Coenzyme Q(10) reverses pathological phenotype and reduces apoptosis in familial CoQ(10) deficiency

Two brothers with myopathic coenzyme Q(10) (CoQ(10)) deficiency responded d ramatically to CoQ(10) supplementation. Muscle biopsies before therapy show ed ragged-red fibers, lipid storage, and complex I + III and II + III defic iency. Approximately 30% of myofibers had multiple features of apoptosis. A fter 8 months of treatment, excessive lipid storage resolved, CoQ(10) level normalized, mitochondrial enzymes increased, and proportion of fibers with TUNEL-positive nuclei decreased to 10%. The authors conclude that muscle C oQ(10) deficiency can be corrected by supplementation of CoQ(10) which appe ars to stimulate mitochondrial proliferation and to prevent apoptosis.