Alberta sets the stage for PRION research

Nov 7, 2018

The recent discovery of chronic wasting disease in three red deer on a farm in Quebec is adding to the growing concern about the spread of the disease and the increase in prevalence.

Chronic wasting disease is a prion disease caused by misfolded prion proteins in the brains of cervids e.g. deer, elk, moose and reindeer.

Like all prion diseases, there is no treatment or cure for CWD. The disease was first detected in Colorado in 1967 and has since spread to 23 US states. It has also been found in the wild in Norway and Finland and in imported cases in South Korea. In Canada it has spread to Alberta, Saskatchewan and now Quebec.

The disease is different from bovine spongiform encephalopathy (BSE) in two important ways: chronic wasting disease is found in cervids that roam in the wild unlike BSE in domestic cows; and prions are found in most parts of a cervid and in fluids unlike in BSE where prions are in contained parts of the cow. These two differences are contributing to the spread of the disease because prions are extremely difficult to kill.

The Quebec cases are significant from a geographical perspective because it the first province in eastern Canada to confirm the disease. These are also the first cases not in a contiguous province. The location of the farm is close to the Ontario border, which is of concern for the government and hunters. Quebec officials have placed bans on hunting in areas around the farm in response to the confirmed cases.

Dr. Stephanie Czub

Dr. Stefanie Czub is an internationally-respected BSE and CWD researcher with the Canadian Food Inspection Agency in Lethbridge who is regularly asked to speak to research and industry groups about her work. She agrees that the spread to Quebec is cause for concern: ‘It is a game changer regarding the way Canada will deal with CWD. It is not confined to the west anymore.’

While CWD hasn’t been scientifically proven to transfer to humans who eat infected meat, which has been proven in the case of BSE, all hunters are encouraged to get their animals tested. Hunters have been submitting animals to the province for testing since 1998. As a result, Alberta has one of the strongest surveillance systems, particularly in the prairie/parkland ecosystem, in North America. In 2017, there were 327 positive cases compared to 592 cases in a 12-year period from 2005 through 2016.

It isn’t all doom and gloom, though. Alberta has the largest concentration of prion researchers in the world because of the long-term research investments made in our province. These researchers also have access to state-of-the-art research facilities in Edmonton and Calgary and large field research facilities in Lethbridge.

Through the Alberta Prion Research Institute, Alberta Innovates is funding research on chronic wasting disease related to protein structure, environmental contamination, possible transfer to other species, vaccinations and decontamination of prions. The innovative research being done in our province has resulted in Alberta being a world leader in prion and protein misfolding diseases research.

It is this international reputation that led to European researchers, policy-makers and regulators looking to Alberta in 2016 for expertise and advice when the first case of CWD in Europe was found in Norway. Alberta has been and continues to be an integral player in international research collaborations and workshops on CWD. Provincial policy-makers and researchers played a significant role in the development of Canada’s CWD management policy.

Alberta and its researchers will continue to shine on the global stage when it hosts PRION 2019 in Edmonton from May 21-24, 2019.