Coenzyme Q 10 reverses pathological phenotype and reduces apoptosis in familial CoQ 10 deficiency
- 14 August 2001
- journal article
- Published by Wolters Kluwer Health in Neurology
- Vol. 57 (3) , 515-518
- https://doi.org/10.1212/wnl.57.3.515
Abstract
Two brothers with myopathic coenzyme Q10 (CoQ10) deficiency responded dramatically to CoQ10 supplementation. Muscle biopsies before therapy showed ragged-red fibers, lipid storage, and complex I + III and II + III deficiency. Approximately 30% of myofibers had multiple features of apoptosis. After 8 months of treatment, excessive lipid storage resolved, CoQ10 level normalized, mitochondrial enzymes increased, and proportion of fibers with TUNEL-positive nuclei decreased to 10%. The authors conclude that muscle CoQ10 deficiency can be corrected by supplementation of CoQ10, which appears to stimulate mitochondrial proliferation and to prevent apoptosis.Keywords
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