Glycogen branching enzyme leads to increased osmotic pressure resulting in cellular swelling and death. The tissues most affected by this disease are the liver, heart, and neuromuscular system, areas with the greatest levels of glycogen accumulation. Abnormal glycogen buildup in the liver interferes with liver functioning and can result in an enlarged liver and liver disease. In muscles, the inability of cells to efficiently breakdown glycogen due to the severe reduction or absence of branching can lead to muscle weakness and atrophy. At least three mutations in the GBE1 gene have been found to cause another disease called adult polyglucosan body disease (APBD).
Reducing sugar dextrin the macromolecule begins with a reducing sugar, a free aldehyde. More hydrolyzed starch contains more reducing sugars. The percentage of reducing sugars present in these starch derivatives is called dextrose equivalent (DE). Glycogen is a highly branched polymer of glucose that serves as the main form of carbohydrate storage in animals. It is a reducing sugar with only one reducing end; no matter how large the glycogen molecule is or how many branches it has, each branch ends in a nonreducing sugar residue. When glycogen is broken down to be used as an energy source, glucose units are removed