Glycogen debranching enzyme system

Tabata, S. and Dohi, Y., An assay for oligo-(l—>4) —5(1—>4)-glucantransferase activity in the glycogen debranching enzyme system by using HPLC with a pulsed amperometric detector, Carb. Res., 230,179, 1992. 

Several types of glycogen-storage disease are now known in most cases, the accumulation of glycogen can be correlated with a relative deficiency of either n-glucose 6-phosphatase or the debranching-enzyme system (Type I and III disease, respectively). In a very few cases, the enzymic defect is either in branching enzyme or in phosphorylase (Type IV or VI disease, respectively) see Table VII. In the Type II and V diseases, the enzyme deficiencies are different from the above, but have not yet been fully identified. 

---