Glucose cycle The glucose cycle can occur in liver cells due to a liver specific enzyme glucose-6-phosphatase, which catalyse the dephosphorylation of glucose 6-phosphate back to glucose. Glucose-6-phosphate is the product of glycogenolysis or gluconeogenesis, where the goal is to increase free glucose in the blood due body being in catabolic state. Other cells such as muscle and brain cells do not contain glucose 6-phosphatase. As a result, any glucose 6-phosphate produced in those cells is committed to cellular metabolic pathways, primarily pentose phosphate pathway or glycolysis. Flux through the glucose cycle is regulated by several hormones including insulin and glucagon as
Glycogen storage disease type 0 liver provides a steady source of glucose from glycogen breakdown (or glycogenolysis). With prolonged fasting, glucose is generated in the liver from noncarbohydrate precursors through gluconeogenesis. Such precursors include alanine (derived from the breakdown of proteins in skeletal muscle) and glycerol (derived from the breakdown of triacylglycerols in fat cells). In patients with glycogen-storage disease type 0, fasting hypoglycemia occurs within a few hours after a meal because of the limited stores of hepatic glycogen and inadequate gluconeogenesis to maintain normoglycemia. Feeding characteristically results in postprandial hyperglycemia and glucosuria, in addition to increased blood lactate levels, because glycogen synthesis is