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Updated: Aug 11, 2026

Assessment of Open Probability of the Mitochondrial Permeability Transition Pore in the Setting of Coenzyme Q Excess
Published on: June 1, 2022
Ineffectiveness of oral coenzyme Q10 supplementation in 3-methylglutaconic aciduria, type 3
H Costeff1, N Apter, O N Elpeleg
1Loewenstein Hospital, Raanana, Israel.
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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