Ludwig-Maximilians-Universität, Chair of Metabolic Biochemistry

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Folding and misfolding of the prion protein in the secretory pathway

Amyloid 11(3): 162-72

Authors/Editors: Winklhofer KF
Publication Date: 2004
Type of Publication: Review
A hallmark of prion diseases in humans and animals is the conversion of the cellular prion protein PrPc to a pathogenic isoform, denoted PrPSc. PrPSc is characterized by distinct biochemical and biophysical properties; in addition, it is the major component of infectious prions. All available data indicate that the only difference between PrPc and PrPSc resides in their conformation, emphasizing a critical role of protein folding in the pathogenesis of prion diseases.

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