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Western University's Richmond Street entrance, August 4, 2017.

Western University researchers received $235,770 in project grants and $50,000 in trainee awards from the ALS Society of Canada to fund three research projects for amyotrophic lateral sclerosis, commonly known as ALS.

Of a total of $3 million awarded by ALS Canada to 12 new research projects, $110,770 was awarded to Western researcher Flavio Beraldo and co-investigators Marco Prado and Vania Prado. The team is researching the development of a test for cognitive impairment in ALS, also known as Lou Gehrig’s disease, using mice models.

ALS is a rapidly progressive neuromuscular disease that is the most common cause of neurological death in Canada with an annual mortality rate of two people per 100,000. Death occurs within two to five years of diagnosis in 80 per cent of cases. There are currently around 3,000 Canadians over the age of 18 who are living with ALS.

“New research has shown that around 50 to 60 per cent of ALS patients, on top of the motor problems, also have some cognitive problems,” Beraldo said. “It's been shown that the cognitive problems are detected earlier than the motor problems, so if we can … have some way to detect cognitive deficits before the motor deficits, we can use these as a kind of diagnosis for ALS even before the disease is established.”

Beraldo and Prado are also members of another research team that was awarded another $125,000 grant. Along with co-investigator Martin Duennwald, they are studying the role of guardian proteins that defend against abnormal ALS protein toxicity.

Finally, a trainee award in the amount of $50,000 was awarded to Sonja Di Gregorio, a Western PhD student in the department of pathology and laboratory medicine who is researching the role of misfolded ALS proteins in cell death using yeast cells.

“We want to understand very basic cellular information that we can then translate up into higher model organisms such as cell culture or mice," Di Gregorio said. "[We] hope that this very basic information will direct us to where we need to go to develop a potential therapy for ALS.” 

Beraldo explained the disease begins with movement impairment. Muscle function then continues to deteriorate until the person stops breathing in the latest stage as the muscles lose the ability to ventilate the lungs. 

“There is no cure for this disease, and there is only one or two [food and drug administration] medications that are approved, but these extend the life of the person two to three months, which is nothing,” Beraldo said. 

“It happens very, very quickly, and oftentimes the families are left financially devastated and their loved one is gone before they’ve even had time to process what’s happened,” Di Gregorio said.

The awards were funded through the ALS Canada Research Program in partnership with the Brain Canada Foundation in efforts to make ALS a treatable disease. 


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