Prion protein oligomer and its neurotoxicity

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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 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 (PrP(C)) has remained mysterious, hampe...

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In several neurodegenerative diseases, such as Parkinson, Alzheimer's, Huntington, and prion diseases, the deposition of aggregated misfolded proteins is believed to be responsible for the neurotoxicity that characterizes these diseases. Prion protein (PrP), the protein responsible of prion diseases, has been deeply studied for the peculiar feature of its misfolded oligomers that are able to pr...

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ژورنال

عنوان ژورنال: Acta Biochimica et Biophysica Sinica

سال: 2013

ISSN: 1672-9145,1745-7270

DOI: 10.1093/abbs/gmt037