Israeli Scientists Highlights Gene That Could Lead To New Therapies For ALSSep. 15, 2016
Researchers from Ben-Gurion University of the Negev (BGU) have published a new study that describes a novel molecular mechanism that could lead to the development of new therapies for Amyotrophic Lateral Sclerosis (ALS).
The study was published online in the prestigious PNAS (Proceedings of the National Academy of Sciences of the United States of America).
ALS, also known as Lou Gehrig’s disease, is a fatal neurodegenerative disease that causes death of motor neurons, which control voluntary muscles. Progressive weakness and paralysis due to muscle atrophy lead to difficulty in speaking, swallowing and eventually breathing. The disease typically starts between ages 40 and 60, and the average survival from onset to death is two to five years.
The cause is not known in about 90 percent of cases, but approximately 10 percent are genetically inherited. Approximately 20 percent of these genetic cases are caused by mutations in the SOD1 gene (superoxide dismutase), which lead to the accumulation of “misfolded” SOD1 proteins that provoke selective killing of motor neurons.