Porcine Prion Protein As a Paradigm of Limited Susceptibility to Prion Strain Propagation

Overview

Journal J Infect Dis

Publisher Oxford University Press

Specialty Infectious Diseases

Date 2020 Jan 11

PMID 31919511

Citations 9

Authors

Juan Carlos Espinosa

Alba Marin-Moreno

Patricia Aguilar-Calvo

Sylvie L Benestad

Olivier Andreoletti

Juan Maria Torres

Affiliations

Soon will be listed here.

Abstract

Although experimental transmission of bovine spongiform encephalopathy (BSE) to pigs and transgenic mice expressing pig cellular prion protein (PrPC) (porcine PrP [PoPrP]-Tg001) has been described, no natural cases of prion diseases in pig were reported. This study analyzed pig-PrPC susceptibility to different prion strains using PoPrP-Tg001 mice either as animal bioassay or as substrate for protein misfolding cyclic amplification (PMCA). A panel of isolates representatives of different prion strains was selected, including classic and atypical/Nor98 scrapie, atypical-BSE, rodent scrapie, human Creutzfeldt-Jakob-disease and classic BSE from different species. Bioassay proved that PoPrP-Tg001-mice were susceptible only to the classic BSE agent, and PMCA results indicate that only classic BSE can convert pig-PrPC into scrapie-type PrP (PrPSc), independently of the species origin. Therefore, conformational flexibility constraints associated with pig-PrP would limit the number of permissible PrPSc conformations compatible with pig-PrPC, thus suggesting that pig-PrPC may constitute a paradigm of low conformational flexibility that could confer high resistance to the diversity of prion strains.

Citing Articles

Genetic characterization of the prion protein gene in camels () with comments on the evolutionary history of prion disease in Cetartiodactyla.

Wright E, Reddock M, Roberts E, Legesse Y, Perry G, Bradley R PeerJ. 2024; 12:e17552.

PMID: 38948234 PMC: 11214740. DOI: 10.7717/peerj.17552.

Classical BSE dismissed as the cause of CWD in Norwegian red deer despite strain similarities between both prion agents.

Marin-Moreno A, Benestad S, Barrio T, Pirisinu L, Espinosa J, Tran L Vet Res. 2024; 55(1):62.

PMID: 38750594 PMC: 11097568. DOI: 10.1186/s13567-024-01320-y.

Experimental transmission of ovine atypical scrapie to cattle.

Konold T, Spiropoulos J, Hills J, Abdul H, Cawthraw S, Phelan L Vet Res. 2023; 54(1):98.

PMID: 37864218 PMC: 10589953. DOI: 10.1186/s13567-023-01224-3.

New Drosophila models to uncover the intrinsic and extrinsic factors that mediate the toxicity of the human prion protein.

Myers R, Sanchez-Garcia J, Leving D, Melvin R, Fernandez-Funez P Dis Model Mech. 2022; 15(4).

PMID: 35142350 PMC: 9093039. DOI: 10.1242/dmm.049184.

Classical BSE prions emerge from asymptomatic pigs challenged with atypical/Nor98 scrapie.

Marin B, Otero A, Lugan S, Espinosa J, Marin-Moreno A, Vidal E Sci Rep. 2021; 11(1):17428.

PMID: 34465826 PMC: 8408226. DOI: 10.1038/s41598-021-96818-2.

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