Article
Nucleation-dependent conformational conversion of the Y145Stop variant of human prion protein: structural clues for prion propagation.
Proceedings of the National Academy of Sciences of the United States of America - 14 Oct 2003
Kundu Bishwajit, Maiti Nilesh R, Jones Eric M, Surewicz Krystyna A, Vanik David L, Surewicz Witold K
Abstract excerpt
One of the most intriguing disease-related mutations in human prion protein (PrP) is the Tyr to Stop codon substitution at position 145. This mutation results in a Gerstmann-Straussler-Scheinker-like disease with extensive PrP amyloid deposits in the brain. Here, we provide evidence for a spontaneous conversion of the recombinant polypeptide corresponding to the Y145Stop variant (huPrP23-144) from a monomeric...
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