Genotype-dependent Molecular Evolution of Sheep Bovine Spongiform Encephalopathy (BSE) Prions in Vitro Affects Their Zoonotic Potential
| Autor: | Michael Jones, Lorenzo González, Marcelo A. Barria, Zuzana Krejciova, Mark Head, James W. Ironside, Martin Jeffrey |
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| Rok vydání: | 2014 |
| Předmět: |
Protein Folding
Glycosylation Genotype Prions animal diseases Bovine spongiform encephalopathy Encephalopathy Scrapie Disease Biology Biochemistry Evolution Molecular Zoonoses mental disorders medicine Animals Humans Molecular Biology Sheep Domestic Transmission (medicine) Zoonosis Brain food and beverages Molecular Bases of Disease Cell Biology medicine.disease Virology nervous system diseases Encephalopathy Bovine Spongiform Protein Misfolding Cyclic Amplification Cattle Protein Processing Post-Translational |
| Zdroj: | Journal of Biological Chemistry. 289:26075-26088 |
| ISSN: | 0021-9258 |
| DOI: | 10.1074/jbc.m114.582965 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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