Document Detail

Molecular interactions between prions as seeds and recombinant prion proteins as substrates resemble the biological interspecies barrier in vitro.
MedLine Citation:
PMID:  21151607     Owner:  NLM     Status:  MEDLINE    
Prion diseases like Creutzfeldt-Jakob disease in humans, Scrapie in sheep or bovine spongiform encephalopathy are fatal neurodegenerative diseases, which can be of sporadic, genetic, or infectious origin. Prion diseases are transmissible between different species, however, with a variable species barrier. The key event of prion amplification is the conversion of the cellular isoform of the prion protein (PrP(C)) into the pathogenic isoform (PrP(Sc)). We developed a sodiumdodecylsulfate-based PrP conversion system that induces amyloid fibril formation from soluble α-helical structured recombinant PrP (recPrP). This approach was extended applying pre-purified PrP(Sc) as seeds which accelerate fibrillization of recPrP. In the present study we investigated the interspecies coherence of prion disease. Therefore we used PrP(Sc) from different species like Syrian hamster, cattle, mouse and sheep and seeded fibrillization of recPrP from the same or other species to mimic in vitro the natural species barrier. We could show that the in vitro system of seeded fibrillization is in accordance with what is known from the naturally occurring species barriers.
Giannantonio Panza; Lars Luers; Jan Stöhr; Luitgard Nagel-Steger; Jürgen Weiss; Detlev Riesner; Dieter Willbold; Eva Birkmann
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Publication Detail:
Type:  In Vitro; Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-12-09
Journal Detail:
Title:  PloS one     Volume:  5     ISSN:  1932-6203     ISO Abbreviation:  PLoS ONE     Publication Date:  2010  
Date Detail:
Created Date:  2010-12-14     Completed Date:  2011-07-05     Revised Date:  2013-07-03    
Medline Journal Info:
Nlm Unique ID:  101285081     Medline TA:  PLoS One     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e14283     Citation Subset:  IM    
Institut für Physikalische Biologie, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany.
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MeSH Terms
Amyloid / chemistry
Brain / metabolism
Circular Dichroism
Neurodegenerative Diseases / metabolism
Prion Diseases / genetics*,  metabolism,  transmission*
Prions / chemistry*
Protein Structure, Secondary
Recombinant Proteins / chemistry*
Sodium Dodecyl Sulfate / chemistry
Species Specificity
Reg. No./Substance:
0/Amyloid; 0/Prions; 0/Recombinant Proteins; 151-21-3/Sodium Dodecyl Sulfate

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