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Understanding the key features of the spontaneous formation of bona fde prions through a novel methodology that enables their swift and consistent generation
(BMC, 2023-09-07)
Among transmissible spongiform encephalopathies or prion diseases afecting humans, sporadic forms such as spo‑
radic Creutzfeldt–Jakob disease are the vast majority. Unlike genetic or acquired forms ...
Development of a new largely scalable in vitro prion propagation method for the production of infectious recombinant prions for high resolution structural studies
(Public Library of Science, 2019-10-23)
The resolution of the three-dimensional structure of infectious prions at the atomic level is pivotal to understand the pathobiology of Transmissible Spongiform Encephalopathies (TSE), but has been long ...
Bona fide atypical scrapie faithfully reproduced for the first time in a rodent model
(BMC, 2022-12-13)
Atypical Scrapie, which is not linked to epidemics, is assumed to be an idiopathic spontaneous prion disease in small ruminants. Therefore, its occurrence is unlikely to be controlled through selective ...
A Protein Misfolding Shaking Amplificationbased method for the spontaneous generation of hundreds of bona fide prions
(Nature Research, 2024-03-08)
Prion diseases are a group of rapidly progressing neurodegenerative disorders
caused by the misfolding of the endogenous prion protein (PrPC) into a
pathogenic form (PrPSc). This process, despite being ...