Neurobiology of Disease Spongiform Encephalopathy in Transgenic Mice Expressing a Point Mutation in the 2 – 2 Loop of the Prion Protein

Abstract

Transmissible spongiform encephalopathies are fatal neurodegenerative diseases attributed to misfolding of the cellular prion protein, PrP(C), into a �-sheet-rich, aggregated isoform, PrP(Sc). We previously found that expression of mouse PrP with the two amino acid substitutions S170N and N174T, which result in high structural order of the �2-�2 loop in the… (More)

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