| Molecular interactions between prions as seeds and recombinant prion proteins as substrates resemble the biological interspecies barrier in vitro. | |
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MedLine Citation:
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PMID: 21151607 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Giannantonio Panza; Lars Luers; Jan Stöhr; Luitgard Nagel-Steger; Jürgen Weiss; Detlev Riesner; Dieter Willbold; Eva Birkmann |
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Publication Detail:
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Type: In Vitro; Journal Article; Research Support, Non-U.S. Gov't Date: 2010-12-09 |
Journal Detail:
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Title: PloS one Volume: 5 ISSN: 1932-6203 ISO Abbreviation: PLoS ONE Publication Date: 2010 |
Date Detail:
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Created Date: 2010-12-14 Completed Date: 2011-07-05 Revised Date: 2013-03-05 |
Medline Journal Info:
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Nlm Unique ID: 101285081 Medline TA: PLoS One Country: United States |
Other Details:
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Languages: eng Pagination: e14283 Citation Subset: IM |
Affiliation:
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Institut für Physikalische Biologie, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Amyloid
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chemistry Animals Brain / metabolism Cattle Circular Dichroism Cricetinae Kinetics Mesocricetus Mice Neurodegenerative Diseases / metabolism Prion Diseases / genetics*, metabolism, transmission* Prions / chemistry* Protein Structure, Secondary Recombinant Proteins / chemistry* Sheep Sodium Dodecyl Sulfate / chemistry Species Specificity Ultracentrifugation |
| Chemical | |
Reg. No./Substance:
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0/Amyloid; 0/Prions; 0/Recombinant Proteins; 151-21-3/Sodium Dodecyl Sulfate |
| Comments/Corrections | |
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