Atypical scrapie isolates involve a uniform prion species with a complex molecular signature

Götte, Dorothea; Benestad, S.L.; Laude, H.; Zurbriggen, Andreas; Oevermann, Anna; Seuberlich, Torsten (2011). Atypical scrapie isolates involve a uniform prion species with a complex molecular signature. PLoS ONE, 6(11), e27510. Lawrence, Kans.: Public Library of Science 10.1371/journal.pone.0027510

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The pathobiology of atypical scrapie, a prion disease affecting sheep and goats, is still poorly understood. In a previous study, we demonstrated that atypical scrapie affecting small ruminants in Switzerland differs in the neuroanatomical distribution of the pathological prion protein (PrP(d)). To investigate whether these differences depend on host-related vs. pathogen-related factors, we transmitted atypical scrapie to transgenic mice over-expressing the ovine prion protein (tg338). The clinical, neuropathological, and molecular phenotype of tg338 mice is similar between mice carrying the Swiss atypical scrapie isolates and the Nor98, an atypical scrapie isolate from Norway. Together with published data, our results suggest that atypical scrapie is caused by a uniform type of prion, and that the observed phenotypic differences in small ruminants are likely host-dependant. Strikingly, by using a refined SDS-PAGE technique, we established that the prominent proteinase K-resistant prion protein fragment in atypical scrapie consists of two separate, unglycosylated peptides with molecular masses of roughly 5 and 8 kDa. These findings show similarities to those for other prion diseases in animals and humans, and lay the groundwork for future comparative research.

Item Type:

Journal Article (Original Article)

Division/Institute:

05 Veterinary Medicine > Research Foci > NeuroCenter
05 Veterinary Medicine > Department of Clinical Research and Veterinary Public Health (DCR-VPH) > Experimental Clinical Research
05 Veterinary Medicine > Department of Clinical Research and Veterinary Public Health (DCR-VPH)

UniBE Contributor:

Götte, Dorothea; Zurbriggen, Andreas; Oevermann, Anna and Seuberlich, Torsten

ISSN:

1932-6203

Publisher:

Public Library of Science

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:32

Last Modified:

06 Jun 2018 10:49

Publisher DOI:

10.1371/journal.pone.0027510

Web of Science ID:

000297553900055

BORIS DOI:

10.7892/boris.12303

URI:

https://boris.unibe.ch/id/eprint/12303 (FactScience: 218623)

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