Core Projects

$1,928,547

$1,200,000

Bioassays – where animals are inoculated and succumb to prion disease – are the best method for detecting prion infectivity. Unfortunately, infectivity can take months to years. Ordinary mice can be used but are not optimal for detecting human and bovine prions, with genetically-engineered (transgenic, Tg) mice serving this purpose best. Even Tg mice have limitations and it is difficult to compare the performance of mice customized for the detection of prions from different donor species. This team has devised a new “universal” genetic engineering technique for the creation of prion susceptible mice with short delays to the onset of neurological disease. Besides being of practical use, this approach to modify any region of the host prion protein may also provide insights into the nature of prion infectivity.

This project proposes to link the chemistry of the prion protein to the new territory of other nervous system diseases, such as ALS (Lou Gehrig's disease) and the socialization disorder autism-diseases which are at least one thousand times more common than prion diseases. It is believed that a different type or prion protein may operate in other types of brain diseases, which could lead to new ways of thinking about incurable disorders. The project will create changes in the amounts of the various forms of the new membrane protein, and then perform an array of analyses on the behavior and nervous system transmission of laboratory mice. Nervous transmission by electrical impulse can be measured in isolated brain cells, a system that is also convenient to study the effect of stress by adding small amounts of toxins to the fluids bathing the cultures. By these means, the project aims to extend the boundaries of what is considered "prion disease."

$520,500

This study will contribute to a better understanding of the pathogenesis of CWD and the potential risks for human and livestock health, by assessing the risk of primary CWD-transmission into humans through activities like meat consumption or hunting. Non-human primates will be inoculated with muscle homogenate from CWD white-tailed deer. This will be done intracerebrally, orally and through their skin. As controls, they will be inoculated with brain homogenate of CWD white-tailed deer using the same methods. The risk of secondary CWD transmission via blood or blood-derived products will be assessed by blood transfusion of monkey-adapted CWD to naïve recipients. Results from three platforms in the project will be compiled and evaluated to assess the risk of primary or secondary CWD transmission to humans. Results of this risk assessment will greatly contribute to policy decisions including the monitoring of human blood products, CWD surveillance and CWD control in farmed and wild cervids.

Chronic Wasting Disease (CWD), a prion disease affecting members of the deer family, has the potential to afflict both wild and domestic herds and is a significant concern for wildlife programs, regional economies and human health. This project looks at four areas of CWD research: increased surveillance of Alberta's deer herds; identification of genetic risk factors contributing to animal susceptibility; accurate prediction of the disease's spread using population genetics; and radio-collar tracking of animal movement to learn more about the mode and rate of transmission. Project leaders will collaborate with Alberta resource and hunting groups, as well as with national and international research teams to provide the means for effective intervention, monitoring and mitigation of CWD in Alberta.

“TSEs and the social-economic impact in Alberta”

By examining the social and economic impact of BSE and related diseases on Albertans, this project links anthropologists, agricultural economists, and communication and technology experts from Alberta and abroad. Researchers will identify how policy and regulation surrounding BSE affects production and market demand in Canada and major importing countries, and will explore how the public's perceptions of risk and safety affect their behaviour. This project will make important contributions to improving best practices for marketing strategies for Alberta meat products and to future policy formation and crisis responses relevant to BSE and other disease outbreaks in Canada.

“Prion inactivation in the environment”

In Alberta alone, the cattle industry produces an estimated 74,000 tons of specific risk material (SRM) annually. In addition to restricting SRM use in feed and fertilizer supplements, the disposal of SRMs (which include brain, spinal cord and other tissues) including beef cattle mortalities, under new proposal regulations, will require permits obtained through the Canadian Food Inspection Agency. Little is known about infectivity of prions in the large volume of wastewater generated in rendering plants and slaughterhouses and the subsequent impact they may have on the environment. In order to minimize existing and future impacts of BSE on the Canadian economy, there is a need to develop applied methods and tools related to detection and decontamination of prions in water, wastewater, industrial wastes, SRM, and other environmental matrices such as compost.