Completed Core Projects

“An integrated approach to characterize the structure, dynamics and kinetics of prions”

Six research teams will seek to define the structure of the prion protein, in both “healthy” (normal) and “disease” (misfolded and aggregated in plaques) folding patterns, at a higher level of detail than ever before. Three groups will apply powerful analytical methods (i.e. electron microscopy) to samples of the prion protein, while other teams will develop computer simulations and modeling tools to interpret the data in new ways. In addition to providing accurate protein structures that researchers might use in designing effective drug treatments for prion diseases, this project will also help create new software and commercial methods for analyzing other proteins affecting human health.

“A novel approach to characterize prion aggregates and the prion propagation complex" (phase II)

This project is phase II of a prior Core project (funded 2006-09) and is aimed at learning about the molecular details of the prion misfolding process. Specifically they are attempting to answer three key questions: 1) how does the normal prion (PrPc) actually unfold and aggregate; 2) what does the abnormal prion (PrPsc) actually look like; and 3) are there structural and stability differences between different PrPsc molecules from different species or different sources? In particular, are there fundamental differences between PrPsc made in the test tube and PrPsc made in the brain? By addressing these questions we can potentially develop a better understanding of most prion diseases, better ways of detecting them early and potentially new routes to curing them. In this phase of this project we will use the tools of structural biology, biophysics, biochemistry, nanotechnology, molecular biology and biocomputing, techniques and knowledge developed from phase I, along with some recently developed techniques (optical tweezers, solid state NMR, microwave hydrolysis, essential dynamic modeling) to extend our studies even further. If these approaches are successful we will have answered some of the most fundamental questions in prion biology and, in so doing, laid the foundations to improved detection, treatment and prevention of prion diseases. 

Dr. Wishart and his team completed two related projects exploring the structure of the infectious form of the prion protein (PrP^Sc). They used mass spectrometry and other techniques to investigate what the harmful version of the protein actually looks like and how it differs from the normal, non-infectious form.  Further research, through a Phase II project funded until 2011, may uncover ways to prevent the development of infectious prion proteins and to detect and treat prion diseases.

An animal's susceptibility to BSE infection is determined by many factors, including mode of exposure, immune system, and genetics. Using the completed bovine genome and the tools it provides, this research team will identify the specific set of genes that determine how susceptible to infection and disease a particular cow may be. This will enable new approaches to improve herd monitoring and management, reducing the risk of BSE in Alberta.

An animal's susceptibility to transmissible spongiform encephalopathies (TSEs) is determined by factors such as its genetics, its immune system and how it was exposed to the disease. For many species, the precise mechanisms behind the transmission and progression of prion diseases are still unknown. In humans and sheep, mutations in the prion protein gene dramatically increase susceptibility to disease. Interestingly, there has been no evidence of a similar effect in cattle, although other genes are thought to play a role in the progression and transmission of bovine spongiform encephalopathy (BSE).  Dr. Moore and his team studied mutations across the bovine genome to identify which specific genes determine susceptibility to BSE infection and disease. They discovered a number of genes that are associated with BSE susceptibility or that are activated when BSE is present. This knowledge will help scientists understand the progression of BSE. It can be used to develop new and improved herd management approaches that can reduce the risk of BSE.

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.

Dr. Goddard's research project focused on the socio-economic impacts of BSE within Alberta and Canada. Her research explored: the economic and social vulnerability of farmers, communities and regions to this and potentially other disease outbreaks; consumer and market responses in terms of quantities, prices, trade flows resulting from BSE and all attendant forms of policy intervention; Canadian risk perceptions and the success of risk communication strategies associated with BSE.

The results show that there is a significant degree of heterogeneity in the responses to BSE that occurred in Canada.  Certain farms and types of farms were more affected by BSE than others.  For example, within the province of Alberta between 2001 and 2006 some farmers have left the industry and others have expanded their livestock business.  Also, different types of farms responded in a variety of ways to BSE and other major economic changes, such as the appreciation of the Canadian dollar, had a bigger impact on certain types of beef operations.  To better handle potential risks of animal disease outbreaks insurance programs will need to recognize the heterogeneity in farm level impact.

The location, structure and agricultural dependence of a community factored into the ability of that community to weather the BSE storm.

Heterogeneity in consumption by Canadians was also found.  This can partially be explained by significant variability in risk perceptions and trust in the food industry across the Canadian population.  Some Canadian consumers initially responded by increasing their beef consumption to support the economically devastated farmers, while others chose to stop consuming beef completely.  

International comparisons show there are significant differences in the risk perceptions of consumers in different countries affected by BSE - Canadians are much more like Dutch consumers than they appear to be like Japanese consumers.  Japanese consumers are more pessimistic, have lower levels of trust concerning the food system, and generally worry more than Canadians and Dutch. This may be a significant contributing factor to the heterogeneity in government policy responses across countries such as the higher levels of animal testing proscribed in Japan.

A high level of consistency between media coverage, actual outcomes and risk perceptions associated with BSE in Alberta and Canada may have resulted from effective risk communication strategies by provincial and federal governments. Generally, people took home the message that the Canadian BSE crisis was more about economic devastation for farmers than it was about human health risk.  However, Canadian's general views about the safety of food were shaken through the BSE crisis.