Glycogen storage disease type 0 glycogen primer. Its action is highly regulated by a mechanism of phosphorylation and dephosphorylation and modulated by counter-regulatory hormones including insulin, epinephrine, and glucagon. Mutations in the gene for liver glycogen synthetase (GYS2, 138571) result in decreased or absent activity of liver glycogen synthetase and moderately decreased amounts of structurally normal glycogen in the liver. Mutational studies of patients with glycogen-storage disease type 0 do not demonstrate correlations between genotype and phenotype. [3] A different gene (GYS1, 138570) encodes muscle glycogen synthetase, which has normal activity in patients with glycogen-storage disease type 0A. In the early stages of fasting, the
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