Results 21 to 30 of about 7,097 (179)
Inoculation of scrapie with the self-assembling RADA-peptide disrupts prion accumulation and extends hamster survival. [PDF]
PLoS ONE, 2009 Intracerebral inoculation of 263K Scrapie brain homogenate (PrPsc) with a self-assembling RADA-peptide (RADA) significantly delayed disease onset and increased hamster survival.
Robert Hnasko, Cathrin E Bruederle
doaj +1 more source
Heterogeneity of the Abnormal Prion Protein (PrPSc) of the Chandler Scrapie Strain
Pathogens, 2013 The pathological prion protein, PrPSc, displays various sizes of aggregates. In this study, we investigated the conformation, aggregation stability and proteinase K (PK)-sensitivity of small and large PrPSc aggregates of mouse-adapted prion strains.
Takashi Yokoyama +5 more
doaj +1 more source
PLoS ONE, 2021 Scrapie is a transmissible spongiform encephalopathy that occurs in sheep. Atypical/Nor98 scrapie occurs in sheep that tend to be resistant to classical scrapie and it is thought to occur spontaneously.
Eric D Cassmann +4 more
doaj +1 more source
What Is Our Current Understanding of PrPSc-Associated Neurotoxicity and Its Molecular Underpinnings?
Pathogens, 2017 The prion diseases are a collection of fatal, transmissible neurodegenerative diseases that cause rapid onset dementia and ultimately death. Uniquely, the infectious agent is a misfolded form of the endogenous cellular prion protein, termed PrPSc ...
Daniel Hughes, Mark Halliday
doaj +1 more source
Prions amplify through degradation of the VPS10P sorting receptor sortilin.
PLoS Pathogens, 2017 Prion diseases are a group of fatal neurodegenerative disorders caused by prions, which consist mainly of the abnormally folded isoform of prion protein, PrPSc.
Keiji Uchiyama +7 more
doaj +1 more source
Toward Molecular Dissection of PrPC-PrPSc Interactions [PDF]
Journal of Biological Chemistry, 2007 Direct interaction between endogenous cellular prion protein (PrP(C)) and misfolded, disease-associated (PrP(Sc)) conformers is a key event in prion propagation, which precedes templated conversion of PrP(C) into nascent PrP(Sc) and prion infectivity. Although almost none of the molecular details of this pivotal process are understood, the persistence ...
Laura, Solforosi +5 more
openaire +2 more sources
Inflammatory response of microglia to prions is controlled by sialylation of PrPSc
Scientific Reports, 2018 Neuroinflammation is recognized as one of the obligatory pathogenic features of neurodegenerative diseases including Alzheimer’s, Parkinson’s or prion diseases.
Saurabh Srivastava +5 more
doaj +1 more source
Nanomaterials, 2018 Prion protein (PrPSc) has drawn widespread attention due to its pathological potential to prion diseases. In this work, we constructed a novel surface plasmon resonance (SPR) detection assay involving magnetic microspheres (MMs) and its controlled ...
Zhichao Lou +4 more
doaj +1 more source
Variably Protease-Sensitive Prionopathy: Two New Cases With Motor Neuron-Dementia Syndrome. [PDF]
Ann Clin Transl NeurolABSTRACT We describe two patients with variably protease‐sensitive prionopathy (VPSPr) who developed progressive upper motor neuron symptoms, insomnia, behavioral and cognitive decline, compatible with primary lateral sclerosis associated with frontotemporal dementia (FTD).
Erro ME +10 more
europepmc +2 more sources
Frontiers in Aging Neuroscience, 2022 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 129 of
Federico Angelo Cazzaniga +30 more
doaj +1 more source