Article
Diverse effects on the native β-sheet of the human prion protein due to disease-associated mutations.
Biochemistry - 16 Nov 2010
Chen Wei, van der Kamp Marc W, Daggett Valerie
Abstract excerpt
Prion diseases are fatal neurodegenerative disorders that involve the conversion of the normal cellular form of the prion protein (PrP(C)) to a misfolded pathogenic form (PrP(Sc)). There are many genetic mutations of PrP associated with human prion diseases. Three of these point mutations are located at the first strand of the native β-sheet in human PrP: G131V, S132I, and A133V. To understand the underlying...
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