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
(glycogen-synthase-D) phosphatase 2_degree_C. (glycogen-synthase-D) phosphatase In enzymology, a [glycogen-synthase-D] phosphatase () is an enzyme that catalyzes the chemical reaction Thus, the two substrates of this enzyme are glycogen-synthase D and HO, whereas its two products are glycogen-synthase I and phosphate. This enzyme belongs to the family of hydrolases, specifically those acting on phosphoric monoester bonds. The systematic name of this enzyme class is [UDP-glucose:glycogen 4-alpha-D-glucosyltransferase-D] phosphohydrolase. Other names in common use include uridine diphosphoglucose-glycogen glucosyltransferase phosphatase, UDP-glycogen glucosyltransferase phosphatase, UDPglucose-glycogen glucosyltransferase phosphatase, glycogen glucosyltransferase phosphatase, glycogen synthetase phosphatase, glycogen synthase phosphatase, glycogen synthase D phosphatase, Mg dependent glycogen synthase phosphatase, and phosphatase