Hargreaves I P
Neurometabolic Unit, National Hosptal, London, UK.
Int J Biochem Cell Biol. 2014 Apr;49:105-11. doi: 10.1016/j.biocel.2014.01.020. Epub 2014 Feb 2.
Treatment of mitochondrial respiratory chain (MRC) disorders is extremely difficult, however, coenzyme Q10 (CoQ10) and its synthetic analogues are the only agents which have shown some therapeutic benefit to patients. CoQ10 serves as an electron carrier in the MRC as well as functioning as a potent lipid soluble antioxidant. CoQ10 supplementation is fundamental to the treatment of patients with primary defects in the CoQ10 biosynthetic pathway. The efficacy of CoQ10 and its analogues in the treatment of patients with MRC disorders not associated with a CoQ10 deficiency indicates their ability to restore electron flow in the MRC and/or increase mitochondrial antioxidant capacity may also be important contributory factors to their therapeutic potential.
线粒体呼吸链（MRC）疾病的治疗极其困难，然而，辅酶Q10（CoQ10）及其合成类似物是仅有的对患者显示出一定治疗益处的药物。CoQ10在MRC中作为电子载体，同时还作为一种强效的脂溶性抗氧化剂发挥作用。补充CoQ10是治疗CoQ10生物合成途径原发性缺陷患者的基础。CoQ10及其类似物在治疗与CoQ10缺乏无关的MRC疾病患者中的疗效表明，它们恢复MRC中电子流动和/或增加线粒体抗氧化能力的能力可能也是其治疗潜力的重要促成因素。
