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Why Prion Research

Prion Disease is Serious

Transmissible spongiform encephalopathies (TSEs)-also known as prion diseases-are diseases that affect the brains of humans and other mammals. They can be infectious and cause fatal neurological disease.

TSEs come in many variations that affect many species. Bovine spongiform encephalopathy (BSE, or mad cow disease) affects livestock. Scrapie affects sheep and goats. Chronic wasting disease (CWD) affects elk, moose and other members of the deer family (cervids). Creutzfeldt-Jacob disease (CJD) is a human TSE.

The agent that causes TSEs is believed to be the abnormal, misfolded form of a protein called a prion. The prion protein, PrP, can exist in two forms: normal and abnormal. The normal prion protein, PrP^c, is present tissues of humans and other mammals, but its function is unknown. Sometimes normal prion proteins misfold into an abnormal form, PrP^Sc, which causes other normal prion proteins to misfold. The result is a domino-like effect in which all normal prion proteins in a cell are converted to the abnormal infectious form that causes cells to die and eventually leads to the death of the infected animal. 

We do not know why or how prions and other proteins misfold, or what mechanism causes the nerve cells in which they collect to die. We do know that diseases caused by abnormal prions are devastating and far-reaching in terms of their social and economic costs. So far, we cannot cure these diseases.

That's why the work of the Alberta Prion Research Institute is so important.

TSEs can result in economic devastation and loss of wildlife and animals. They can endanger our food supply and threaten human health.

• In 2003, Canada was the world's third-largest exporter of beef with a market of $4.1 billion. It is estimated that the BSE crisis cost Canadians $11 million per day while borders were closed. As of March 31, 2010, 18 Canadian cows with BSE have been identified-17 in Canada and one in the United States-with one identified in 2009 and the latest in February 2010.
• The first case of chronic wasting disease (CWD) found in wild deer in Alberta was in 2005. By March 2010, 74 cases had been detected in wild deer in the province. The potential spread of the disease from wild to farmed populations of elk and deer poses significant economic concerns, particularly for farmers who trade in live elk and elk products. Its spread may have an impact on herd size, hunting and tourism industries, and long-term effects on the food and hunting of Aboriginal communities.
• Creutzfeldt-Jakob disease is estimated to affect one person per million per year. In Canada, this translates to about 34 new cases annually. Researchers are also investigating ways in which other proteins unfold in diseases like Alzheimer's disease and Lou Gehrig's disease (amyotrophic lateral sclerosis, or ALS).