Glycogen storage disease branching enzyme deficiency

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. 

Glycogen Storage Disease Type IV Branching Enzyme Deficiency. 304... 

Table 15.11. Glycogen storage disease type 4 (branching enzyme deficiency) ...

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