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.
Cell Mol Biol (Noisy-le-grand). 1997 Jul;43(5):741-9.
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.
我们采用磷磁共振波谱法（31P-MRS）对6例患有不同线粒体细胞病的患者进行了为期6个月的辅酶Q10治疗，以研究其对脑和骨骼肌线粒体呼吸效率的体内影响。在使用辅酶Q之前，我们发现所有患者枕叶中的磷酸肌酸含量较低（平均比对照组降低25%）。计算得出的[ADP]和ATP合成相对速率较高（平均分别比对照组高57%和16%），而胞质磷酸化电位较低（平均为对照组值的60%）。31P-MRS还显示，与对照组相比，患者骨骼肌中的线粒体功能平均降低了29%。在每天服用150毫克辅酶Q10进行6个月治疗后，所有患者的所有脑部指标均有显著改善，所有病例均恢复到正常范围内。辅酶Q治疗也使肌肉线粒体功能得到了足够改善，使平均缺陷降至对照组的56%。这些体内研究结果表明辅酶Q对线粒体细胞病患者具有有益作用，并且与线粒体膜中辅酶Q浓度增加可独立于酶缺陷提高氧化磷酸化效率的观点一致。
