Ineffectiveness of oral coenzyme Q10 supplementation in 3-methylglutaconic aciduria, type 3.
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.
Costeff
H
H
Loewenstein Hospital, Raanana, Israel.
Apter
N
N
Elpeleg
O N
ON
Prialnic
M
M
Böhles
H J
HJ
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
D003067:Coenzymes; D005977:Glutarates; D014451:Ubiquinone; C022034:3-methylglutaconic acid; C024989:coenzyme Q10