Long‐term coenzyme Q ₁₀ therapy for a mitochondrial encephalomyopathy with cytochrome c oxidase deficiency

Abstract

For 2 years we administered high doses of coenzyme Q10 (CoQ) to a patient having mitochondrial encephalomyopathy with cytochrome c oxidase deficiency. Abnormal elevation of the serum lactate per pyruvate ratio and the increased concentration of serum lactate plus pyruvate induced by exercise decreased with CoQ treatment. This therapeutic effect continued for 2 years. 31P nuclear magnetic resonance spectroscopy showed acceleration of the postexercise recovery of the ratio of phosphocreatine to inorganic phosphate in muscle during CoQ treatment. These observations support the beneficial effect of CoQ on the impaired mitochondrial oxidative metabolism in muscle. Also, impaired central and peripheral nerve conductivities consistently improved during CoQ treatment. These results indicate that CoQ has clinical value in the long-term management of patients with mitochondrial encephalomyopathies, even though there are clinical limitations to the effects of this therapy.

The effects of coenzyme Q 10 treatment on maternally inherited diabetes mellitus and deafness, and mitochondrial DNA 3243 (A to G) mutation

“…Apart from the improvement of insulin secretory defects, there were no other significant differences in insulin requirement, metabolic control or other clinical parameters between the CoQ 10 -DM group and the control-DM group. In addition, there were no detectable side effects from CoQ 10 .…”

“…Silvestre-Aillaud et al treated a MIDD patient with a combination of CoQ 10 and L-carnitine for 6 months, but could not confirm a significant effect either on insulin secretion or on insulin sensitivity [21]. Some reports described CoQ 10 as deficient in some patients with mitochondrial encephalomyopathy and that its replacement improved the clinical symptoms [22]. However, there was no CoQ 10 deficiency in the MIDD patient.…”

Coenzyme Q 10 administration increases brain mitochondrial concentrations and exerts neuroprotective effects

“…Coenzyme Q, which also is known as ubiquinone, is a lipid-soluble compound composed of a redox active quinoid moiety and a hydrophobic ''tail.'' The predominant form of coenzyme Q in humans is coenzyme Q 10 , which contains 10 isoprenoid units in the tail, whereas the predominant form in rodents is coenzyme Q 9 , which has nine isoprenoid units in the tail. Coenzyme Q is soluble and mobile in the hydrophobic core of the phospholipid bilayer of the inner membrane of the mitochondria where it transfers electrons one at a time to complex III of the electron transport chain.…”

“…Several reports found both clinical and biochemical improvement in patients with mitochondrial disorders (6)(7)(8)(9)(10). If defects in energy metabolism and oxidative damage play a role in the pathogenesis of neurodegenerative diseases (11,12), then treatment with coenzyme Q 10 could exert beneficial therapeutic effects.…”

Mitochondrial drug delivery and mitochondrial disease therapy – An approach to liposome-based delivery targeted to mitochondria

“…Although theses strategies resulted in positive therapeutic effects, to some extent, they were not sufficient to permit the loss of function to be completely recovered (Nishikawa et al, 1989, Suzuki et al, 1995, Tanaka et al, 1997.…”