Effects of polymorphisms in ovine and caprine prion protein alleles on cell-free conversion

<p>Abstract</p> <p>In sheep polymorphisms of the prion gene (<it>PRNP</it>) at the codons 136, 154 and 171 strongly influence the susceptibility to scrapie and bovine spongiform encephalopathy (BSE) infections. In goats a number of other gene polymorphisms were found wh...

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Bibliographic Details
Main Authors: Eiden Martin, Soto Elizabeth, Mettenleiter Thomas C, Groschup Martin H
Format: Article
Language:English
Published: BMC 2011-02-01
Series:Veterinary Research
Online Access:http://www.veterinaryresearch.org/content/42/1/30
Description
Summary:<p>Abstract</p> <p>In sheep polymorphisms of the prion gene (<it>PRNP</it>) at the codons 136, 154 and 171 strongly influence the susceptibility to scrapie and bovine spongiform encephalopathy (BSE) infections. In goats a number of other gene polymorphisms were found which are suspected to trigger similar effects. However, no strong correlation between polymorphisms and TSE susceptibility in goats has yet been obtained from epidemiological studies and only a low number of experimental challenge data are available at present. We have therefore studied the potential impact of these polymorphisms in vitro by cell-free conversion assays using mouse scrapie strain Me7. Mouse scrapie brain derived PrP<sup>Sc </sup>served as seeds and eleven recombinant single mutation variants of sheep and goat PrP<sup>C </sup>as conversion targets. With this approach it was possible to assign reduced conversion efficiencies to specific polymorphisms, which are associated to low frequency in scrapie-affected goats or found only in healthy animals. Moreover, we could demonstrate a dominant-negative inhibition of prion polymorphisms associated with high susceptibility by alleles linked to low susceptibility in vitro.</p>
ISSN:0928-4249
1297-9716