Coenzyme Q ₁₀ with multiple vitamins is generally ineffective in treatment of mitochondrial disease

Abstract

We followed 16 patients with a variety of mitochondrial diseases over one to four periods of treatment (2 months each) with coenzyme Q10 plus vitamins K3 and C, riboflavin, thiamine, and niacin, using independent measures of oxidative metabolism to assess efficacy. There were large (> threefold) increases in serum coenzyme Q10 concentrations with treatment, but no measure of oxidative metabolism showed significant improvement with treatment for the group, nor did any individual patient show significant, reproducible, objective clinical improvement. The results suggest that coenzyme Q10 plus vitamin therapy does not significantly improve mitochondrial oxidative metabolism in patients with mitochondrial disease in general. Any clinical benefit that may follow from short-term administration appears slight.

Mitochondrial encephalomyopathies: what next?

“…The rationale is obvious and the results have been clearly beneficial in the few reported cases of hereditary CoQI0 deficiency, characterized by the association of central nervous system dysfunction (seizures or mental retardation) and mitochondrial myopathy with recurrent myoglobinuria (Ogasahara et al 1989;Hirano et al 1996;Servidei et al 1996). The efficacy has been more controversial in patients with mitochondrial encephalomyopathies (Ogasahara et al 1986;Bresolin et at 1988;Bendahan et al 1992;Matthews et al 1993), but the frequent reports of subjective or objective beneficial results and the virtual lack of deleterious side-effects have encouraged clinicians to use CoQ10 at least as a palliative therapy. This positive attitude is strengthened by experimental evidence that CoQ10 and nicotinamide prevent striatal lesions induced in rats by the administration of malonate (Beal et al 1994).…”

Lipid‐lowering drugs and mitochondrial function: effects of HMG‐CoA reductase inhibitors on serum ubiquinone and blood lactate/pyruvate ratio

“…Moreover, most studies an increased blood lactate/pyruvate ratio and had lower ubiquinone serum levels than untreated patients. No evaluating the effect of oral supplementation by ubiquinone in patients with mitochondrial cytopathies have correlation could be established between blood lactate/ pyruvate ratio and ubiquinone serum concentration at yielded negative results [35 ]. It is still unknown whether supplementation with oral doses of ubiquinone in statin the individual level.…”

Neurodegenerative and Fatiguing Illnesses, Infections and Mitochondrial Dysfunction: Use of Natural Supplements to Improve Mitochondrial Function.

“…In patients with early Huntington's disease CoQ10 administration for 30 months slowed the usual decline in total functional capacity, but these differences did not reach statistical significance [275]. In contrast, in a multi-center placebo-controlled phase II trial with amyotrophic lateral sclerosis patients CoQ10 did not significantly modify functional decline over a nine-month period [276], and in genetic-based mitochondrial diseases CoQ10 plus several vitamins was shown to be ineffective [277].…”

“…300-302]. However, their sole use in the treatment of mitochondrial diseases has proved disappointing [277]. But it is reassuring to find that many commercial mitochondrial supplements contain adequate amounts of these important molecules (for example [282,292,295,302]).…”