Bona fide atypical scrapie faithfully reproduced for the first time in a rodent model.

ABSTRACT: 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 breeding or other strategies as it is done for classical scrapie outbreaks. Its spontaneous nature and its sporadic incidence worldwide is reminiscent of the incidence of idiopathic spontaneous prion diseases in humans, which account for more than 85% of the cases in humans. Hence, developing animal models that consistently reproduce this phenomenon of spontaneous PrP misfolding, is of importance to study the pathobiology of idiopathic spontaneous prion disorders. Transgenic mice overexpressing sheep PrP^C with I112 polymorphism (TgShI112, 1-2 × PrP levels compared to sheep brain)

SUBMITTER: Vidal E 

PROVIDER: S-EPMC9749341 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature