Conference focuses on deadly ‘prion’ diseases
Researchers gather to discuss proteins at root of rare neurological illnesses
Researchers from around the world gathered in Edmonton this week to share expertise on a set of mysterious, debilitating and often fatal neurological diseases in humans and animals at the PRION 2019 conference.
“Prion disease is such a rare disease that you really need international collaboration. These meetings are a wonderful place to share ideas and network and get tips on this because you’re never going to solve this disease by yourself,” said Valerie Sim, associate professor of neurology at the Centre for Prions and Protein Folding Diseases at the University of Alberta.
The four-day conference heard from experts working to better categorize, diagnose and treat a wide range of prion and prionlike diseases that have some similarities, such as Alzheimer’s and Parkinson’s. There is debate over what should be categorized as a prion disease, but it can be spontaneous or genetically inherited, like Creutzfeldt-Jakob disease, or infectious, such as mad cow disease. It is always fatal.
A prion is a type of irregular protein particle that can trigger normal proteins in the brain to fold abnormally. And although researchers don’t know exactly how the prion works, they know that its shape is important, said Sim.
“If it turns into the wrong fold, or the wrong shape, that’s what triggers the disease and triggers the death of brain cells. It happens very fast,” said Sim.
When she met her first patient with prion disease, it was both emotionally difficult and intellectually inspiring.
“Seeing how rapidly she went from being able to speak and converse to just staring and saying nothing, and just dying, was amazing and shocking on the same level,” said Sim.
Researchers focused on prions have made huge strides and received important support since mad cow was found in Canada in 2003, Sim said.
“Over the past decade there have been closer and closer models and experimental evidence to suggest what the structure of this prion protein is. It’s not all proven yet, there’s a lot of debate out there still, but every year we’re another step closer,” said Sim.
The conference saw more than 300 attendees from 25 countries, including Stanley Prusiner, who won the Nobel Prize in 1997 for his discovery of prions. Co-hosted by the Centre for Prions and Protein Folding Diseases at the U of A and the Calgary Prion Research Unit at the University of Calgary, the conference wrapped up on Friday.