Fig. 1.

Mitochondrial function in myoclonic epilepsy with ragged-red fibers (MERRF) fibroblasts. (A) Coenzyme Q₁₀ (CoQ) levels in control and MERRF fibroblasts. (B) Complex I + III, activity measured in cells grown in the presence or absence of CoQ (100 μM) for 72 h. Results (mean ± SD) are expressed in units per citrate synthase (U/CS). (C) Adenosine-5'-triphosphate (ATP) levels in control and MERRF fibroblasts grown in the absence or presence of CoQ (100 μM) for 72 h. (D) Cell proliferation of control and MERRF fibroblasts cultured in the absence or presence of CoQ (100 μM) for 72 h. Data represent the mean ± SD of 3 separate experiments. *p < 0.01 between control and MERRF fibroblasts. ^ap < 0.05 between the presence and the absence of CoQ