Article
The H187R mutation of the human prion protein induces conversion of recombinant prion protein to the PrP(Sc)-like form.
Biochemistry - 12 Oct 2010
Hosszu Laszlo L P, Tattum M Howard, Jones Samantha, Trevitt Clare R, Wells Mark A, Waltho Jonathan P, Collinge John, Jackson Graham S, Clarke Anthony R
Abstract excerpt
Prion diseases are associated with a conformational switch in the prion protein (PrP) from its normal cellular form (denoted PrP(C)) to a disease-associated "scrapie" form (PrP(Sc)). A number of PrP(Sc)-like conformations can be generated by incubating recombinant PrP(C) at low pH, indicating that protonation of key residues is likely to destabilize PrP(C), facilitating its conversion to PrP(Sc). Here, we examine...
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