G6PC, a critical enzyme for providing glucose during starvation and diabetes , is involved in the terminal step in gluconeogenic and glycogenolytic
pathways and catalyzes the hydrolysis of glucose 6-phosphate (G6P) to glucose and inorganic phosphate.
General objective: The implementation of an EU registry of patients with McArdle disease and very rare muscle glycogenolytic
disorders (MGD) with exercise intolerance as the major symptom (PR-MDMGD).
Furthermore, the defect in glycogenolytic
pathways and limited lipolysis also contribute to the occurrence of hypoglycaemia in undernutrition.
In vitro studies of the carbohydrate metabolism in isolated frog hepatocytes revealed that they are sensitive to insulin and glycogenolytic
hormones, similar to verified in mammal hepatocytes.