Sign Up to like & getrecommendations! Published in 2018 at "Annals of Neurology"
DOI: 10.1002/ana.25132
Abstract: Multiple system atrophy (MSA) is a fatal neurodegenerative disorder that causes autonomic failure, parkinsonism, and cerebellar ataxia in any combination. Pathological hallmarks of this condition are a-synucleinrich glial cytoplasmatic inclusions (GCIs). Misfolded a-synuclein aggregates can… read more here.
Keywords: system; synuclein; prion; disease ... See more keywords
Structural Mechanism of Barriers to Interspecies Seeding Transmissibility of Full‐Length Prion Protein Amyloid
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DOI: 10.1002/cbic.201900218
Abstract: A puzzling feature of prion diseases is the cross‐species barriers. The detailed molecular mechanisms underlying these interspecies barriers remain poorly understood because of a lack of high‐resolution structural information on the scrapie isoform of the… read more here.
Keywords: 165 167; structural mechanism; prion; prion protein ... See more keywords
A Small‐Molecule Inhibitor of Prion Replication and Mutant Prion Protein Toxicity
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DOI: 10.1002/cmdc.201700302
Abstract: Into the fold: Prion diseases are neurodegenerative disorders characterized by the accumulation in the brain of a self-replicating, misfolded isoform (PrPSc ) of the cellular prion protein (PrPC ). No therapies are available for these… read more here.
Keywords: prion protein; prion; prion replication; toxicity ... See more keywords
Optimization of the photodynamic inactivation of prions by a phthalocyanine photosensitizer: The crucial involvement of singlet oxygen.
Sign Up to like & getrecommendations! Published in 2019 at "Journal of biophotonics"
DOI: 10.1002/jbio.201800430
Abstract: Prion disorders are fatal neurodegenerative diseases caused by the autocatalytic conversion of a natively occurring prion protein (PrPC ) into its misfolded infectious form (PrPTSE ). The proven resistance of PrPTSE to common disinfection procedures… read more here.
Keywords: prion; singlet oxygen; inactivation; photodynamic inactivation ... See more keywords
A case report of genetic prion disease with two different PRNP variants
Sign Up to like & getrecommendations! Published in 2020 at "Molecular Genetics & Genomic Medicine"
DOI: 10.1002/mgg3.1134
Abstract: Prion diseases are a group of lethal neurodegenerative conditions that occur when the normal, cellular form of the prion protein (PrPC) is converted into an abnormal, scrapie, form of the protein (PrPSc). Disease may be… read more here.
Keywords: prnp variants; prion; disease; case ... See more keywords
Amyloidogenesis of Tau protein
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DOI: 10.1002/pro.3275
Abstract: The role of microtubule‐associated protein Tau in neurodegeneration has been extensively investigated since the discovery of Tau amyloid aggregates in the brains of patients with Alzheimer's disease (AD). The process of formation of amyloid fibrils… read more here.
Keywords: protein; tau; amyloid; prion ... See more keywords
Location of the cross‐β structure in prion fibrils: A search by seeding and electron spin resonance spectroscopy
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DOI: 10.1002/pro.4326
Abstract: Prion diseases are transmissible fatal neurodegenerative disorders spreading between humans and other mammals. The pathogenic agent, prion, is a protease‐resistant, β‐sheet‐rich protein aggregate, converted from a membrane protein called PrPC. PrPSc is the misfolded form… read more here.
Keywords: prion; structure; spectroscopy; electron spin ... See more keywords
Isolation of Exosomes and Microvesicles from Cell Culture Systems to Study Prion Transmission.
Sign Up to like & getrecommendations! Published in 2017 at "Methods in molecular biology"
DOI: 10.1007/978-1-4939-6728-5_11
Abstract: Extracellular vesicles (EVs) are composed of microvesicles and exosomes. Exosomes are small membrane vesicles (40-120 nm sized) of endosomal origin released in the extracellular medium from cells when multivesicular bodies fuse with the plasma membrane,… read more here.
Keywords: cell culture; prion; exosomes microvesicles; isolation exosomes ... See more keywords
Expression of Heterologous PrP and Prion Propagation in RK13 Cells.
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DOI: 10.1007/978-1-4939-7244-9_8
Abstract: Cultured cells are valuable models to study prion infections at the cellular level. Unfortunately, the vast majority of cell lines are resistant to the propagation of prion agents. The rabbit epithelial RK13 cell line is… read more here.
Keywords: rk13 cells; cell; prion; heterologous prp ... See more keywords
Amplification and Detection of Minuscule Amounts of Misfolded Prion Protein by Using the Real-Time Quaking-Induced Conversion.
Sign Up to like & getrecommendations! Published in 2018 at "Methods in molecular biology"
DOI: 10.1007/978-1-4939-7816-8_16
Abstract: A characteristic feature of transmissible spongiform encephalopathies (TSE) is the progressive accumulation of protein aggregates in the brain in a self-propagation manner. Based on this mechanism, in vitro protein amplification systems (such as real-time quaking-induced… read more here.
Keywords: protein; real time; prion; quaking induced ... See more keywords