Figure 5. Mitochondrial metabolic investigations in the patient and controls. A: Coupling efficiency (ATP/O): Reduced efficiency of ATP synthesis was found in the patient’s fibroblasts in comparison to controls. B: Reduced mitochondrial ATP synthesis, measured by luminescence from aliquot samples in a polarographic chamber, was found in the patient’s fibroblasts in comparison to controls. C: Maximal coupled respiration rates, i.e., ADP-stimulated oxygen consumption with malate, pyruvate, and succinate, was measured in the same polarographic chamber.