Glycogen storage disease type I similar, but Ib incurs a few additional problems (described below). Glycogen in liver and (to a lesser degree) kidneys serves as a form of stored, rapidly accessible glucose, so that the blood glucose level can be maintained between meals. For about 3 hours after a carbohydrate-containing meal, high insulin levels direct liver cells to take glucose from the blood, to convert it to glucose-6-phosphate (G6P) with the enzyme glucokinase, and to add the G6P molecules to the ends of chains of glycogen (glycogen synthesis). Excess G6P is also shunted into production of triglycerides and exported for storage in adipose tissue
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