New In vitro and in vivo models of acute prion neurotoxicity

May 19th 2017
Versione stampabile

Venue: Edificio Povo 2, via Sommarive nr. 9, Povo (Tn) - Room B102
 At 2:00 p.m.

  • Steven Collins - Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR), Department of Medicine, the University of Melbourne, Australia

There is a great need for the development of effective therapies for prion diseases, a class of fatal neurodegenerative conditions which in human may occur sporadically, genetically, or horizontally transmitted. Although misfolding of normal prion protein (PrPC) into abnormal conformers (PrPSc) is critical for prion disease pathogenesis, our current understanding of the underlying molecular pathophysiology is rudimentary. The relative lack of biologically relevant models has contributed to slow progress in this area. To assist in redressing these deficiencies we have recently developed both an in vitro and an in vivo model of acute prion neurotoxicity. Our models offer important new approaches for providing mechanistic insights into the acute neurotoxicity and synaptic impairment underlying prion disease, enhancing prospects for development of targeted effective therapies.