Research Seminar: Progress in understanding prion disease: structural characterisation of mammalian prions
19 October 2017
Dr. Cassandra Terry, UCL Institute for Prion Diseases, London
Tuesday 24th October, 1.00 p.m., Stacey Lecture Theatre 1
Prion diseases are fatal neurodegenerative diseases caused by the misfolding of the prion protein PrP. Obtaining three-dimensional atomic resolution structures of the disease causing structures (prions) is challenging however, we have made great progress in isolating prions from mammalian brain and demonstrating that these structures directly cause disease. Analysis by electron microscopy and atomic force microscopy reveal that ex vivo prions are composed of paired double helical fibrils which are markedly different to non-infectious recombinant fibrils generated in vitro and are the first reported cryo-EM images of bona fide infectious prions.