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Dr. Turgay Akay, newest member of the ALS Research Group

A unique group of neuroscientists at Dalhousie’s Brain Repair Centre is revealing the mechanisms that cause ALS (amyotrophic lateral sclerosis, or Lou Gehrig’s disease), a fatal neurodegenerative disease that results in progressive muscle paralysis. Most people with the disease eventually succumb to respiratory failure.

"No one else in Canada is studying ALS in such a way, from so many complementary angles," says BRC director, Dr. Victor Rafuse. He played a lead role in building the ALS research group, which includes Dr. Rob Brownstone, who came to Dalhousie from the University of Manitoba in 2000, Dr. James Fawcett,
who joined Dalhousie from the University of Toronto in 2006, Dr. Ying Zhang, who came from the Salk Institute in San Diego in 2010, and Dr. Turgay Akay, who came to Halifax from Columbia University in New York in 2014. 

Together with Dr. Devanand Pinto at the National Research Council in Halifax, the researchers are discovering the molecular makeup of motor neurons—nerve cells in the brain and spinal cord that control movement—and how their function is disrupted by ALS-related genetic mutations. At the same time, they’re learning how ALS affects the excitation of motor neurons, the motor neuron-to-muscle connection, and the ability to move—while developing new techniques for exploring ALS and how to treat it.

"There is currently only one drug available for ALS patients, which can extend life by several months," notes Dr. Rafuse. "Our goal is to help find ways to extend life by several years."

In fact, the Dalhousie/BRC researchers are collaborating with the universities of Montreal, Laval and Calgary, as well as Harvard, to screen compounds that could potentially be used in treating ALS. This collaboration will use new high-throughput ALS drug-screening technology that Dr. Zhang is developing with support from a BRC Knowledge Translation Grant.

Meanwhile, the group’s newest member, Dr. Akay, is working with a revolutionary model he’s developed to study the process of muscle de-innervation and its effects on the ability to move, over time. He’s also learning how circuits in the spinal cord adapt to the massive loss of motor neurons in ALS, in which as many as half of the motor neurons are dead before any symptoms emerge.

"If we could enhance this compensation mechanism, we could improve quality of life for people living with ALS," says Dr. Akay.

Among his numerous ALS projects, Dr. Rafuse is exploring new strategies for re-innervating muscles and even bypassing the motor-neuron-to-muscle connection—for example, to help the diaphragm function.

"It’s early days," Dr. Rafuse says, "but we have a strong group with a lot of synergy to propel the work forward."

Stay tuned for more stories featuring the important work of our Brain Repair Centre researchers.

Wednesday the 13th. © 2017. All rights reserved.. Bridgewater Media Services