Disease-Associated Prion Protein Oligomers Inhibit the 26S Proteasome

The mechanism of cell death in prion disease is unknown but is associated with the production of a misfolded conformer of the prion protein. We report that disease-associated prion protein specifically inhibits the proteolytic β subunits of the 26S proteasome. Using reporter substrates, fluorogenic...

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Veröffentlicht in:	Molecular cell 2007-04, Vol.26 (2), p.175-188
Hauptverfasser:	Kristiansen, Mark, Deriziotis, Pelagia, Dimcheff, Derek E., Jackson, Graham S., Ovaa, Huib, Naumann, Heike, Clarke, Anthony R., van Leeuwen, Fijs W.B., Menéndez-Benito, Victoria, Dantuma, Nico P., Portis, John L., Collinge, John, Tabrizi, Sarah J.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Cell Death - drug effects Cell Death - physiology Cell Line In Vitro Techniques Mice Mice, Transgenic MOLNEURO Nerve Degeneration - enzymology Nerve Degeneration - etiology Nerve Degeneration - pathology Prion Diseases - enzymology Prion Diseases - etiology Prion Diseases - pathology Prions - chemistry Prions - toxicity Protease Inhibitors - chemistry Protease Inhibitors - toxicity Proteasome Endopeptidase Complex - chemistry Proteasome Inhibitors Protein Denaturation Protein Structure, Quaternary Protein Subunits PROTEINS PrPSc Proteins - chemistry PrPSc Proteins - toxicity Recombinant Proteins - chemistry Recombinant Proteins - toxicity Ubiquitin - metabolism
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