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 storage disease type II Glycogen storage disease type II Glycogen storage disease type II, also called Pompe disease, is an autosomal recessive metabolic disorder which damages muscle and nerve cells throughout the body. It is caused by an accumulation of glycogen in the lysosome due to deficiency of the lysosomal acid alpha-glucosidase enzyme. It is the only glycogen storage disease with a defect in lysosomal metabolism, and the first glycogen storage disease to be identified, in 1932 by the Dutch pathologist J. C. Pompe. The build-up of glycogen causes progressive muscle weakness (myopathy) throughout the body and affects various body tissues, particularly in the