Prion prognosticator: national research chair renews his mission to understand brain behaviour

    David Westaway unravels the mysteries of the mind as the Canada Research Chair in Prion Disease.

    By Amy Hewko on April 13, 2015

    On April 9, the Canada Research Chair Program announced its new and renewed research chairs, including the reappointment of David Westaway as the Tier 1 Canada Research Chair in Prion Disease. Westaway has held the title since 2007.

    Westaway, also the director of the Centre for Prions and Protein Folding Diseases, hopes to spend his new term of seven years understanding the sequence of events that leads to the development of prion and prion-like diseases, specifically the approximately 90 per cent of cases that are considered sporadic.

    Prion diseases are infectious and can be inherited through genetic mutations or transmitted through exposure, such as ingesting beef products that are contaminated with bovine spongiform encephalopathy (BSE), also known as mad cow disease. Prion-like diseases, such as Alzheimer’s or Parkinson’s disease, can be inherited but are not infectious. Sporadic prion and prion-like diseases develop at random, despite a lack of exposure to the infection or genetic mutation.

    “Ninety per cent of Alzheimer’s disease and Parkinson’s disease are sporadic. We’re trying to understand a basic problem: why one little corner of the brain goes wrong and you get an internal infection that radiates,” Westaway explains. “These diseases are ultimately diseases of our own chemistry.”

    Westaway explains that he has particular interest in the brain’s natural quality control measures, which remove misshaped prion proteins in a process known as proteostasis. “The suspicion is that if quality control processes for proteins in our brains go wrong or they lose their power and erode a little bit, then we start making the [misshaped] proteins,” he says, adding that the malformed prions start a domino effect, infecting neighbouring cells.

    Prior to the discovery of the quality control measures, there was an assumption that the body could not fight against prion disease because there isn’t an immune response to this type of infection. But, Westaway notes, he believes that his lab may have discovered a natural self-defence mechanism against prion diseases.

    “I think it came as a bit of a surprise that we might have found that they body is actually doing something,” he says. “If we can understand it better, we might be able to push it to get a stronger protective response.”

    To better understand this defence response, Westaway will conduct genetic experiments where he will infect specific parts of the brain to see what the consequences are. He will use this data to map the “brain geography” of susceptibility to damaging events in an attempt to understand how sporadic prion disease develops and spreads, and how the brain responds to this attack.

    Westaway’s research is currently funded by grants from the Canadian Institutes of Health Research, Alberta Innovates – Bio Solutions and Alberta Innovates – Health Solutions.