Prion protein interconversions†

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Inhibition of interactions and interconversions of prion protein isoforms by peptide fragments from the C-terminal folded domain.

The formation of protease-resistant prion protein (PrP-res or PrP(Sc)) involves selective interactions between PrP-res and its normal protease-sensitive counterpart, PrP-sen or PrP(C). Previous studies have shown that synthetic peptide fragments of the PrP sequence corresponding to residues 119-136 of hamster PrP (Ha119-136) can selectively block PrP-res formation in cell-free systems and scrap...

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The chemical nature of prions and the mechanism by which they propagate are now reasonably well understood. In contrast, much less is known about the identity of the toxic prion protein (PrP) species that are responsible for neuronal death, and the cellular pathways that these forms activate. In addition, the normal, physiological function of cellular PrP (PrP(C)) has remained mysterious, hampe...

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Prion Neurotoxicity 51 Prion Neurotoxicity: Insights from Prion Protein Mutants

The chemical nature of prions and the mechanism by which they propagate are now reasonably well understood. In contrast, much less is known about the identity of the toxic prion protein (PrP) species that are responsible for neuronal death, and the cellular pathways that these forms activate. In addition, the normal, physiological function of cellular PrP (PrPC) has remained mysterious, hamperi...

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Prion protein and aging

The cellular prion protein (PrP(C)) has been widely investigated ever since its conformational isoform, the prion (or PrP(Sc)), was identified as the etiological agent of prion disorders. The high homology shared by the PrP(C)-encoding gene among mammals, its high turnover rate and expression in every tissue strongly suggest that PrP(C) may possess key physiological functions. Therefore, defini...

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ژورنال

عنوان ژورنال: Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences

سال: 2001

ISSN: 0962-8436,1471-2970

DOI: 10.1098/rstb.2000.0765