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. 2021 Feb 11;16(2):e0246503.
doi: 10.1371/journal.pone.0246503. eCollection 2021.

Transmission of the atypical/Nor98 scrapie agent to Suffolk sheep with VRQ/ARQ, ARQ/ARQ, and ARQ/ARR genotypes

Eric D Cassmann  1 , Najiba Mammadova  1 , S Jo Moore  1 , Sylvie Benestad  2 , Justin J Greenlee  1
Affiliations

Transmission of the atypical/Nor98 scrapie agent to Suffolk sheep with VRQ/ARQ, ARQ/ARQ, and ARQ/ARR genotypes

Eric D Cassmann et al. PLoS One. .

Abstract

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.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Fig 1
Fig 1. Immunoreactivity of PrPSc in sheep with atypical scrapie.
(A) There is a large amount of PrPSc (red color) within the molecular layer of cerebellum in sheep 958 (ARQ/ARQ). (B) PrPSc (red color) is confined to the spinal trigeminal tract in the medulla oblongata in sheep 948 (ARQ/ARR). (C) The dorsal motor nucleus of the vagus nerve (circle) is devoid of PrPSc in sheep 948. (D) There are multifocal patchy aggregates of PrPSc (red color) in the molecular layer of the cerebellum in sheep 943 (VRQ/ARQ). (E) A small amount of PrPSc (red color) is present in the retina of sheep 943. (F) In sheep 958 there are large amounts of PrPSc (red color) in the plexiform layers of the retina.
See this image and copyright information in PMC

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