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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione