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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...
Autores principales: | Quaglio, Elena, Restelli, Elena, Garofoli, Anna, Dossena, Sara, De Luigi, Ada, Tagliavacca, Luigina, Imperiale, Daniele, Migheli, Antonio, Salmona, Mario, Sitia, Roberto, Forloni, Gianluigi, Chiesa, Roberto |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2011
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3084828/ https://www.ncbi.nlm.nih.gov/pubmed/21559407 http://dx.doi.org/10.1371/journal.pone.0019339 |
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