Glycogen storage disease type III enzymes to further convert these branched glycogen polymers to glucose, limit dextrinosis abnormally accumulates in the cytoplasm. Glycogen is a molecule the body uses to store carbohydrate energy. Symptoms of GSD-III are caused by a deficiency of the enzyme amylo-1,6 glucosidase, or debrancher enzyme. This causes excess amounts of an abnormal glycogen to be deposited in the liver, muscles and, in some cases, the heart. Glycogen storage disease type III presents during infancy with hypoglycemia and failure to thrive. Clinical examination usually reveals hepatomegaly. Muscular disease, including hypotonia and cardiomyopathy, usually occurs later. The liver pathology typically regresses as 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