Human prion diseases exhibit a spectrum of clinical symptoms and pathology. This variability is linked to variation in the 3-dimensional shapes of prions, however, the molecular diversity of human prions across the whole spectrum of human prion diseases is not fully known. In this study, we aim to enhance surveillance of human prion diseases and establish assays for the identification of Chronic Wasting Disease (CWD) infection. CWD is a fatal brain disease caused by prions that affects deer, elk, reindeer and moose. It is reaching epidemic proportions in parts of North America, resulting in increasing human exposure. Currently the risk of the spread of CWD to humans and the characteristics of any resulting disease remain unknown.
About the Researcher:
Stephanie Booth, PhD
Public Health Agency of Canada and University of Manitoba
Dr. Booth completed her D.Phil. degree and postdoctoral training in Virology and Biochemistry at the University of Oxford, prior to relocation to Canada in 1997. She joined the Public Health Agency of Canada’s National Microbiology Laboratory in Winnipeg, Manitoba in 1999, and the Department of Medical Microbiology and Infectious Diseases at the University of Manitoba shortly after. In addition to providing laboratory support for CJD diagnosis and surveillance in Canada, the NML houses Canada’s only level 4 lab, and is a key player in response to outbreaks of infectious diseases around the world. Dr. Booth’s research focuses on prion diseases of humans including the development of innovative molecular techniques for CJD surveillance and diagnosis. These studies include identifying biomarkers for early diagnosis, the development of animal and novel cell culture models of human prion diseases, and the use genomics to determine the molecular mechanisms by which prions kill brain cells.
- The Ricki/Rivka Angelus Memorial Research Grant, contributed by Debbie Callif
- The Sherry Maxwell Fabian Memorial Grant, contributed by Tom Fabian
- The Thomas Lord Charitable Trust
- The Strides for CJD Grant, contributed by the Families of the CJD Foundation
- The CJD Foundation Grant, contributed by the Families of the CJD Foundation