Current advancements in Amyotrophic Lateral Sclerosis (ALS) research have created some attention as University of Toronto (U of T) researchers present their new findings on the FUS protein. They believe it may be the culprit behind why motor neurons are not being fed properly, which eliminates the ability for neurons to stay healthy and perform effectively.
ALS, sometimes called Lou Gehrig's disease, is a disease that progressively degenerates the motor neurons in the brain and spine, which then causes the inability of the brain to control voluntary muscle movement. As the disease progresses, one may lose their ability to walk, speak, and even breathe. There is currently no cure for ALS, but researchers like St. George-Hyslop, director of U of T's Tanz Centre for Research in Neurodegenerative Diseases, are making advancements toward understanding the source of the problem.
Trying to understand the “underlying causal mechanisms” was the goal of the study, St. George-Hyslop told Patient Daily News in a recent interview. “It’s groundbreaking because we discovered the disease’s mechanism.”
St. George-Hyslop’s “causal mechanism” is an over-gelled FUS protein, which is supposed to gel enough to travel to neurons, then melt to deliver the necessary nutrients.
Instead, the FUS protein does not melt and “kills the nerve by throttling it and preventing it from making new protein in the parts of the cell that desperately need it," St. George-Hyslop said. "The mutations force the gelling process to go further than it should have gone."
What is next for U of T’s research -- now that they can pinpoint the cause -- is to “work to find out how to reverse or stop the process,” said St. George-Hyslop.
The research was recently published in Neuron.
