Transfers the glycosyl residue from UDP-Glc to the non- reducing end of alpha-1,4-glucan. Defects in GYS2 are the cause of glycogen storage disease type 0 (GSD0); A metabolic disorder characterized by fasting hypoglycemia presenting in infancy or early childhood, high blood ketones and low alanine and lactate concentrations. Although feeding relieves symptoms, it often results in postprandial hyperglycemia and hyperlactatemia. Belongs to the glycosyltransferase 3 family. Note: This description may include information from UniProtKB.
Protein type: Carbohydrate Metabolism - starch and sucrose; Cytoskeletal; EC 184.108.40.206; Transferase