The Effects of Red Dragon Fruit Betacyanin Extract on the Motor Function and Health of Neuromuscular Junctions in the G93A Mutant hSOD1 Mouse Model of ALS

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by oxidative stress, neuroinflammation, and axonopathy leading to the loss of upper and lower muscular neurons. The G93A hSOD1 ALS mouse model, used in this study, has a point mutation in the SOD1 gene causing an overexpression of superoxide dismutase. This animal model experiences skeletal muscular atrophy, muscle weakness and weight loss beginning at the onset of the disease (~90 days old) until they reach end stage (~120 days old). This is due in part to the deterioration of neuromuscular junctions, which are synaptic connections between the terminal end of the motor nerve and the muscle. Betacyanin, a nutraceutical compound found in red dragon fruit (DFE), has been shown to have powerful antioxidant and anti-inflammatory properties, making it a potential therapeutic treatment for ALS. This study tested the effect of betacyanin on motor function and the neuromuscular junctions in the gastrocnemii muscles of the G93A mutant hSOD1 ALS mouse model. In this study, G93A hSOD1 mutant mice were treated orally with 5% (v/v) DFE in drinking water ad libitum from disease onset until end-stage. In each group, (consisting of three littermates: one wild type, one untreated mutant and one treated mutant), the progression of the disease was monitored by measuring the body weight and conducting grip strength and rotarod tests. Then histopathological analyses were performed to determine the effect of DFE on the neuromuscular junctions in the gastrocnemii muscles. Overall, the treated mutants have shown reduced deterioration of neuromuscular junctions in the gastrocnemius muscle, delayed wet weight loss of the gastrocnemius muscle and improved motor function compared to the untreated control ALS littermate. These findings indicate that DFE slowed the progression of ALS and shows potential as a therapeutic treatment for patients with ALS.