Prion diseases are transmitted by unconventional infectious agents (prions) generated by the conformational conversion of PrPC, a normal, cell-surface glycoprotein, into PrPSc, a misfolded isoform that propagates itself by a self-templating mechanism. Although PrPSc has commonly been considered the primary neurotoxic species in prion diseases, strong experimental evidence now challenges this dogma and suggests that alternative pathogenic forms of PrP may operate by altering the normal physiological function of PrPC. In the past 15 years, we and others have generated cellular and animal models for studying prion diseases that shed light on important aspects of PrP infectivity, aggregation, and toxicity. In this chapter, we review some of these results and discuss our current understanding of the molecular processes responsible for the formation of aberrant forms of PrP and their acquisition of infectious and toxic properties.

Infectious and pathogenic forms of PrP / Biasini, E.; Harris, D. A.. - (2013), pp. 135-146. [10.1007/978-1-4614-5305-5]

Infectious and pathogenic forms of PrP

Biasini E.;
2013-01-01

Abstract

Prion diseases are transmitted by unconventional infectious agents (prions) generated by the conformational conversion of PrPC, a normal, cell-surface glycoprotein, into PrPSc, a misfolded isoform that propagates itself by a self-templating mechanism. Although PrPSc has commonly been considered the primary neurotoxic species in prion diseases, strong experimental evidence now challenges this dogma and suggests that alternative pathogenic forms of PrP may operate by altering the normal physiological function of PrPC. In the past 15 years, we and others have generated cellular and animal models for studying prion diseases that shed light on important aspects of PrP infectivity, aggregation, and toxicity. In this chapter, we review some of these results and discuss our current understanding of the molecular processes responsible for the formation of aberrant forms of PrP and their acquisition of infectious and toxic properties.
2013
Prions and Diseases: Volume 1, Physiology and Pathophysiology
New York, USA
Springer New York
978-1-4614-5304-8
978-1-4614-5305-5
Biasini, E.; Harris, D. A.
Infectious and pathogenic forms of PrP / Biasini, E.; Harris, D. A.. - (2013), pp. 135-146. [10.1007/978-1-4614-5305-5]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11572/283891
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