Abstract
Gene mutations of Cu/Zn superoxide dismutase (SOD) have been discovered in familial amyotrophic lateral sclerosis (ALS) . Oxidative stress also plays a role in the pathogenesis of sporadic ALS. Whether antioxidant therapy is beneficial in this fatal disease is now crucial. We have shown that phosphatidyl choline-bound Cu/Zn SOD (PC-SOD) treatment improves neuromuscular dysfunction and morphological changes in wobbler mouse motor neuron disease. Progressive spinal motor neuronopathy and axonopathy, predominantly in the cervical cord, occur at postnatal age 3-4 weeks, leading to muscle weakness and contracture of the forelimbs in this animal. These motor deficits rapidly increase by postnatal age 6-8 weeks, and then slowly progress.Wobbler mice were given two doses daily of PC-SOD, unmodified SOD (104, 105 U/kg, respectively) or a vehicle solution by intraperitoneal injection from postnatal 3-4 to postnatal 7-8 weeks of age. PC-SOD, but not unmodified SOD treatment attenuated progression of motor dysfunction, prevented denervation muscle atrophy, and delayed degeneration of spinal motor neurons in wobbler mice. Intraperitoneally administered PC-SOD was significantly transferred into the spinal cord, as compared with unmodified SOD.This raises the possibility that PC-SOD may have therapeutic potential in human motor neuron disease or ALS.
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