Literature DB >> 9533558

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

H Costeff1, N Apter, O N Elpeleg, M Prialnic, H J Böhles.   

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.

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Year:  1998        PMID: 9533558     DOI: 10.1016/s0387-7604(97)00096-x

Source DB:  PubMed          Journal:  Brain Dev        ISSN: 0387-7604            Impact factor:   1.961


  2 in total

1.  Type III 3-methylglutaconic aciduria (optic atrophy plus syndrome, or Costeff optic atrophy syndrome): identification of the OPA3 gene and its founder mutation in Iraqi Jews.

Authors:  Y Anikster; R Kleta; A Shaag; W A Gahl; O Elpeleg
Journal:  Am J Hum Genet       Date:  2001-10-19       Impact factor: 11.025

2.  Costeff syndrome: clinical features and natural history.

Authors:  Gilad Yahalom; Yair Anikster; Ruth Huna-Baron; Chen Hoffmann; Lubov Blumkin; Dorit Lev; Rakefet Tsabari; Zeev Nitsan; Sheera F Lerman; Bruria Ben-Zeev; Ben Pode-Shakked; Shira Sofer; Avraham Schweiger; Tally Lerman-Sagie; Sharon Hassin-Baer
Journal:  J Neurol       Date:  2014-09-09       Impact factor: 4.849

  2 in total

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