Article
Different structural stability and toxicity of PrP(ARR) and PrP(ARQ) sheep prion protein variants.
Journal of neurochemistry - 1 Dec 2007
Paludi Domenico, Thellung Stefano, Chiovitti Katia, Corsaro Alessandro, Villa Valentina, Russo Claudio, Ianieri Adriana, Bertsch Uwe, Kretzschmar Hans A, Aceto Antonio, Florio Tullio
Abstract excerpt
The polymorphisms at amino acid residues 136, 154, and 171 in ovine prion protein (PrP) have been associated with different susceptibility to scrapie: animals expressing PrP(ARQ) [PrP(Ala136/Arg154/Gln171)] show vulnerability, whereas those that express PrP(ARR) [PrP(Ala136/Arg154/Arg171)] are resistant to scrapie. The aim of this study was to evaluate the in vitro toxic effects of PrP(ARR) and PrP(ARQ) variants...
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