COENZYME Q(10) IMPROVES MITOCHONDRIAL RESPIRATION IN PATIENTS WITH MITOCHONDRIAL CYTOPATHIES - AN IN-VIVO STUDY ON BRAIN AND SKELETAL-MUSCLE BY PHOSPHORUS MAGNETIC-RESONANCE SPECTROSCOPY

With phosphorus magnetic resonance spectroscopy (P-31-MRS) we studied in vivo the effect of six-month coenzyme Q(10) treatment on the effici ency of brain and skeletal muscle mitochondrial respiration in six pat ients with different mitochondrial cytopathies. Before CoQ we found a low phosphocreatine content (average of 25% decrease from controls) in the occipital lobes of all patients. Calculated [ADP] and the relativ e rate of ATP synthesis were high (as an average, 57% and 16% above co ntrol group respectively), whereas the cytosolic phosphorylation poten tial was low (as an average, 60% of control value). P-31-MRS also reve aled an average of 29% reduction of the mitochondrial function in the skeletal muscle of patients compared with controls. After a six-month treatment with 150 mg CoQ(10)/day all brain variables were remarkably improved in all patients, returning within the control range in all ca ses. Treatment with CoQ also improved the muscle mitochondrial functio nality enough to reduce the average deficit to 56% of the control grou p. These in vivo findings show the beneficial effect of CoQ in patient s with mitochondrial cytopathies, and are consistent with the view tha t increased CoQ concentration in the mitochondrial membrane increases the efficiency of oxidative phosphorylation independently of enzyme de ficit.