Coenzyme Q10 as a therapy for mitochondrial disease.
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.
Neurometabolic Unit, National Hosptal, London, UK. Electronic address: iain.hargreaves@uclh.nhs.uk.
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IP
Hargreaves
D000818:Animals; D001259:Ataxia / Q000188:drug therapy; D006801:Humans; D028361:Mitochondrial Diseases / Q000188:drug therapy*; D015394:Molecular Structure; D018908:Muscle Weakness / Q000188:drug therapy; D016896:Treatment Outcome; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000737:chemistry / Q000172:deficiency / Q000627:therapeutic use
D014451:Ubiquinone; C571746:alpha-tocotrienol quinone; C024989:coenzyme Q10; C036619:idebenone
Coenzyme Q10; EPI-743; Idebenone; Mitochondrial respiratory chain; Oxidative stress