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.
Adult polyglucosan body disease shortage (deficiency) of the glycogen branching enzyme, which leads to the production of abnormal glycogen molecules. These abnormal glycogen molecules, called polyglucosan bodies, accumulate within cells and cause damage. Neurons appear to be particularly vulnerable to the accumulation of polyglucosan bodies in people with this disorder, leading to impaired neuronal function. Some mutations in the "GBE1" gene that cause adult polyglucosan body disease do not result in a shortage of glycogen branching enzyme. In people with these mutations, the activity of this enzyme is normal. How mutations cause the disease in these individuals is unclear. Other people with adult polyglucosan