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...

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Veröffentlicht in:	Brain & development (Tokyo. 1979) 1998, Vol.20 (1), p.33-35
Hauptverfasser:	Costeff, Hanan, Apter, Naomi, Elpeleg, Orly N, Prialnic, Michael, Böhles, Hans Josef
Format:	Artikel
Sprache:	eng
Schlagworte:	3-Methylglutaconic aciduria type 3 Administration, Oral Adolescent Adult Biological and medical sciences Child Coenzyme Q10 Coenzymes Cross-Over Studies Errors of metabolism Female Glutarates - urine Humans Iraqi Jewish optic atrophy syndrome Lipids (lysosomal enzyme disorders, storage diseases) Male Medical sciences Metabolic diseases Movement Disorders - complications Neuromuscular Diseases - complications Optic Atrophy - complications Optic atrophy plus Single-Blind Method Treatment Failure Tropical medicine Ubiquinone - analogs & derivatives Ubiquinone - blood Ubiquinone - therapeutic use Visual Acuity - drug effects
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Zusammenfassung:	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.
ISSN:	0387-7604 1872-7131
DOI:	10.1016/S0387-7604(97)00096-X