In a study, in which fibroblast cells, derived from six unrelated RR-MADD patients, were cultured and compared to that of control fibroblasts, RR-MADD cells showed decreases in the protein levels of the antioxidant enzyme MnSOD, the protein quality control enzyme Lon, and the p62 protein — which is needed for recognition and selection of oxidative damage mitochondria for autophagy degradation. The gene discussed is SOD2; the disease is multiple acyl-CoA dehydrogenase deficiency.