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Enzymatic activities in muscle mitochondria and fibroblast homogenates. Respiratory chain complexes I-V (C I-IV), citrate synthase (CS) and aconitase (ACO) from control (C) and patient (P) were measured in isolated muscle mitochondria (a), whereas respiratory chain complexes II, II+III and citrate synthase were measured in fibroblast homogenate (b), by spectrophotometric methods. The graphs depict mean±SEM of controls (n>5).
