Dimethyl Fumarate Ameliorates the Amyotrophic Lateral Sclerosis Symptoms in Tg-SOD1^G93A Mice

Amyotrophic lateral sclerosis (ALS) is a progressive fatal neurodegenerative disorder that affects motor neurons and has limited therapeutic approaches. Dimethyl fumarate (DMF) is a drug approved for the treatment of psoriasis and relapsing-remitting multiple sclerosis (RRMS). Here, we found that DMF improved the neuromuscular strength of Tg-SOD1^G93A mice, a well-established ALS model, without however extending the life expectancy. Also, administration of DMF increased the expression of Nqo1, indicating in vivo activation of the Nrf2 pathway, decreased the motor neuron degeneration, and suppressed the number of vacuolated neurons present in the lumbar spinal cord of Tg-SOD1^G93A mice. Thus, our study supports the further clinical evaluation of DMF for the treatment of ALS.