Abstract:
:Classical bovine spongiform encephalopathy (BSE) is the only zoonotic prion disease described to date. Although the zoonotic potential of atypical BSE prions have been partially studied, an extensive analysis is still needed. We conducted a systematic study by inoculating atypical BSE isolates from different countries in Europe into transgenic mice overexpressing human prion protein (PrP): TgMet129, TgMet/Val129, and TgVal129. L-type BSE showed a higher zoonotic potential in TgMet129 mice than classical BSE, whereas Val129-PrP variant was a strong molecular protector against L-type BSE prions, even in heterozygosis. H-type BSE could not be transmitted to any of the mice. We also adapted 1 H- and 1 L-type BSE isolate to sheep-PrP transgenic mice and inoculated them into human-PrP transgenic mice. Atypical BSE prions showed a modification in their zoonotic ability after adaptation to sheep-PrP producing agents able to infect TgMet129 and TgVal129, bearing features that make them indistinguishable of sporadic Creutzfeldt-Jakob disease prions.
journal_name
Emerg Infect Disjournal_title
Emerging infectious diseasesauthors
Marín-Moreno A,Huor A,Espinosa JC,Douet JY,Aguilar-Calvo P,Aron N,Píquer J,Lugan S,Lorenzo P,Tillier C,Cassard H,Andreoletti O,Torres JMdoi
10.3201/eid2606.181790subject
Has Abstractpub_date
2020-06-01 00:00:00pages
1130-1139issue
6eissn
1080-6040issn
1080-6059journal_volume
26pub_type
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