Cassmann Eric D, Mammadova Najiba, Moore S Jo, Benestad Sylvie, Greenlee Justin J
Virus and Prion Research Unit, National Animal Disease Center, Agricultural Research Service, United States Department of Agriculture, Ames, Iowa, United States of America.
Norwegian Veterinary Institute, Oslo, Norway.
PLoS One. 2021 Feb 11;16(2):e0246503. doi: 10.1371/journal.pone.0246503. eCollection 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. The purpose of this study was to test the transmission of the Atypical/Nor98 scrapie agent in three genotypes of Suffolk sheep and characterize the distribution of misfolded prion protein (PrPSc). Ten sheep were intracranially inoculated with brain homogenate from a sheep with Atypical/Nor98 scrapie. All sheep with the ARQ/ARQ and ARQ/ARR genotypes developed Atypical/Nor98 scrapie confirmed by immunohistochemistry, and one sheep with the VRQ/ARQ genotype had detectable PrPSc consistent with Atypical/Nor98 scrapie at the experimental endpoint of 8 years. Sheep with mild early accumulations of PrPSc in the cerebellum had concomitant retinal PrPSc. Accordingly, large amounts of retinal PrPSc were identified in clinically affected sheep and sheep with dense accumulations of PrPSc in the cerebellum.
羊瘙痒病是一种发生在绵羊身上的传染性海绵状脑病。非典型/挪威98型羊瘙痒病发生在对经典羊瘙痒病具有抗性的绵羊身上，被认为是自发产生的。本研究的目的是测试非典型/挪威98型羊瘙痒病病原体在三种基因型萨福克绵羊中的传播情况，并表征错误折叠的朊病毒蛋白（PrPSc）的分布。将10只绵羊颅内接种来自患有非典型/挪威98型羊瘙痒病绵羊的脑匀浆。所有ARQ/ARQ和ARQ/ARR基因型的绵羊都出现了经免疫组织化学证实的非典型/挪威98型羊瘙痒病，一只VRQ/ARQ基因型的绵羊在8年的实验终点时检测到与非典型/挪威98型羊瘙痒病一致的PrPSc。小脑早期PrPSc轻度蓄积的绵羊同时伴有视网膜PrPSc。因此，在临床患病绵羊和小脑PrPSc密集蓄积的绵羊中发现了大量视网膜PrPSc。
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