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 storage disease type IV Glycogen storage disease type IV Glycogen storage disease type IV, also known as Andersen’s Disease, is a form of glycogen storage disease, which is caused by an inborn error of metabolism. It is the result of a mutation in the GBE1 gene, which causes a defect in the glycogen branching enzyme. Therefore, glycogen is not made properly and abnormal glycogen molecules accumulate in cells; most severely in cardiac and muscle cells. The severity of this disease varies on the amount of enzyme produced. Glycogen Storage Disease Type IV is autosomal recessive, which means each parent has a mutant copy of