COX5A and myopathy: Isolated COX deficiencies can arise from mutations in one of the three mt-DNA encoded subunits genes, or, more rarely, of mutations in one of the ten nuclear-encoded subunits genes, resulting in a variety of mild (myopathies with ragged-red fibers) or severe (encephalopathy, lactic acidosis, and stroke-like episodes) phenotypes [57,63].