Analyses of Protease Resistance and Aggregation State of Abnormal Prion Protein across the Spectrum of Human Prions

Prion diseases are characterized by tissue accumulation of a misfolded, β-sheet-enriched isoform (scrapie prion protein (PrP(Sc))) of the cellular prion protein (PrP(C)). At variance with PrP(C), PrP(Sc) shows a partial resistance to protease digestion and forms highly aggregated and detergent-insol...

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Bibliographic Details
Main Authors: Saverioni, Daniela, Notari, Silvio, Capellari, Sabina, Poggiolini, Ilaria, Giese, Armin, Kretzschmar, Hans A., Parchi, Piero
Format: Artigo
Language:Inglês
Published: American Society for Biochemistry and Molecular Biology 2013
Subjects:
Molecular Bases of Disease
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC3784711/
https://ncbi.nlm.nih.gov/pubmed/23897825
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M113.477547
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