Sodium pyruvate for the treatment of disorders of oxidative phosphorylation

Posted by & filed under Part 10: DISORDERS OF MITOCHONDRIAL FUNCTION.

Fujii T, Nozaki F, Saito K, Hayashi A, Nishigaki Y, Murayama K, Tanaka M, Koga Y, Hiejima I, Kumada T. Efficacy of pyruvate therapy in patients with mitochondrial disease: a semi-quantitative clinical evaluation study. Mol Genet Metab. 2014 Jun;112(2):133-8. 

Tanaka M, Nishigaki Y, Fuku N, Ibi T, Sahashi K, Koga Y. Therapeutic potential of pyruvate therapy for mitochondrial diseases. Mitochondrion2007 Dec;7(6):399-401.

 

In 2007, Tanaka et al formulated the elegant and exciting theory that pyruvate may be helpful in mitochondrial diseases with an elevated lactate/pyruvate ratio by regenerating NAD+ and thus promoting energy production through glycolysis, by activating pyruvate dehydrogenase and by non-enzymatically removing hydrogen peroxide. In Fujii et al’s recent study, four severely affected patients with disorders of oxidative phosphorylation were treated with sodium pyruvate; three showed some improvement, and the effect was maintained for two years in one. The authors found that blood lactate and lactate/pyruvate ratios were not informative for the purpose of monitoring therapy.

The use of sodium pyruvate as a potential treatment for disorders of oxidative phosphorylation certainly merits further study, perhaps in a placebo-controlled trial with less severely affected patients, in whom treatments effect might be more apparent.

 

Posted by Alina Levtova, MD

 

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