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OXPHOS complex IV activity is reduced in patient mitochondria. (A–E) Activities of the five OXPHOS complexes (CI–CV), and (F) citrate synthase activity. All measurements were performed in isolated mitochondria from patient-derived fibroblasts. Thick, dotted lines represent the reference range from our center; thin, dotted lines represent the means of control individuals. CI to CV, OXPHOS complexes I to V; CS, citrate synthase activity.
