Affiliations: Biochimica Clinica, Dipartimento di Medicina Clinica e Biotecnologia Applicata “D. Campanacci”, Università di Bologna, Italy
Note: [] Correspondence to: Prof. Bruno Barbiroli, MD, Biochimica Clinica, Dipartimento di Medicina Clinica e Biotecnologia Applicata “D. Campanacci”, Via Massarenti 9, 40138 Bologna, Italy. Tel.: +39 051 305 993; Fax: +39 051 303 962; E‐mail: barbirol@med.unibo.it.
Abstract: We used in vivo phosphorus magnetic resonance spectroscopy (^{31}P‐MRS) to study the effect of CoQ_{10} on the efficiency of brain and skeletal muscle mitochondrial respiration in ten patients with mitochondrial cytopathies. Before CoQ, brain [PCr] was remarkably lower in patients than in controls, while [Pi] and [ADP] were higher. Brain cytosolic free [Mg^{2+}] and \Delta G of ATP hydrolysis were also abnormal in all patients. MRS also revealed abnormal mitochondrial function in the skeletal muscles of all patients, as shown by a decreased rate of PCr recovery from exercise. After six‐months of treatment with CoQ (150 mg/day), all brain MRS‐measurable variables as well as the rate of muscle mitochondrial respiration were remarkably improved in all patients. These in vivo findings show that treatment with CoQ in patients with mitochondrial cytopathies improves mitochondrial respiration in both brain and skeletal muscles, and are consistent with Lenaz’s view that increased CoQ concentration in the mitochondrial membrane increases the efficiency of oxidative phosphorylation independently of enzyme deficit.
Keywords: Mitochondrial cytopathies, CoQ treatment, brain bioenergetics, muscle energy metabolism, in vivo MR spectroscopy
Journal: Biofactors, vol. 9, no. 2-4, pp. 253-260, 1999
