Alzheimer’s disease is a type of dementia that impairs a person’s memory, thinking and behavior and that gets progressively worse over time. It affects 500,000 Canadians, a number that is thought to triple in the next generation. Though the cause of Alzheimer’s disease is not known, one hypothesis is that it is actually a prion disease.
Probably the most well know prion disease is Bovine Spongiform Encephalopathy, or mad cow disease, which became a household name in Canada in 1993 when a case was discovered in a cow that had been imported from the UK. Prion diseases are characterized by cellular proteins that take on an incorrect 3-dimentional shape which impairs their ability to function within the cell.
According to Westaway, “a protein in a living cell is a bit like a verb in a sentence – it does the work. If there’s no verb, the sentence does not make any sense”. In addition to not working properly, these misfolded proteins can also cause other proteins to fold into the wrong shape. Unfortunately the effect is not localized and these misshaped proteins can move into different areas of the brain causing progressively more damage.
David Westaway, Professor and Director of the Centre for Prions and Protein Folding Disease at the University of Alberta, is interested in the use of genetic models to help understand human neurologic disorders. His lab created a robust model of early-onset Alzheimer’s Disease that is used in many research labs in the US and EU. His group is currently investigating the genetic basis of prion disease susceptibility and the roles that different proteins play in the development of prion diseases as well as the novel roles for prion proteins in protecting brain cells against permanent damage.