Book/Journal Article (Review Article)

DZNE-2020-02777

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Neuroprotective and neurotoxic signaling by the prion protein.

Resenberger, U. K. ; Winklhofer, K. F.DZNE* ; Tatzelt, J. (Last author)DZNE*

2011
Springer Heidelberg
ISBN: 978-3-642-24066-9 (print), 978-3-642-24067-6 (electronic)

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Please use a persistent id in citations: doi:10.1007/128_2011_160

Abstract: Prion diseases in humans and animals are characterized by progressive neurodegeneration and the formation of infectious particles called prions. Both features are intimately linked to a conformational transition of the cellular prion protein (PrP(C)) into aberrantly folded conformers with neurotoxic and self-replicating activities. Interestingly, there is increasing evidence that the infectious and neurotoxic properties of PrP conformers are not necessarily coupled. Transgenic mouse models revealed that some PrP mutants interfere with neuronal function in the absence of infectious prions. Vice versa, propagation of prions can occur without causing neurotoxicity. Consequently, it appears plausible that two partially independent pathways exist, one pathway leading to the propagation of infectious prions and another one that mediates neurotoxic signaling. In this review we will summarize current knowledge of neurotoxic PrP conformers and discuss the role of PrP(C) as a mediator of both stress-protective and neurotoxic signaling cascades.

Keyword(s): Animals (MeSH) ; Humans (MeSH) ; Mice (MeSH) ; Models, Biological (MeSH) ; Mutation (MeSH) ; Neurodegenerative Diseases: metabolism (MeSH) ; Neurons: metabolism (MeSH) ; Neurons: pathology (MeSH) ; Neurotoxicity Syndromes: metabolism (MeSH) ; Prion Diseases: metabolism (MeSH) ; Prion Diseases: pathology (MeSH) ; Prions: metabolism (MeSH) ; Protein Conformation (MeSH) ; Protein Denaturation (MeSH) ; Protein Folding (MeSH) ; Protein Structure, Tertiary (MeSH) ; Signal Transduction (MeSH) ; Prions

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Contributing Institute(s):

1. Neurobiochemistry (AG Winklhofer)
2. Ext Neurobiochemistry, LMU (Ext AG Tatzelt)

Research Program(s):

1. 341 - Molecular Signaling (POF3-341) (POF3-341)
2. 342 - Disease Mechanisms and Model Systems (POF3-342) (POF3-342)

Appears in the scientific report 2011

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Database coverage:
; Clarivate Analytics Master Journal List ; NCBI Molecular Biology Database ; SCOPUS ; Science Citation Index ; Science Citation Index Expanded ; Web of Science Core Collection

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