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We found a common coupling defect of oxidative phosphorylation in fibroblasts of patients affected by autosomal dominant optic atrophy (mutations of <jats:italic>OPA1<\/jats:italic>), autosomal dominant optic atrophy associated with cataract (mutations of <jats:italic>OPA3<\/jats:italic>), and Leber's hereditary optic neuropathy, a disorder associated with point mutations of mitochondrial DNA complex I genes. Interestingly, the energetic defect was significantly more pronounced in Leber's hereditary optic neuropathy and autosomal dominant optic atrophy patients with a more complex phenotype, the so\u2010called plus phenotype. 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