Coenzyme Q10 reverses pathological phenotype and reduces apoptosis in familial CoQ10 deficiency

Neurology. 2001 Aug 14;57(3):515-8. doi: 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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Coenzymes
  • Humans
  • Immunohistochemistry
  • Male
  • Microscopy, Electron
  • Muscles / pathology*
  • Muscles / ultrastructure
  • Muscular Diseases / drug therapy*
  • Muscular Diseases / pathology*
  • Phenotype
  • Ubiquinone / analogs & derivatives*
  • Ubiquinone / deficiency*
  • Ubiquinone / therapeutic use*

Substances

  • Coenzymes
  • Ubiquinone
  • coenzyme Q10