Breakthrough research on fruit flies may pave way for ALS cure
The work on fruit flies in the lab shows how protein interactions can preserve or prevent the nerve cell death that is a hallmark of ALS.
Amyotrophic lateral sclerosis (ALS or Lou Gehrig’s disease named for the Yankee baseball hero who died from it) has been viewed as a severely disabling and inevitably fatal neurological disorder for many years. But now, Canadian researchers claim they have made a “breakthrough” leading to a cure, even though it will take about five years to begin clinical trials on patients.A team at Western University in Ontario led by Dr. Michael Strong, who is resident, Canadian Institutes of Health Research, has uncovered a potential path toward a cure for ALS, among whose victims was famed Prof. Stephen Hawking, the English theoretical physicist and cosmologist.
The work on fruit flies in the lab shows how protein interactions can preserve or prevent the nerve cell death that is a hallmark of ALS and is the culmination of decades of research at the university.“As a doctor, it’s been so important for me to be able to sit down with a patient or their family and say to them, ‘we’re trying to stop this disease,’” said Strong, a clinician-scientist who has devoted his career to finding a cure for ALS. He and his colleagues have just published their findings in the journal Brain under the title “Mitigation of TDP-43 toxic phenotype by an RGNEF fragment in amyotrophic lateral sclerosis models.”
“It’s been 30 years of work to get here; three decades of looking after families and patients and their loved ones when all we had was hope. This gives us reason to believe we’ve discovered a path to treatment,” Strong said with enthusiasm.Halting or reversing the progression of the disease
ALS is a debilitating neurodegenerative condition that progressively impairs nerve cells responsible for muscle control, leading to muscle wastage, paralysis, and ultimately, death. The average life expectancy of an ALS patient post-diagnosis is a mere two to five years (although, unusually, Hawking – diagnosed at 21 – managed to survive with severe disability until the age of 76).human health and healthcare in Canada. Their total investment in it brings the neurodegenerative disease research at Western to $18 million.
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