Strain diversity in neurodegenerative disease: an argument for a personalized medicine approach to diagnosis and treatment
Sign Up to like & getrecommendations! Published in 2021 at "Acta Neuropathologica"
DOI: 10.1007/s00401-021-02311-5
Abstract: The protein misfolding mechanism of disease was first described almost 40 years ago in a group of neurodegenerative disorders caused by the prion protein (PrP) [1]. In these diseases, misfolding of cellular PrP (PrPC) into… read more here.
Keywords: medicine; prion; disease; conformation ... 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
Correlating diffusion-weighted MRI intensity with type 2 pathology in mixed MM-type sporadic Creutzfeldt-Jakob disease
Sign Up to like & getrecommendations! Published in 2020 at "Journal of the Neurological Sciences"
DOI: 10.1016/j.jns.2019.116515
Abstract: The existence of affected subjects with both abnormal prion protein (PrPSc) types has been reported, and their clinical features were somewhat similar to the dominant PrPSc type but varied in sporadic Creutzfeldt-Jakob disease (sCJD). Presently,… read more here.
Keywords: sporadic creutzfeldt; disease; intensity; pathology ... See more keywords
A novel approach for scrapie-associated prion (PrPSc) detection in blood using the competitive affinity of an aggregate-specific antibody and streptavidin to PrPSc.
Sign Up to like & getrecommendations! Published in 2017 at "Research in veterinary science"
DOI: 10.1016/j.rvsc.2017.09.007
Abstract: Scrapie is a fatal neurodegenerative disorder affecting sheep and goats, originating from exposure to disease-associated prions (PrPSc). An ante-mortem screening test that can detect native PrPSc in body fluids remains unavailable due to insufficient sensitivity… read more here.
Keywords: detection; blood using; antibody; scrapie ... See more keywords
Glial activation in prion diseases is selectively triggered by neuronal PrPSc.
Sign Up to like & getrecommendations! Published in 2022 at "Brain pathology"
DOI: 10.1111/bpa.13056
Abstract: Although prion infections cause cognitive impairment and neuronal death, transcriptional and translational profiling shows progressive derangement within glia but surprisingly little changes within neurons. Here we expressed PrPC selectively in neurons and astrocytes of mice.… read more here.
Keywords: prion diseases; diseases selectively; prpsc; prion ... See more keywords
Conformational diversity in purified prions produced in vitro
Sign Up to like & getrecommendations! Published in 2023 at "PLOS Pathogens"
DOI: 10.1371/journal.ppat.1011083
Abstract: Prion diseases are caused by misfolding of either wild-type or mutant forms of the prion protein (PrP) into self-propagating, pathogenic conformers, collectively termed PrPSc. Both wild-type and mutant PrPSc molecules exhibit conformational diversity in vivo,… read more here.
Keywords: prpsc; conformational diversity; diversity; purified prions ... See more keywords
PMCA-Based Detection of Prions in the Olfactory Mucosa of Patients With Sporadic Creutzfeldt–Jakob Disease
Sign Up to like & getrecommendations! Published in 2022 at "Frontiers in Aging Neuroscience"
DOI: 10.3389/fnagi.2022.848991
Abstract: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder caused by the conformational conversion of the prion protein (PrPC) into an abnormally folded form, named prion (or PrPSc). The combination of the polymorphism at codon… read more here.
Keywords: creutzfeldt jakob; olfactory mucosa; prpsc; jakob disease ... See more keywords
What is the role of lipids in prion conversion and disease?
Sign Up to like & getrecommendations! Published in 2023 at "Frontiers in Molecular Neuroscience"
DOI: 10.3389/fnmol.2022.1032541
Abstract: The molecular cause of transmissible spongiform encephalopathies (TSEs) involves the conversion of the cellular prion protein (PrPC) into its pathogenic form, called prion scrapie (PrPSc), which is prone to the formation of amorphous and amyloid… read more here.
Keywords: prion; prpsc; conversion; prion conversion ... See more keywords
Discrimination of Classical and Atypical BSE by a Distinct Immunohistochemical PrPSc Profile
Sign Up to like & getrecommendations! Published in 2023 at "Pathogens"
DOI: 10.3390/pathogens12020353
Abstract: Bovine spongiform encephalopathy (BSE) belongs to the group of transmissible spongiform encephalopathies and is associated with the accumulation of a pathological isoform of the host-encoded glycoprotein, designated prion protein (PrPSc). Classical BSE (C-type) and two… read more here.
Keywords: prpsc; bse; atypical bse; prpsc profile ... See more keywords