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

Eric D Cassmann; Najiba Mammadova; S Jo Moore; Sylvie Benestad; Justin J Greenlee

doi:10.1371/journal.pone.0246503

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

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

Authors

Eric D Cassmann¹, Najiba Mammadova¹, S Jo Moore¹, Sylvie Benestad², Justin J Greenlee¹

Affiliations

¹ Virus and Prion Research Unit, National Animal Disease Center, Agricultural Research Service, United States Department of Agriculture, Ames, Iowa, United States of America.
² Norwegian Veterinary Institute, Oslo, Norway.

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.

Publication types

Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

Animals
Genotype*
Haplotypes
Polymorphism, Genetic*
Prion Proteins / genetics*
Scrapie / genetics*
Scrapie / transmission
Sheep / genetics*

Substances

Prion Proteins

Grants and funding

This research was funded in its entirety by congressionally appropriated funds to the United States Department of Agriculture, Agricultural Research Service. The funders of the work did not influence study design, data collection and analysis, decision to publish, or the preparation of the manuscript. The findings and conclusions in this publication are those of the author(s) and should not be construed to represent any official USDA or U.S. Government determination or policy. This research was supported in part by appointments (N. Mammadova and S. Jo Moore) to the Agricultural Research Service (ARS) Research Participation Program administered by the Oak Ridge Institute for Science and Education (ORISE) through an interagency agreement between the U.S. Department of Energy (DOE) and the U.S. Department of Agriculture (USDA). ORISE is managed by ORAU under DOE contract number DESC0014664. All opinions expressed in this paper are the author’s and do not necessarily reflect the policies and views of USDA, ARS, DOE, or ORAU/ORISE.