Blood sugar level In humans, glucose is the primary source of energy, and is critical for normal function, in a number of tissues, particularly the human brain which consumes approximately 60% of blood glucose in fasted, sedentary individuals. Glucose can be transported from the intestines or liver to other tissues in the body via the bloodstream. Cellular glucose uptake is primarily regulated by insulin, a hormone produced in the pancreas. Glucose levels are usually lowest in the morning, before the first meal of the day, and rise after meals for an hour or two by a few millimoles. Blood sugar levels outside the
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