Review
doi: 10.1080/1350-6120400000723.
Folding and misfolding of the prion protein in the secretory pathway
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- PMID: 15523918
- DOI: 10.1080/1350-6120400000723
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Review
Folding and misfolding of the prion protein in the secretory pathway
Amyloid.
2004 Sep.
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.
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