title: Ineffectiveness of oral coenzyme Q10 supplementation in 3-methylglutaconic aciduria, type 3.
abstract: Coenzyme Q10 was administered under placebo controlled blinded crossover conditions to six subjects suffering from type 3 3-methylglutaconic aciduria ('optic atrophy plus'), following a report of benefit. Despite attainment of high plasma levels of coenzyme Q10, no clinical benefit was observed and there was no diminution of urinary excretion of 3-methylglutaconic acid.
authors.affiliation: Loewenstein Hospital, Raanana, Israel.
authors.forename: H
authors.initials: H
authors.lastname: Costeff
authors.forename: N
authors.initials: N
authors.lastname: Apter
authors.forename: O N
authors.initials: ON
authors.lastname: Elpeleg
authors.forename: M
authors.initials: M
authors.lastname: Prialnic
authors.forename: H J
authors.initials: HJ
authors.lastname: Böhles
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D002648:Child; D003067:Coenzymes; D018592:Cross-Over Studies; D005260:Female; D005977:Glutarates / Q000652:urine*; D006801:Humans; D008297:Male; D009069:Movement Disorders / Q000150:complications; D009468:Neuromuscular Diseases / Q000150:complications; D009896:Optic Atrophy / Q000150:complications; D016037:Single-Blind Method; D017211:Treatment Failure; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000097:blood / Q000627:therapeutic use; D014792:Visual Acuity / Q000187:drug effects
chemical_list: D003067:Coenzymes; D005977:Glutarates; D014451:Ubiquinone; C022034:3-methylglutaconic acid; C024989:coenzyme Q10