Results 1 to 10 of about 7,028 (118)
Membrane-anchored PrPSc is the trigger for prion synaptotoxicity. [PDF]
PLoS PathogensThe mechanism by which prions composed of PrPSc cause the neuropathological aberrations characteristic of prion diseases remains elusive. Previous studies have defined a synaptotoxic signaling pathway in which extracellular PrPSc stimulates NMDA receptor-
Jean R P Gatdula +4 more
doaj +2 more sources
The prion 2018 round tables (I): the structure of PrPSc
Prion, 2019 Understanding the structure of PrPSc is without doubt a sine qua non to understand not only PrPSc propagation, but also critical features of that process such as the strain phenomenon and transmission barriers.
Holger Wille +2 more
exaly +3 more sources
Propagation of PrPSc in mice reveals impact of aggregate composition on prion disease pathogenesis
Communications Biology, 2023 Infectious prions consist of PrPSc, a misfolded, aggregation-prone isoform of the host’s prion protein. PrPSc assemblies encode distinct biochemical and biological properties.
Sheng Chun Chang +6 more
doaj +1 more source
Acta Neuropathologica Communications, 2020 In the human prion disease Creutzfeldt-Jakob disease (CJD), different CJD neuropathological subtypes are defined by the presence in normal prion protein (PrPC) of a methionine or valine at residue 129, by the molecular mass of the infectious prion ...
Anne Ward +5 more
doaj +1 more source
PLoS Pathogens, 2021 Prion diseases are transmissible neurodegenerative disorders that affect mammals, including humans. The central molecular event is the conversion of cellular prion glycoprotein, PrPC, into a plethora of assemblies, PrPSc, associated with disease ...
Leonardo M Cortez +7 more
doaj +1 more source
Distribution of peripheral PrP(Sc) in sheep with naturally acquired scrapie. [PDF]
PLoS ONE, 2014 Accumulation of prion protein (PrPSc) in the central nervous system is the hallmark of transmissible spongiform encephalopathies. However, in some of these diseases such as scrapie or chronic wasting disease, the PrPSc can also accumulate in other ...
María Carmen Garza +4 more
doaj +1 more source
Analysis of prion strains by PrPSc profiling in sporadic Creutzfeldt-Jakob disease. [PDF]
PLoS Medicine, 2006 BACKGROUND: Prion diseases are a group of invariably fatal neurodegenerative disorders affecting humans and a wide range of mammals. An essential part of the infectious agent, termed the prion, is composed of an abnormal isoform (PrPSc) of a host-encoded
Gaby Schoch +6 more
doaj +1 more source
Scientific Reports, 2021 Misfolding of the cellular prion protein, PrPC, into the amyloidogenic isoform, PrPSc, which forms infectious protein aggregates, the so-called prions, is a key pathogenic event in prion diseases.
Hideyuki Hara +6 more
doaj +1 more source
Detecting a psoriatic antigen analogous to infectious prion proteins
Инфекция и иммунитет, 2019 Until now, psoriatic antigen as a specific antigen derived from some infectious agent potentially related to origin of psoriasis has not been identified, thereby strongly arguing against infectious theory of psoriasis.
B. F. Sinitsyn
doaj +1 more source
Recombinant PrPSc shares structural features with brain-derived PrPSc: Insights from limited proteolysis. [PDF]
PLoS Pathogens, 2018 Very solid evidence suggests that the core of full length PrPSc is a 4-rung β-solenoid, and that individual PrPSc subunits stack to form amyloid fibers. We recently used limited proteolysis to map the β-strands and connecting loops that make up the PrPSc
Alejandro M Sevillano +18 more
doaj +1 more source