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@INPROCEEDINGS{Tatzelt:145359,
author = {Tatzelt, Jörg},
title = {{OR}-28: {T}he cellular prion protein mediatesneurotoxic
signaling of scrapie prions and {A}β},
journal = {Prion},
volume = {6},
number = {Supplement 1},
issn = {1933-6896},
reportid = {DZNE-2020-00715},
pages = {15},
year = {2012},
abstract = {Neuronal dysfunction in different neurodegenerative
diseases,such as Alzheimer disease, Parkinson disease,
polyglutaminediseases or prion diseases is causally linked
to aberrant proteinfolding. Our goal is to reach an
understanding of how neurotoxicconformers are formed within
the context of neuronal cells and interfere with neuronal
integrity. We have previously establisheda cell culture
model to shown that PrPSc-induced cell death isdependent on
the expression of GPI-anchored PrPC and to identify domains
of PrPC required for this activity.1 Employing celllines and
primary neurons prepared from wild-type or PrP0/0mice we
recently showed that PrPC can interact with and mediate
toxic signaling of various β-sheet-rich conformers of
different origins, including Aβ, suggesting a
pathophysiological role ofthe prion protein beyond prion
diseases. Notably, the N-terminaldomain of PrPC mediated
binding to the β-conformers, and asecreted version of the
isolated N-terminal domain interferedwith toxic signaling
via PrPC.2 The mechanisms underlyingthe toxic activity of
β-sheet-rich conformers are far from beingunderstood, but
it is conceivable that different upstream eventsconverge at
similar neurotoxic signaling pathways. We thereforestarted
to define components downstream of PrPC involved intoxic
signaling. Interestingly, Aβ-, PrPSc- and
β-peptide-inducedtoxicity was significantly reduced by
pharmacological blockage ofNMDA receptor activity. In
addition, we observed that PrPSc caninduce mitochondrial
alterations in neurons, such as perinuclearclustering of
mitochondria.},
month = {May},
date = {2012-05-09},
organization = {International Prion Congress—Prion
2012, Amsterdam (The Netherlands), 9
May 2012 - 12 May 2012},
cin = {Ext AG Tatzelt},
ddc = {570},
cid = {I:(DE-2719)5000053},
pnm = {342 - Disease Mechanisms and Model Systems (POF3-342)},
pid = {G:(DE-HGF)POF3-342},
typ = {PUB:(DE-HGF)1 / PUB:(DE-HGF)16},
url = {https://pub.dzne.de/record/145359},
}