H. Costeff et al., INEFFECTIVENESS OF ORAL COENZYME-Q10 SUPPLEMENTATION IN 3-METHYLGLUTACONIC ACIDURIA, TYPE-3, Brain & development, 20(1), 1998, pp. 33-35
Coenzyme Q10 was administered under placebo controlled blinded crossov
er conditions to six subjects suffering from type 3 3-methylglutaconic
aciduria ('optic atrophy plus'), following a report of benefit. Despi
te attainment of high plasma levels of coenzyme Q10, no clinical benef
it was observed and there was no diminution of urinary excretion of 3-
methylglutaconic acid. (C) 1998 Elsevier Science B.V.