Beyond Mad Cows: Demystifying Prion Disease
“Mad cow disease” (bovine spongiform encephalopathy, or BSE) is alarming because of its horrific effects—turning the brain of an infected animal into a sponge-like mass. But a BSE or “mad cow” outbreak also devastates livelihoods and industries: farmers must kill entire herds, after which export markets remain closed to them for years or even decades.
Perhaps most troubling is recent evidence suggesting the disease might not only be transmitted from animal to animal, but could also emerge spontaneously in livestock. As Canada Research Chair in Prion Diseases, Dr. David Westaway is working to uncover the causes of BSE and other prion diseases, with particular attention to new “sporadic” cases.
Prions are infectious agents that cause fatal brain diseases. They are very different from bacteria, viruses and other commonly known causes of infection as they contain only a single molecule—a misfolded protein. Prion diseases are poorly understood; sporadic prion diseases present an even greater challenge, because they can emerge in clean livestock, and may be associated with novel variants that are not only more difficult to combat, but more likely to spread to new species, perhaps including humans.
Building on their extensive prion and Alzheimer’s disease research and a chain of recent discoveries, Westaway and his research team will provide new insight into prion diseases threatening Canadian livestock. Their work will also offer insights into related neurodegenerative diseases like Alzheimer’s and Parkinson’s, which together affect almost one million Canadians.