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Proceedings of the National Academy of Sciences of the United States of America logoLink to Proceedings of the National Academy of Sciences of the United States of America
. 1974 May;71(5):2098–2102. doi: 10.1073/pnas.71.5.2098

Effect of Coenzyme Q on Serum Levels of Creatine Phosphokinase in Preclinical Muscular Dystrophy*,

Karl Folkers , Ryo Nakamura , Gian Paolo Littarru , Hans Zellweger §, John B Brunkhorst , Coyle W Williams Jr ǁ, John H Langston ǁ
PMCID: PMC388394  PMID: 4525474

Abstract

Coenzyme Q10 (CoQ10) exists in human tissue, and is indispensable to mitochondrial enzymes of respiration. CoQ was administered to children with preclinical muscular dystrophy, CoQ enzymology was emphasized, and serum creatine phosphokinase, CPK, (ATP:creatine N-phosphotransferase, EC 2.7.3.2) was repeatedly monitored.

A 40-week treatment of an infant, 1-2 years of age, reduced serum CPK (P < 0.001; total CPK assays, 76). A 40-week treatment of a boy, 3-5 years of age, reduced serum CPK (P < 0.01); treatment through 80 weeks reduced CPK (P < 0.001; total CPK assays, 118).

This response of preclinical dystrophy to CoQ implies a deficiency of CoQ in skeletal muscle that was actually found previously by assay of the activity of the succinate dehydrogenase:coenzyme Q10 reductase of the rectus abdominis. The relationships among a CoQ deficiency in muscle, serum CPK, and use of CPK in muscle are uncertain; however, restoration of CoQ enzyme activity in muscle by oral administration of CoQ could lead to increased use of CPK in muscle to form phosphocreatine from creatine and ATP, with a corresponding decrease in serum levels of CPK. The great excess of CPK in serum comes from deteriorating muscle in which CPK is below normal.

Keywords: treatment, bioenergetics

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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