Question

Von Gierke’s is a glycogen storage disease that results from glucose 6-phosphatase deficiency. Why is this considered a glycogen storage disease though glucose 6-phosphatase is not involved in glycogen synthesis or catabolism? The patients present with hypoglycemia, lactic acidosis, hepatomegaly (enlarged liver), and increased uric acid production, uric acid being a product of nucleotide degradation. Explain the molecular basis of these symptoms given what you know of the function of glucose 6-phosphatase and its localization.