Deciphering the Cellular Biology of Prion Diseases
Bovine spongiform encephalopathy (BSE)—commonly known as “mad cow disease”—also has a human variant, called Creutzfeldt-Jakob disease. The consequences of these diseases are so significant that diagnosis of the first BSE case in Canada led to an immediate closure of trade borders for beef and beef products, and a huge economic loss for the country.
Both diseases are prion diseases, a family of rare, progressive and transmissible brain disorders that affect both humans and animals. No therapy is available for these fatal diseases, which can occur in sporadic, genetic or infectious forms. The most contagious prion disease is “chronic wasting disease” affecting deer, elk and moose.
Chronic wasting disease can affect both farmed and wild animals.,S spread of the disease is currently uncontrollable. The infectious agent, the prion , is unique because it consists solely of a misfolded form of a protein produced naturally in the body. Unlike bacteria or viruses, prions don’t require genetic information to propagate.
Dr. Sabine Gilch , Canada Research Chair in Prion Disease Research, aims to decipher how neurons respond to prion infection, and how prions are released and transported by these cells, by using cell-culture models of prion infection. Gilch is also evaluating the effects that new compounds have on the spread of prions and the progression of disease in animal models.
Gilch’s research will improve understanding of the molecular biology of prion diseases, and eventually lead to better treatments for these serious diseases.