Article
Theoretical studies of sequence effects on the conformational properties of a fragment of the prion protein: implications for scrapie formation.
Chemistry & biology - 1 May 1995
Kazmirski S L, Alonso D O, Cohen F E, Prusiner S B, Daggett V
Abstract excerpt
BACKGROUND: Prion diseases are neurodegenerative disorders that appear to be due to a conformational change, involving the conversion of alpha-helices in the normal, cellular isoform of the prion protein (PrPC) to beta-structure in the infectious scrapie form (PrPSc). One form of Gerstmann-Sträussler-Scheinker syndrome (GSS), an inherited prion disease, is caused by mutation of Ala117 of PrPC to Val. We therefore...
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