The H187R mutation of the human prion protein induces conversion of recombinant prion protein to the PrP(Sc)-like form.

Abstract

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… (More)
DOI: 10.1021/bi100572j

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@article{Hosszu2010TheHM, title={The H187R mutation of the human prion protein induces conversion of recombinant prion protein to the PrP(Sc)-like form.}, author={Laszlo L. P. Hosszu and M. Howard Tattum and Samantha Jones and Clare R. Trevitt and Mark A Wells and J. P. Waltho and John Collinge and Graham S. Jackson and A. R. Clarke}, journal={Biochemistry}, year={2010}, volume={49 40}, pages={8729-38} }