Percentage of impaired biomarkers (A), muscular involvement (B) and positive histopathological features (C) in nDNA (black bars) and mtDNA (grey bars) patients. Whiskers indicate significant differences (chi-square test) between nDNA and mtDNA groups. nDNA patients showed significantly higher frequency of SDH reduction and SDH-positive vessels, while mtDNA patients shower a higher frequency of RRF and blue fibers, and near significative for ophthalmoplegia and exercise intolerance. nDNA, nuclear DNA; mtDNA, mitochondrial DNA; CPK, creatine phosphate kinase; EMG, myopathic electromyography; RRF, ragged red; SDH, succinate dehydrogenase; COX, cytochrome c oxidase.