Glycogen storage disease type IX of an individual. Mutations in PHKA2 have been seen in individuals with glycogen storage disease IXa2. The management of Glycogen storage disease IX requires treatment of symptoms by frequent intake of complex carbohydrates and protein to combat the low blood sugar. A nutritionist will advise on suitable diets. Liver function is regularly monitored and problems managed as they arise. However, liver problems have only been successfully treated by a transplant. Routine checks of metabolism are needed to ensure blood sugar (glucose) and ketones are managed. Regular moderate exercise is beneficial, although over-vigorous exercise is to be avoided, especially in those
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