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-branching enzyme deficiency known as glycogen synthase. This bond may be broken by amylase when the body wishes to break down glycogen into glucose for energy. Glycogen branching enzyme is responsible for the required α-1,6-glycosidic bonds needed to start a branch off of these linear chains. These branches are important, as they provide additional "free ends" for linear chains of α-1,4-glycosidic bonds, which can then be broken down by amylase. This allows for glucose to be removed at a faster rate than if all glucose molecules were in a single chain with only two free ends on which amylase could attach. GBED is