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Abstract
Prion diseases are rare fatal neurological conditions of humans and animals, one of which (variant Creutzfeldt-Jakob disease) is known to be a zoonotic form of the cattle disease bovine spongiform encephalopathy (BSE). What makes one animal prion disease zoonotic and others not is poorly understood, but it appears to involve compatibility between the prion strain and the host prion protein sequence. Concerns have been raised that the United Kingdom sheep flock may have been exposed to BSE early in the cattle BSE epidemic and that serial BSE transmission in sheep might have resulted in adaptation of the agent, which may have come to phenotypically resemble scrapie while maintaining its pathogenicity for humans. We have modeled this scenario in vitro. Extrapolation from our results suggests that if BSE were to infect sheep in the field it may, with time and in some sheep genotypes, become scrapie-like at the molecular level. However, the results also suggest that if BSE in sheep were to come to resemble scrapie it would lose its ability to affect humans.
Original language | English |
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Pages (from-to) | 26075-26088 |
Number of pages | 14 |
Journal | Journal of Biological Chemistry |
Volume | 289 |
Issue number | 38 |
DOIs | |
Publication status | Published - 19 Sept 2014 |
Keywords / Materials (for Non-textual outputs)
- CREUTZFELDT-JAKOB-DISEASE
- MISFOLDING CYCLIC AMPLIFICATION
- INFECTED SHEEP
- ORAL-TRANSMISSION
- PRP ACCUMULATION
- GREAT-BRITAIN
- BRITISH SHEEP
- SCRAPIE
- PROTEIN
- VARIANT
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Dive into the research topics of 'Genotype-dependent Molecular Evolution of Sheep Bovine Spongiform Encephalopathy (BSE) Prions in Vitro Affects Their Zoonotic Potential'. Together they form a unique fingerprint.Projects
- 1 Finished
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The NCJDSU Tissue Resource: support for continued banking actiities
Ironside, J. (Principal Investigator), Knight, R. (Co-investigator) & Smith, C. (Co-investigator)
1/11/09 → 31/12/11
Project: Research
Profiles
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Marcelo Barria Matus
- Deanery of Clinical Sciences - Senior Research Fellow
- Centre for Clinical Brain Sciences
- Edinburgh Neuroscience
Person: Academic: Research Active (Research Assistant)