Why would you expect the amount of g ycogen in type I glycogen-storage disease (
ID: 220137 • Letter: W
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 highExplanation / 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.