Prions are responsible for a heterogeneous group of fatal neurodegenerative diseases. They can be sporadic, genetic, or infectious disorders involving post-translational modifications of the cellular prion protein (PrP(C)). Prions (PrP(Sc)) are characterized by their infectious property and intrinsic ability to convert the physiological PrP(C) into the pathological form, acting as a template. The "protein-only" hypothesis, postulated by Stanley B. Prusiner, implies the possibility to generate de novo prions in vivo and in vitro. Here we describe major milestones towards proving this hypothesis, taking into account physiological environment/s, biochemical properties and interactors of the PrP(C).

De novo mammalian prion synthesis / Benetti, F; Legname, Giuseppe. - In: PRION. - ISSN 1933-6896. - 3:4(2009), pp. 213-219. [10.4161/pri.3.4.10181]

De novo mammalian prion synthesis

Legname, Giuseppe
2009-01-01

Abstract

Prions are responsible for a heterogeneous group of fatal neurodegenerative diseases. They can be sporadic, genetic, or infectious disorders involving post-translational modifications of the cellular prion protein (PrP(C)). Prions (PrP(Sc)) are characterized by their infectious property and intrinsic ability to convert the physiological PrP(C) into the pathological form, acting as a template. The "protein-only" hypothesis, postulated by Stanley B. Prusiner, implies the possibility to generate de novo prions in vivo and in vitro. Here we describe major milestones towards proving this hypothesis, taking into account physiological environment/s, biochemical properties and interactors of the PrP(C).
2009
https://doi.org/10.4161/pri.3.4.10181
Benetti, F; Legname, Giuseppe
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.11767/12831
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