Coenzyme Q10 reverses pathological phenotype and reduces apoptosis in familial CoQ10 deficiency
S Di Giovanni, M Mirabella, A Spinazzola, P Crociani… - Neurology, 2001 - AAN Enterprises
Neurology, 2001•AAN Enterprises
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 …
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 …
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.
American Academy of Neurology