A comparative study on glucagon effect between McArdle disease and Tarui disease.
Pretreatment with glucagon relieved patients with McArdle disease from muscular symptoms during exercise and enhanced exercise performance, though it did not produce any improvement in patients with Tarui disease. The difference in glucagon effect between the two diseases was clearly demonstrated in the bicycle ergometer exercise tests. In addition, the semi-ischemic forearm exercise tests performed after glucagon injection showed that increased lactate production was significantly induced by exercise in McArdle disease, but it was not the case in Tarui disease. In McArdle disease, the augmentation in exercise-induced lactate production was also observed after administration of glucose, or glucose plus insulin, but it was neither observed after administration of insulin alone nor after arginine or epinephrine administration. These findings suggest that the beneficial effect of glucagon in McArdle disease is due to the enhanced utilization of circulating glucose through the muscular glycolytic pathway realized in the coexistence of hyperglycemia and hyperinsulinemia.
D000328:Adult; D005260:Female; D005934:Glucagon / Q000494:pharmacology*; D005947:Glucose / Q000378:metabolism; D006008:Glycogen Storage Disease / Q000188:drug therapy*; D006012:Glycogen Storage Disease Type V / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D007511:Ischemia / Q000188:drug therapy / Q000503:physiopathology; D007773:Lactates / Q000097:blood; D019344:Lactic Acid; D008297:Male; D009132:Muscles / Q000187:drug effects / Q000503:physiopathology; D010732:Phosphofructokinase-1 / Q000172:deficiency*; D005082:Physical Exertion
D007773:Lactates; D019344:Lactic Acid; D005934:Glucagon; D010732:Phosphofructokinase-1; D005947:Glucose