Research: Prion Proteins of Primary Concern

ALBERTA, CANADA - The Alberta Prion Research Institute is funding the largest prion project since its inception in an effort to learn more about the human health risks associated with chronic wasting disease (CWD), a disease that is spreading through deer and elk herds across Saskatchewan and Alberta.

22 April 2008

2 minute read

North America

Alberta Agriculture, Food and Rural Development

Because chronic wasting disease is caused by prion proteins it has often been connected to BSE in cattle and Creutzfeldt-Jacob-Disease (CJD) in humans and the worry is that it may be possible for this disease to cross the species barrier.

Dr. Stefanie Czub of the University of Calgary’s Faculty of Veterinary Medicine, is leading the project worth $5 million over five years.

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"We need to know whether it’s possible for it to cross the species barrier."

Dr. Stephen Moore, Scientific Director for the Prion Institute.

CWD is a progressive, fatal disease of the nervous system, affecting cervids such as mule deer, white-tailed deer and elk. It is not confirmed how CWD is transmitted, but based on experience with the disease in captive deer and elk, both lateral (animal to animal) and environmental (animal to premises to animal) transmissions occur. This makes it different from bovine spongiform encephalopathy (BSE) in cows and much more difficult to manage or control.

“We’re concerned with the possible ramifications CWD could have on human health,” said Dr. Stephen Moore, Scientific Director for the Prion Institute. “In order to continue researching this disease, informing public policy, and advising hunters and ranchers, we need to know whether it’s possible for it to cross the species barrier.”

The research team includes Dr. Czub’s co-principal investigator Dr. Dirk Motzkus, from the Department of Virology and Immunology at the German Primate Center in Goettingen, Germany. The project is also supported by the Canadian Food Inspection Agency, University of Calgary’s Faculties of Medicine and Veterinary Medicine, the Robert Koch Institute in Berlin, Institute of Virology of the Technical University of Munich, and the Medical Faculty of the Georg-August-University Goettingen. Due to its international importance, the project is also co-funded by the Ministry for Science and Culture of Lower Saxony, Germany.

Dr. Czub’s project includes the inoculation of non-human primates with high doses of chronic wasting disease to see whether they develop clinical evidence of the disease. The project model is in accordance with the German Animal Welfare Act, French Animal Welfare Act, relevant EU regulations, and the protocol approved in Germany is also approved by the University of Calgary Health Sciences Animal Care Committee.

The Alberta Prion Research Institute is a $35 million initiative that supports top researchers working on solutions to the serious scientific and socioeconomic challenges associated with prions, the proteins that cause diseases such as Creutzfeldt-Jacob-Disease (CJD) in humans, BSE in cattle, and CWD in deer and elk.