Oxidative stress mediated by elevated ROS has been shown to be involved in the pathological mechanisms of various diseases, such as Barth syndrome [42], neurodegenerative diseases [43], atherosclerosis [44], and Duchenne cardiomyopathy [45] etc. Inhibition of ROS by administration of antioxidant drugs, such as GSH, glutathione peroxidase (GPX) mimicsa and superoxide dismutase (SOD) mimics, has been shown to slow disease progression and improve disease phenotype [42, 45, 46]. The gene discussed is SOD1; the disease is Barth syndrome.