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- Title
Porcine Prion Protein as a Paradigm of Limited Susceptibility to Prion Strain Propagation.
- Authors
Espinosa, Juan Carlos; Marín-Moreno, Alba; Aguilar-Calvo, Patricia; Benestad, Sylvie L; Andreoletti, Olivier; Torres, Juan María
- 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.
- Subjects
BOVINE spongiform encephalopathy; PRIONS; PRION diseases; BIOLOGICAL evolution; TRANSGENIC mice
- Publication
Journal of Infectious Diseases, 2021, Vol 223, Issue 6, p1103
- ISSN
0022-1899
- Publication type
journal article
- DOI
10.1093/infdis/jiz646