Mutant knock-in mice display enhanced susceptibility to pure prion protein fibrils.

Abstract excerpt

Prion diseases manifest clinically in three different forms. Sporadic and infectious forms of prion disease are caused by the conversion of WT, cellular prion protein (PrPC) into its pathogenic conformer (PrPSc). In contrast, genetic forms of prion diseases are caused by mutations in the PrP sequence that promote mutant PrPSc formation. When reconstituted with either polyanionic or lipid cofactors, purified PrPC...

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