Genetic ablation of SOD2, which encodes a mitochondrial manganese SOD (MnSOD), leads to early postnatal death in mice accompanied by a dilated cardiomyopathy, metabolic acidosis, accumulation of lipid in liver and skeletal muscle, increased oxidative damage, and enzymatic abnormalities in mitochondria [216, 217]. The gene discussed is SOD2; the disease is dilated cardiomyopathy.