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
Wanamaker Organ This level of dedication was maintained when corporate parentage shifted from the Wanamaker family to Carter Hawley Hale Stores followed by Woodward & Lothrop, The May Department Stores Company, Lord & Taylor, and finally to Macy's. The Wanamaker Organ was originally built by the Los Angeles Art Organ Company, successors to the Murray M. Harris Organ Co., for the 1904 St. Louis World's Fair. It was designed to be the largest organ in the world, an imitation of a full-size orchestra with particularly complete resources of full organ tone including mixtures. In addition to its console, the organ was originally