Folding and misfolding of the prion protein in the secretory pathway
- 1 September 2004
- journal article
- review article
- Published by Taylor & Francis in Amyloid
- Vol. 11 (3) , 162-172
- https://doi.org/10.1080/1350-6120400000723
Abstract
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.Keywords
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