Expression of mutant or cytosolic PrP in transgenic mice and cells is not associated with endoplasmic reticulum stress or proteasome dysfunction.

Expression of mutant or cytosolic PrP in transgenic mice and cells is not associated with endoplasmic reticulum stress or proteasome dysfunction.

The cellular pathways activated by mutant prion protein (PrP) in genetic prion diseases, ultimately leading to neuronal dysfunction and degeneration, are not known. Several mutant PrPs misfold in the early secretory pathway and reside longer in the endoplasmic reticulum (ER) possibly stimulating ER...

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Main Authors: Elena Quaglio, Elena Restelli, Anna Garofoli, Sara Dossena, Ada De Luigi, Luigina Tagliavacca, Daniele Imperiale, Antonio Migheli, Mario Salmona, Roberto Sitia, Gianluigi Forloni, Roberto Chiesa
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2011-04-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3084828?pdf=render

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http://europepmc.org/articles/PMC3084828?pdf=render

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