Glycogen debranching enzyme Glycogen debranching enzyme A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen, which serves as a store of glucose in the body, through glucosyltransferase and glucosidase activity. Together with phosphorylases, debranching enzymes mobilize glucose reserves from glycogen deposits in the muscles and liver. This constitutes a major source of energy reserves in most organisms. Glycogen breakdown is highly regulated in the body, especially in the liver, by various hormones including insulin and glucagon, to maintain a homeostatic balance of blood-glucose levels. When glycogen breakdown is compromised by mutations in the glycogen debranching enzyme, metabolic diseases such
Glucose The free energy of formation of α-D-glucose is 917.2 kilojoules per mole. In humans, gluconeogenesis occurs in the liver and kidney, but also in other cell types. In the liver about 150 g of glycogen are stored, in skeletal muscle about 250 g.<ref name="Löffler/Petrides 389">Peter C. Heinrich: "Löffler/Petrides Biochemie und Pathobiochemie." Springer-Verlag, 2014, , p. 389. (german)</ref> However, the glucose released in muscle cells upon cleavage of the glycogen can not be delivered to the circulation because glucose is phosphorylated by the hexokinae, and a glucose-6-phosphatase is not expressed to remove the phosphate group. Unlike for glucose, there is no