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Science. 2002 Nov 29;298(5599):1785-8. Epub 2002 Oct 17.

Conversion of PrP to a self-perpetuating PrPSc-like conformation in the cytosol.

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Howard Hughes Medical Institute (HHMI), University of Chicago, Chicago, IL 60637, USA.


A rare conformation of the prion protein, PrPSc, is found only in mammals with transmissible prion diseases and represents either the infectious agent itself or a major component of it. The mechanism for initiating PrPSc formation is unknown. We report that PrP retrogradely transported out of the endoplasmic reticulum produced both amorphous aggregates and a PrPSc-like conformation in the cytosol. The distribution between these forms correlated with the rate of appearance in the cytosol. Once conversion to the PrPSc-like conformation occurred, it was sustained. Thus, PrP has an inherent capacity to promote its own conformational conversion in mammalian cells. These observations might explain the origin of PrPSc.

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