CSCC Webinar Lectureship on Critical Care Medicine
Thursday March 7, 2019
08:30 PT / 09:30 MT / 10:30 CT / 11:30 ET / 12:30 AT / 13:00 NL
Creutzfeldt Jakob Disease: Prions, Detection and Surveillance
Zoonotic Diseases & Special Pathogens
National Microbiology Laboratory
Public Health Agency of Canada
Canadian Science Centre for Human and Animal Health
Creutzfeldt-Jakob disease (CJD) is a rare, neurodegenerative, and invariably fatal disease. It affects 1-2 in every million people per year; in Canada there are about 65 cases per year. CJD belongs to a family of human and animal diseases known as ‘prion’ diseases, all of which result in a characteristic appearance of infected brains, which become filled with holes until they resemble sponges when examined under a microscope. A prion is made entirely of a protein that is found in abundance in the brains of mammals. This normal protein can misfold into a shape or conformation that is insoluble and can aggregate leading to death of cells in the brain. It is also able to change the shape of normal prion proteins enabling it to progressively spread throughout the brain. Hereditary CJD is caused by genetic mutations that cause the prion protein to spontaneously change shape into the infectious form. Rarely, prion proteins are acquired from outside the body, for example through contaminated meat as seen during the bovine spongiform encephalopathy (BSE) epidemic in the UK, which led to cases of a new variant form of CJD.
At the conclusion of this session, participants will be able to:
- Understand what a ‘prion’ is and how it differs from other infectious agents
- Understand how human prion diseases arise and how they can (and cannot) be transmitted
- Understand how a diagnosis of CJD is made
- Understand why the national surveillance of human prion diseases is important to public health in Canada
Stephanie Booth is the Public Health Agency of Canada’s senior researcher on prion diseases and has a laboratory at the National Microbiology Laboratory in Winnipeg. She completed her Bachelor’s degree in Microbiology at University College London, followed by a Doctorate in Biochemistry and Virology at the University of Oxford. Her primary research interests include developing innovative molecular techniques for CJD surveillance and understanding the molecular mechanisms by which prions kill brain cells.
There is no charge to attend this lecture but it is necessary to register