Stability and Cu(II) binding of prion protein variants related to inherited human prion diseases

From: Biophysical Journal | Date: March 1, 2003| Author: | Copyright information

ABSTRACT All inherited forms of human prion diseases are linked with mutations in the prion protein (PrP) gene. Here we have investigated the stability and Cu(II) binding properties of three recombinant variants of murine full-length PrP(23-231)-containing destabilizing point mutations that are a ssociated with human Gerstmann-Straussler-Scheinker disease (F198S), Creutzfeld-Jakob disease (E200K), and fatal familial insomnia (D178N) by electron paramagnetic resonance and circular dichroism spectroscopy. Furthermore, we analyzed the variants H140S, H177S, and H187S of the isolated C-terminal ...

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