Dieting complex carbohydrates and fats, for energy. The first source to which the body turns is glycogen (by glycogenolysis). Glycogen is a complex carbohydrate, 65% of which is stored in skeletal muscles and the remainder in the liver (totaling about 2,000 kcal in the whole body). It is created from the excess of ingested macronutrients, mainly carbohydrates. When glycogen is nearly depleted, the body begins lipolysis, the mobilization and catabolism of fat stores for energy. In this process, fats, obtained from adipose tissue, or fat cells, are broken down into glycerol and fatty acids, which can be used to generate energy.
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