Why would you expect the amount of g ycogen in type I glycogen-storage disease (

Question

Why would you expect the amount of g ycogen in type I glycogen-storage disease (von Gierke disease) to be increased? CAMP inhibition of glycogen synthesis is defective. Glycogen phosphatase is activated by increases in glucose 6-phosphate. Epinephrine inhibition of glycogen synthesis is defective. The R form of glycogen synthase is stabilized permanently. O Glucose 6-phosphatase is the final step in glycogenolysis. Thus, glcose 6-phosphate, the first metabolite in olycogen synthesis, remains high

Explanation / Answer

von gierke or glycogen storage disease 1 is the most common of glycogen storage disease. enzyme defected is glucose 6 phosphatase . this disease leads to alteration in liver to produce free glucose from glycogen. so body cannot breakdown glycogen. this results in hypoglycemia.

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