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024 7 _ |a 10.1159/000332596
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024 7 _ |a pmid:22156337
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024 7 _ |a 1660-2854
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024 7 _ |a 1660-2862
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037 _ _ |a DZNE-2020-02832
041 _ _ |a English
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100 1 _ |a Resenberger, Ulrike K
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245 _ _ |a Cellular prion protein mediates toxic signaling of amyloid beta.
260 _ _ |a Basel
|c 2012
|b Karger
264 _ 1 |3 print
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|b S. Karger AG
|c 2012-01-01
336 7 _ |a article
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336 7 _ |a ARTICLE
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336 7 _ |a Journal Article
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520 _ _ |a Prion diseases in humans and animals comprise a group of invariably fatal neurodegenerative diseases characterized by the formation of a pathogenic protein conformer designated PrP(Sc) and infectious particles denoted prions. The cellular prion protein (PrP(C)) has a central role in the pathogenesis of prion disease. First, it is the precursor of PrP(Sc) and infectious prions and second, its expression on neuronal cells is required to mediate toxic effects of prions. To specifically study the role of PrP(C) as a mediator of toxic signaling, we have developed novel cell culture models, including primary neurons prepared from PrP-deficient mice. Using these approaches we have been able to show that PrP(C) can interact with and mediate toxic signaling of various β-sheet-rich conformers of different origins, including amyloid β, suggesting a pathophysiological role of the prion protein beyond prion diseases.
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650 _ 7 |a Amyloid beta-Peptides
|2 NLM Chemicals
650 _ 7 |a Prions
|2 NLM Chemicals
650 _ 2 |a Amyloid beta-Peptides: metabolism
|2 MeSH
650 _ 2 |a Amyloid beta-Peptides: toxicity
|2 MeSH
650 _ 2 |a Animals
|2 MeSH
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Mice
|2 MeSH
650 _ 2 |a Mice, Transgenic
|2 MeSH
650 _ 2 |a Prions: genetics
|2 MeSH
650 _ 2 |a Prions: metabolism
|2 MeSH
650 _ 2 |a Protein Conformation
|2 MeSH
650 _ 2 |a Signal Transduction: drug effects
|2 MeSH
650 _ 2 |a Signal Transduction: physiology
|2 MeSH
700 1 _ |a Winklhofer, Konstanze F
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700 1 _ |a Tatzelt, Jörg
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773 1 8 |a 10.1159/000332596
|b : S. Karger AG, 2012-01-01
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|t Neurodegenerative Diseases
|v 10
|y 2012
|x 1660-2862
773 _ _ |a 10.1159/000332596
|g Vol. 10, no. 1-4, p. 298 - 300
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|t Neurodegenerative diseases
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909 C O |o oai:pub.dzne.de:136510
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910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
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910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
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913 1 _ |a DE-HGF
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Marc 21