Starvation their normal body weight. Once the loss reaches 40% death is almost inevitable. Under normal metabolic conditions, the human body relies on free blood glucose as its primary energy source. The level of blood sugar is tightly regulated; as blood glucose is consumed, the pancreas releases glucagon, a hormone that stimulates the liver to convert stored glycogen into glucose. The glycogen stores are ordinarily replenished after every meal, but if the store is depleted before it can be replenished, the body enters hypoglycemia, and begins the starvation response. After the exhaustion of the glycogen reserve, and for the next 2–3
Glycogen debranching enzyme glucoamylase, respectively. Their active sites are selective towards the respective substrates, ensuring proper activation of GDE. Besides the active sites GDE have additional binding sites for glycogen, which are important for its recruitment to glycogen. Mapping the disease-causing mutations onto the GDE structure provided insights into glycogen storage disease type III. The official name for the gene is "amylo- α- 1,6- glucosidase, 4- α- glucanotransferase", with the official symbol AGL. AGL is an autosomal gene found on chromosome lp21. The AGL gene provides instructions for making several different versions, known as isoforms, of the glycogen debranching enzyme. These isoforms vary