Ineffectiveness of oral coenzyme Q10 supplementation in 3-methylglutaconic aciduria, type 3

Brain Dev. 1998 Jan;20(1):33-5. doi: 10.1016/s0387-7604(97)00096-x.

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.

Publication types

  • Clinical Trial

MeSH terms

  • Administration, Oral
  • Adolescent
  • Adult
  • Child
  • Coenzymes
  • Cross-Over Studies
  • Female
  • Glutarates / urine*
  • Humans
  • Male
  • Movement Disorders / complications
  • Neuromuscular Diseases / complications
  • Optic Atrophy / complications
  • Single-Blind Method
  • Treatment Failure
  • Ubiquinone / analogs & derivatives*
  • Ubiquinone / blood
  • Ubiquinone / therapeutic use
  • Visual Acuity / drug effects

Substances

  • Coenzymes
  • Glutarates
  • Ubiquinone
  • 3-methylglutaconic acid
  • coenzyme Q10