ubiquinone and Muscular-Dystrophies

Topics: Adolescent; Adult; Animals; Avitaminosis; Child; Child, Preschool; Creatine Kinase; Cricetinae; Deficiency Diseases; Electron Transport; Humans; Leukocytes; Male; Mice; Middle Aged; Mitochondria, Muscle; Muscles; Muscular Dystrophies; Muscular Dystrophy, Animal; Oxidoreductases; Rabbits; Succinate Dehydrogenase; Ubiquinone

Topics: Adolescent; Adult; Age Factors; Animals; Child; Child, Preschool; Contracture; Creatine Kinase; Humans; Infant; Infant, Newborn; Muscular Dystrophies; Myotonia; Myotonia Congenita; Myotonic Dystrophy; Ubiquinone

Topics: Animals; Avitaminosis; Benzoates; Binding Sites; Electron Transport; Folic Acid; Haplorhini; Humans; Lipid Metabolism; Metabolism, Inborn Errors; Mevalonic Acid; Mice; Muscular Dystrophies; Oxidoreductases; Peroxides; Rabbits; Rats; Saccharomyces; Semantics; Succinate Dehydrogenase; Ubiquinone; Vitamin E Deficiency; Vitamins

Topics: Antioxidants; Cholesterol; Enzymes; Fatty Acids; Humans; Lipid Metabolism; Muscular Dystrophies; Ubiquinone; Vitamin E; Vitamin E Deficiency

Coenzyme Q10 (vitamin Q10) is biosynthesized in the human body and is functional in bioenergetics, anti-oxidation reactions, and in growth control, etc. It is indispensable to health and survival. The first double-blind trial was with twelve patients, ranging from 7-69 years of age, having diseases including the Duchenne, Becker, and the limb-girdle dystrophies, myotonic dystrophy. Charcot-Marie-Tooth disease, and the Welander disease. The control coenzyme Q10 (CoQ10) blood level was low and ranged from 0.5-0.84 microgram/ml. They were treated for three months with 100 mg daily of CoQ10 and a matching placebo. The second double-blind trial was similar with fifteen patients having the same categories of disease. Since cardiac disease is established to be associated with these muscle diseases, cardiac function was blindly monitored, and not one mistake was made in assigning CoQ10 and placebo to the patients in both trials. Definitely improved physical performance was recorded. In retrospect, a dosage of 100 mg was too low although effective and safe. Patients suffering from these muscle dystrophies and the like, should be treated with vitamin Q10 indefinitely.

Topics: Adolescent; Adult; Aged; Charcot-Marie-Tooth Disease; Child; Coenzymes; Double-Blind Method; Female; Humans; Male; Middle Aged; Muscular Atrophy; Muscular Dystrophies; Myotonic Dystrophy; Ubiquinone

Cardiac disease is commonly associated with virtually every form of muscular dystrophy and myopathy. A double-blind and open crossover trial on the oral administration of coenzyme Q10 (CoQ10) to 12 patients with progressive muscular dystrophies and neurogenic atrophies was conducted. These diseases included the Duchenne, Becker, and limb-girdle dystrophies, myotonic dystrophy, Charcot-Marie-Tooth disease, and Welander disease. The impaired cardiac function was noninvasively and extensively monitored by impedance cardiography. Solely by significant change or no change in stroke volume and cardiac output, all 8 patients on blind CoQ10 and all 4 on blind placebo were correctly assigned (P less than 0.003). After the limited 3-month trial, improved physical well-being was observed for 4/8 treated patients and for 0/4 placebo patients; of the latter, 3/4 improved on CoQ10; 2/8 patients resigned before crossover; 5/6 on CoQ10 in crossover maintained improved cardiac function; 1/6 crossed over from CoQ10 to placebo relapsed. The rationale of this trial was based on known mitochondrial myopathies, which involve respiratory enzymes, the known presence of CoQ10 in respiration, and prior clinical data on CoQ10 and dystrophy. These results indicate that the impaired myocardial function of such patients with muscular disease may have some association with impaired function of skeletal muscle, both of which may be improved by CoQ10 therapy. The cardiac improvement was definitely positive. The improvement in well-being was subjective, but probably real. Likely, CoQ10 does not alter genetic defects but can benefit the sequelae of mitochondrial impairment from such defects. CoQ10 is the only known substance that offers a safe and improved quality of life for such patients having muscle disease, and it is based on intrinsic bioenergetics.

Topics: Administration, Oral; Cardiac Output; Charcot-Marie-Tooth Disease; Coenzymes; Double-Blind Method; Electrocardiography; Heart; Humans; Muscular Diseases; Muscular Dystrophies; Stroke Volume; Ubiquinone

The muscular dystrophies (MDs) are genetic disorders of muscle degeneration due to mutations in genes that encode a wide variety of proteins. Dysferlinopathy are characterized by the absence of dysferlin in skeletal muscle and an autosomal recessive mode of inheritance. Both histological and ultrastructural pathology have been well established in dysferlinopathy patients and dysferlin-deficient animal models. To our knowledge the effect of antioxidant supplementation on this level has not been described previously. This article therefore focuses on the histopathology to reveal the effect of antioxidant supplementation. The study aimed to determine, at cellular level, the histopathological changes in the SJL/J mouse model following a 90 day trial with antioxidant supplementation. Markedly reduced inflammatory insult in the more affected quadriceps muscles of animals treated with high doses of CoQ10 and a combination of resveratrol/CoQ10 were observed. The outcome provides evidence that high doses of antioxidant supplementation resulted in decreased dystrophic markers and enhanced tissue integrity at cellular level.

Topics: Animals; Antioxidants; Disease Models, Animal; Female; Mice; Muscle, Skeletal; Muscular Dystrophies; Resveratrol; Stilbenes; Ubiquinone

Coenzyme Q(10) (CoQ(10)) 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 Q(10) 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.

Topics: Child, Preschool; Creatine Kinase; Humans; Infant; Male; Muscular Dystrophies; Ubiquinone

Topics: 17-Ketosteroids; Adolescent; Child; Creatine Kinase; Creatinine; Glucose Tolerance Test; Humans; Insulin; Male; Motor Activity; Muscular Diseases; Muscular Dystrophies; Ubiquinone

Topics: Child; Creatine Kinase; Diseases in Twins; Fructose-Bisphosphate Aldolase; Humans; Male; Muscular Dystrophies; Ubiquinone

Topics: Animals; Hindlimb; Mice; Mitochondria, Muscle; Muscular Dystrophies; Muscular Dystrophy, Animal; Myocardium; Ubiquinone

Topics: Animals; Female; Hindlimb; Male; Mice; Muscles; Muscular Dystrophies; Muscular Dystrophy, Animal; Myocardium; Oxidoreductases; Succinate Dehydrogenase; Ubiquinone

Topics: Animals; Chickens; Diet; Muscular Dystrophies; Ubiquinone; Vitamin E

Topics: Animals; Antioxidants; Deficiency Diseases; Disease Models, Animal; Freeze Drying; Kidney; Liver; Muscular Dystrophies; Myocardium; Oxidoreductases; Rabbits; Succinate Dehydrogenase; Ubiquinone; Vitamin E Deficiency

Topics: Aging; Animals; Benzoates; Carbon Isotopes; Kidney; Liver; Mice; Muscular Dystrophies; Myocardium; Ubiquinone

Topics: Animals; Electron Transport; Hindlimb; Metabolism, Inborn Errors; Mice; Movement; Muscular Dystrophies; Ubiquinone

Topics: Adenosine Triphosphate; Amino Acids; Anabolic Agents; Diet Therapy; Digitoxin; Glucocorticoids; Humans; Insulin; Muscles; Muscular Dystrophies; Phosphates; Physical Therapy Modalities; Psychotherapy; Ubiquinone; Vitamin E; Vitamins

Topics: Animals; Chlorides; Mice; Muscles; Muscular Dystrophies; Potassium; Sodium; Ubiquinone

Topics: Animals; Cattle; Kidney; Liver; Lung; Muscles; Muscular Dystrophies; Myocardium; Sclera; Selenium; Ubiquinone

Topics: Anemia; Animals; Antioxidants; Creatine; Creatinine; Diet; Electron Transport; Haplorhini; Hematopoiesis; Hemoglobins; Muscular Dystrophies; Oxidative Phosphorylation; Polycythemia; Ubiquinone; Vitamin E; Vitamin E Deficiency

Topics: Adenosine Triphosphate; Animals; Animals, Newborn; Female; In Vitro Techniques; Mice; Muscular Dystrophies; Pregnancy; Pregnancy, Animal; Reserpine; Ubiquinone

Topics: Animals; Antioxidants; Body Weight; Creatine; Enzyme Precursors; Muscular Dystrophies; Oxidative Phosphorylation; Oxygen Consumption; Rabbits; Ubiquinone; Vitamin E Deficiency

Topics: Animals; Mice; Muscular Dystrophies; Nutrition Disorders; Ubiquinone; Vitamin E Deficiency

Topics: Animals; Creatine; Creatinine; Lagomorpha; Mortality; Muscular Dystrophies; Pharmacology; Quinones; Rabbits; Research; Ubiquinone; Vitamin E Deficiency; Vitamins

Topics: Animals; Haplorhini; Muscular Dystrophies; Reticulocytes; Ubiquinone; Vitamin E

Topics: Guinea Pigs; Lipid Metabolism; Lipid Peroxidation; Liver; Metabolism; Muscles; Muscular Dystrophies; Myocardium; Peroxidases; Research; Ubiquinone; Vitamin E Deficiency

Topics: Animals; Brain Diseases; Chickens; Female; Muscular Dystrophies; Naphthoquinones; Pregnancy; Pregnancy Complications; Prothrombin Time; Rabbits; Rats; Retinoids; Ubiquinone; Vitamin E; Vitamin K

Topics: Animals; Brain; Brain Diseases; Chickens; Chromans; Coenzymes; Encephalomalacia; Muscular Dystrophies; Quinones; Ubiquinone; Vitamin B 12 Deficiency; Yeasts