The cellular prion protein mediates neurotoxic signalling of β-sheet-rich conformers independent of prion replication.

ABSTRACT: Formation of aberrant protein conformers is a common pathological denominator of different neurodegenerative disorders, such as Alzheimer's disease or prion diseases. Moreover, increasing evidence indicates that soluble oligomers are associated with early pathological alterations and that oligomeric assemblies of different disease-associated proteins may share common structural features. Previous studies revealed that toxic effects of the scrapie prion protein (PrP(Sc)), a β-sheet-rich isoform of the cellular PrP (PrP(C)), are dependent on neuronal expression of PrP(C). In this study, we demonstrate that PrP(C) has a more general effect in mediating neurotoxic signalling by sensitizing cells to toxic effects of various β-sheet-rich (β) conformers of completely different origins, formed by

SUBMITTER: Resenberger UK 

PROVIDER: S-EPMC3098494 | biostudies-literature | 2011 May

REPOSITORIES: biostudies-literature