The Biology of Mammalian Prions

15 March 2019 - 1:30 PM

Adriano AguzziInstitute of Neuropathology, Univeritäts Spital Zürich, Switzerland

Adriano Aguzzi is professor and director of the Institute of Neuropathology at the University of Zurich. He has devoted the past 25 years to studying the immunological and molecular basis of prion pathogenesis, combining transgenetics with molecular and immunological techniques to clarify the pathogenesis of the disease, and to identify cells and molecules involved in prion neuroinvasion. He is the Founder and Director of the Swiss National Reference Center for Prion Diseases and has developed diagnostic and therapeutic methods in the field of transmissible spongiform encephalopathies. He serves on the editorial board of Science and is the Editor in Chief of the Swiss Medical Weekly; he also serves on the scientific advisory board of philanthropic foundations and biomedical companies. Among other honors, Prof. Aguzzi has won Ernst-Jung Prize, the Robert Koch Prize, and the medal of the European Molecular Biology Organization, and has held two ERC Advanced Grants. 

Location: Amphi Charpak (LPNHE), Tower 22, Pierre et Marie Curie Campus

Abstract :Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases of humans and many animal species caused by prions. The main constituent of prions is PrPSc, an aggregated moiety of the host-derived membrane glycolipoprotein PrPC. Prions were found to encipher many phenotypic, genetically stable TSE variants. The latter is very surprising, since PrPC is encoded by the host genome and all prion strains share the same amino acid sequence. Here I will review what is known about the infectivity, the neurotoxicity, and the neuroinvasiveness of prions. Also, I will explain why I regard the prion strain question as a fascinating challenge – with implications that go well beyond prion science. Finally, I will report some recent results obtained in my laboratory, which is attempting to address the strain question and some other basic issues of prion biology with a “systems” approach that utilizes organic chemistry, photophysics, proteomics, and mouse transgenesis. 

For more information, see the lab website