Accumulation of lipid peroxidation products in human myotonic dystrophic muscle.

The possibility of oxygen radical-induced injury contributing to the pathogenesis of muscle disorders was studied. Significant increases in fluorescent lipid peroxidation products was found in the muscle samples of myotonic dystrophy (MyD) patients as compared to controls as well as patients with Duchenne Muscular dystrophy (DMD), Amyotrophic Lateral Sclerosis (ALS), Polymyositis and Limb Girdle Dystrophy (LGD). The results demonstrate the possibility that in MyD the primary genetic disorder leads to the rapid generation of oxygen radicals, tissue depletion of antioxidants followed by peroxidation of membrane lipids, impaired calcium homeostasis and finally atrophy of the muscle.