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000136510 1001_ $$0P:(DE-HGF)0$$aResenberger, Ulrike K$$b0$$eCorresponding author
000136510 245__ $$aCellular prion protein mediates toxic signaling of amyloid beta.
000136510 260__ $$aBasel$$bKarger$$c2012
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000136510 520__ $$aPrion diseases in humans and animals comprise a group of invariably fatal neurodegenerative diseases characterized by the formation of a pathogenic protein conformer designated PrP(Sc) and infectious particles denoted prions. The cellular prion protein (PrP(C)) has a central role in the pathogenesis of prion disease. First, it is the precursor of PrP(Sc) and infectious prions and second, its expression on neuronal cells is required to mediate toxic effects of prions. To specifically study the role of PrP(C) as a mediator of toxic signaling, we have developed novel cell culture models, including primary neurons prepared from PrP-deficient mice. Using these approaches we have been able to show that PrP(C) can interact with and mediate toxic signaling of various β-sheet-rich conformers of different origins, including amyloid β, suggesting a pathophysiological role of the prion protein beyond prion diseases.
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000136510 650_7 $$2NLM Chemicals$$aAmyloid beta-Peptides
000136510 650_7 $$2NLM Chemicals$$aPrions
000136510 650_2 $$2MeSH$$aAmyloid beta-Peptides: metabolism
000136510 650_2 $$2MeSH$$aAmyloid beta-Peptides: toxicity
000136510 650_2 $$2MeSH$$aAnimals
000136510 650_2 $$2MeSH$$aHumans
000136510 650_2 $$2MeSH$$aMice
000136510 650_2 $$2MeSH$$aMice, Transgenic
000136510 650_2 $$2MeSH$$aPrions: genetics
000136510 650_2 $$2MeSH$$aPrions: metabolism
000136510 650_2 $$2MeSH$$aProtein Conformation
000136510 650_2 $$2MeSH$$aSignal Transduction: drug effects
000136510 650_2 $$2MeSH$$aSignal Transduction: physiology
000136510 7001_ $$0P:(DE-2719)9000369$$aWinklhofer, Konstanze F$$b1$$udzne
000136510 7001_ $$0P:(DE-2719)9000396$$aTatzelt, Jörg$$b2$$eLast author$$udzne
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000136510 773__ $$0PERI:(DE-600)2126858-7$$a10.1159/000332596$$gVol. 10, no. 1-4, p. 298 - 300$$n1-4$$p298-300$$q10:1-4<298 - 300$$tNeurodegenerative diseases$$v10$$x1660-2862$$y2012
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