Worm Study Suggests Innate Immune Response to Mutant ALS Proteins Drives Neurodegeneration in ALS; Blocking of Response May Be Treatment Possibility

A previously unknown link between the immune system and the death of motor neurons in amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, has been discovered by scientists at the CHUM Research Centre and the University of Montreal. The finding paves the way to a whole new approach for finding a drug that can cure, or at least slow the progression, of such neurodegenerative diseases as ALS, Alzheimer's, Parkinson's, and Huntington's diseases. The study, published online on June 10, 2015 in Nature Communications, shows that the immune system in the animal model C. elegans, a tiny 1 mm-long roundworm, plays a critical role in the development of this animal model ALS. “An imbalance of the immune system can contribute to the destruction of motor neurons and trigger the disease,” said Dr. Alex Parker, CRCHUM researcher and Associate Professor in the Department of Neuroscience at the University of Montreal. The article is titled “Neurodegeneration in C. elegans Models of ALS Requires TIR-1/Sarm1 Immune Pathway Activation in Neurons.” ALS is a neuromuscular disease that attacks neurons and the spinal cord. Those affected gradually become paralyzed and typically die within five years of the onset of symptoms. No effective remedy currently exists for this devastating affliction. Riluzole, the only approved medication, only extends the patient's life by a few months. More than a dozen genes are related to ALS. If a mutation occurs in one of them, the person develops the disease. Scientists introduced a mutated human ALS gene (TDP-43 or FUS) into C. elegans, a nematode worm widely used for genetic experiments. The worms became paralyzed within approximately 10 days. The challenge was to find a way of saving them from certain death.
Login Or Register To Read Full Story