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  Targeting of the prion protein to the cytosol: mechanisms and consequences

Miesbauer, M., Rambold, A., Winkelhofer, K. F., & Tatzelt, J. (2010). Targeting of the prion protein to the cytosol: mechanisms and consequences. Current Issues in Molecular Biology, 12, 656-657.

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Item Permalink: http://hdl.handle.net/21.11116/0000-0005-C53C-A Version Permalink: http://hdl.handle.net/21.11116/0000-0005-C53D-9
Genre: Journal Article

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https://www.caister.com/cimb/v/v12/109.pdf (Publisher version)
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 Creators:
Miesbauer, Margit1, Author
Rambold, Angelika2, Author              
Winkelhofer, Konstanze F.1, Author
Tatzelt, Jörg1, Author
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1External Organizations, ou_persistent22              
2Max Planck Institute of Immunobiology and Epigenetics, Max Planck Society, ou_2243650              

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 Abstract: Prion diseases are characterized by the conformational transition of the cellular prion protein (PrPC) into an aberrant protein conformer, designated scrapie-prion protein (PrPSc). A causal link between protein misfolding and neurodegeneration has been established for a variety of neurodegenerative disease, such as Alzheimer's disease, Parkinson's disease and polyglutamine diseases, but there is an ongoing debate about the nature of the neurotoxic species and how non-native conformers can damage neuronal populations. PrP is normally imported into the endoplasmic reticulum (ER) and targeted to the outer leaflet of the plasma membrane via a glycosylphosphatidylinositol (GPI) anchor. However, several conditions, such as ER stress or some pathogenic mutations in the PrP gene, can induce the mislocalization of PrP in the cytosol, where it has a neurotoxic potential as demonstrated in cell culture and transgenic mouse models. In this review we focus on intrinsic factors and cellular pathways implicated in the import of PrP into the ER and its mistargeting to the cytosol. The findings summarized here not only reveal a complex regulation of the biogenesis of PrP, but also provide interesting new insight into toxic activities of pathogenic protein conformers and quality control pathways of ER-targeted proteins.

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Language(s): eng - English
 Dates: 2010
 Publication Status: Published in print
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 Rev. Type: Peer
 Identifiers: PMID: 19767654
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Title: Current Issues in Molecular Biology
Source Genre: Journal
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Pages: - Volume / Issue: 12 Sequence Number: - Start / End Page: 656 - 657 Identifier: -