Transmissible spongiform encephalopathies (TSEs), or prion diseases, are neurodegenerative disorders characterized by the accumulation in the CNS of insoluble fibrils composed of the abnormally folded isoform (PrPTSE) of the cellular prion protein (PrPC). One of the main challenges in this field is to disclose the mechanisms underlying the still only partially understood PrPC misfolding process and the accumulation of PrPTSE pathological aggregates. In this context, our recent studies, aimed both to the elucidation of the effective role of mutant PrP allotypes in the molecular pathogenesis of human genetic TSEs and to the identification of protein factors associated to PrPTSE in insoluble deposits extracted from brains of experimentally infected animals, will be presented and discussed.

The role of PrP and other proteins in prion diseases

NOTARI, SILVIO;CAPELLARI, SABINA;PARCHI, PIERO;
2009

Abstract

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are neurodegenerative disorders characterized by the accumulation in the CNS of insoluble fibrils composed of the abnormally folded isoform (PrPTSE) of the cellular prion protein (PrPC). One of the main challenges in this field is to disclose the mechanisms underlying the still only partially understood PrPC misfolding process and the accumulation of PrPTSE pathological aggregates. In this context, our recent studies, aimed both to the elucidation of the effective role of mutant PrP allotypes in the molecular pathogenesis of human genetic TSEs and to the identification of protein factors associated to PrPTSE in insoluble deposits extracted from brains of experimentally infected animals, will be presented and discussed.
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S. Principe; F. Cardone; L. Di Francesco; A. Giorgi; G. Mignogna; M. Sbriccoli; R. Galeno; S. Notari; S. Capellari; P. Parchi; J. Rappsilber; B. Maras; M.E. Schininà; M. Pocchiari
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11585/78311
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