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Coenzyme Q 10 in the Treatment of Mitochondrial Disease

机译:辅酶Q 10在线粒体疾病的治疗中

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Currently, there is a paucity of available treatment strategies for oxidative phosphorylation disorders. Coenzyme Q 10 (CoQ 10 ) and related synthetic quinones are the only agents to date that have proven to be beneficial in the treatment of these heterogeneous disorders. The therapeutic efficacy of CoQ 10 is not restricted to patients with an underlying CoQ 10 deficiency and is thought to result from its ability to restore electron flow in the mitochondrial respiratory chain (MRC) as well as to increase the cellular antioxidant capacity. At present, however, there is no consensus on the appropriate dosage or therapeutic plasma level of CoQ 10 , and this information will be required before CoQ 10 can be utilized effectively in the treatment of mitochondrial disease. The following review will outline our current knowledge on the use of CoQ 10 in the treatment of MRC disorders and primary CoQ 10 deficiencies.
机译:目前,缺乏用于氧化磷酸化疾病的可用治疗策略。迄今为止,辅酶Q 10(CoQ 10)和相关的合成醌是唯一被证明对这些异质性疾病有益的药物。辅酶Q 10的治疗功效不仅限于具有潜在辅酶Q 10缺乏症的患者,而且被认为是由于其恢复线粒体呼吸链(MRC)中电子流动以及增加细胞抗氧化能力的能力所致。但是,目前尚无关于CoQ 10的适当剂量或治疗性血浆水平的共识,并且在CoQ 10可以有效地用于线粒体疾病的治疗之前,需要此信息。以下评论将概述我们目前在使用辅酶Q 10治疗MRC疾病和原发性辅酶Q 10缺陷方面的知识。

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