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Top prion researchers from across the country are pooling their laboratory resources, students and expertise to strengthen Canada's efforts to diagnose, treat and hopefully prevent bovine spongiform encephalopathy (BSE) and other prion-related diseases.

On November 24, the Networks of Centres of Excellence (NCE) awarded $5 million per year over seven years to PrioNet, a national network that will accelerate Canada's understanding of and response to BSE ("mad cow" disease) in cattle, chronic wasting disease (CWD) in elk and deer and Creutzfeldt-Jacob disease (CJD), which is fatal to humans.

Funding is being used to establish an administration headquarters at Vancouver Coastal Health on the Vancouver campus of the University of British Columbia (UBC). It will also facilitate collaboration between experts from several research areas, universities and government departments within Canada and other countries. PrioNet plans to award the first research grants by next spring, and has already hired an experienced CEO to head the network – Dr. Ying Gravel, former CEO of the NCE's Canadian Bacterial Diseases Network.

"Most of the funding will be used to develop an intellectual infrastructure for dealing with these diseases, including operating funds for lab facilities, training, meetings and recruitment of high level prion scientists into Canada," says Dr. Neil Cashman, PrioNet's scientific director and one of the world's leading prion researchers.

"We will fund research, but we're not a typical granting council," he adds. "Our success will be defined as the application of basic research and social research to the socio-economic problems posed by prion diseases."

PrioNet will pool the collective expertise of virtually every prion researcher in Canada – more than two dozen from nine Canadian universities. The goal is to triple that number over the next four years, through graduate training and recruiting internationally renowned scientists to Canada. PrioNet expects to train about 75 students, postdoctoral fellows and research associates over the first four years.

"The training through PrioNet will be more comprehensive than what can be offered by a single lab. Students will have an opportunity to train on specialized equipment and facilities available only at certain institutions across the country," says Dr. Cashman, who also holds a Canada Research Chair in Neurodegeneration and Protein Misfolding Diseases at UBC.

PrioNet represents the most significant step Canada has taken to date to build the country's research capacity in prions, and to use this research to safeguard animal and human health. PrioNet will also measure the sociological and economic impact of prion diseases, data that could prove valuable in developing risk management strategies for other disaster scenarios.

Around the world, governments, industries and scientists are scrambling to unravel the mystery of prions and to somehow mitigate or avoid their devastating consequences. A single BSE-infected cow from an Alberta farm in May 2003 has already cost this country an estimated $8 billion to date, and an incalculable human cost of failed farms and industries. More than two years later, there are still restrictions on the export of Canadian beef to the United States.

Partnering with Alberta
PrioNet's closest partner will be the newly formed Alberta Prion Research Institute, which has $38 million available from the Alberta government for prion research. The two organizations will work together to recruit top scientists, deliver a training program for universities, governments and the private sector, and co-fund research projects, as well as share management.

"This is a very comprehensive partnership that will see a total $70 million made available for prion research in Canada. This will be an amazing kick-start to prion science and prion research in Canada," says Dr. Cashman.

Internationally, PrioNet will partner with the Prion Centre for Excellence (NeuroPrion) in France, as well as the TSE Research Platform in Germany on joint research and training. Within Canada, the new NCE will work with Agriculture and Agri-Food Canada, the Public Health Agency of Canada, the Canadian Food Inspection Agency and Health Canada, along with industry groups, including agricultural and biotechnology companies.

Dr. Cashman says they also hope to collaborate with other NCEs, including the Advanced Food and Materials Network. AFMnet's scientific director, Dr. Rickey Yadda, sits on PrioNet's management committee as there are common elements between the two networks' strategies in risk mitigation and public education.

Unlocking the mysteries of prions
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of fatal neurological diseases of humans and animals characterized by deterioration of the central nervous system which causes vacuoles (sponge-like holes) in the brain. They progress rapidly, are untreatable and almost always fatal.

These infectious agents are not conventional microbes, such as viruses and bacteria. Prion infection does not require nucleic acid (DNA or RNA) and spreads through a process more akin to crystallization than biological reproduction.

PrioNet themes: BSE Chronic wasting disease and scrapie Creutzfeldt-Jacob disease prion protein structure and function prion disease risk management

"The prion protein is intimately involved in the infectious activity of prions," explains Dr. Cashman. "This is a normal protein that can go awry by adopting a rogue form which is toxic to brain cells. On the basic science plane, PrioNet will try to understand how this protein becomes subverted in disease, and develop novel diagnostics and therapeutics from this knowledge."

Developing practical research results that can help industry and governments is a priority for PrioNet. As part of its BSE research, the network wants to identify which genes in cattle make them susceptible to BSE, and develop cost-effective ways to detect and eliminate BSE contamination on farms.

Another prion illness, chronic wasting disease, is spreading through Canada's elk and deer populations. There is growing concern that Aboriginal people who rely on these and related animals for food could be at risk of contracting a human variation of the disease.

"Although we don't know for sure if CWD can infect human beings, we want to come to grips with this disease in Canada and attempt to eradicate it as soon as possible."

CJD provides proof that prion diseases in animals can sometimes have fatal consequences for humans. A type of CJD can be contracted through eating products from a BSE-infected animal. This type of CJD is still relatively rare, infecting less than 200 people to date. New alarms were raised last year, however, after scientists discovered that CJD can be transmitted through blood transfusions. Canadian Blood Services is among those supporting PrioNet's NCE application.

"CJD is a challenge we need to be able to face in Canada. Through PrioNet, we will pursue strategies for being able to treat or perhaps even cure this disease," says Dr. Cashman.

Putting this knowledge into the hands of policy makers is a key priority for the new NCE. Working with provincial and federal bureaucracies, it will set up a government policy regulation committee to develop risk assessments to better manage and prevent such outbreaks.

As Dr. Cashman notes: "It is possible that best intended risk analysis could be siloed without some sort of impact on government policy. A major thrust of PrioNet is to ensure that our research is effectively translated to the authorities that can use this knowledge."

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