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Coenzyme Q10 improves
mitochondrial respiration in patients with mitochondrial cytopathies.
An in vivo study on brain and skeletal muscle by phosphorous
magnetic resonance spectroscopy.
Barbiroli B,
Frassineti C,
Martinelli P,
Iotti S,
Lodi R, Cortelli P,
Montagna P.
Dipartimento di Medicina Clinica e
Biotecnologia applicata D. Campanacci, Bologna, Italy.
With phosphorus magnetic resonance
spectroscopy (31P-MRS) we studied in vivo the effect of six-month
coenzyme Q10 treatment on the efficiency of brain and skeletal
muscle mitochondrial respiration in six patients 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 relative rate of ATP
synthesis were high (as an average, 57% and 16% above control group
respectively), whereas the cytosolic phosphorylation potential was
low (as an average, 60% of control value). 31P-MRS also revealed 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 CoQ10/day all brain variables were
remarkably improved in all patients, returning within the control
range in all cases. Treatment with CoQ also improved the muscle
mitochondrial functionality enough to reduce the average deficit to
56% of the control group. These in vivo findings show the beneficial
effect of CoQ in patients with mitochondrial cytopathies, and are
consistent with the view that increased CoQ concentration in the
mitochondrial membrane increases the efficiency of oxidative
phosphorylation independently of enzyme deficit.
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