Deficiencies in the enzyme glucose-6-phosphatase are likely to lead to which of the following?
High-Yield Explanation
Glucose-6-phosphate is required in the liver in order for this organ to supply the rest of the body with glucose that has been produced by hepatic gluconeogenesis or released from stored hepatic glycogen. A deficiency in glucose-6- phosphatase leads to the most common of the glycogen storage diseases, von Gierke's disease. This glycogen storage disease results from excess hepatic glucose-6-phosphate allosterically activating the beta form of glycogen synthase leading to increased incorporation of glucose into glycogen. Ref: Bender D.A., Mayes P.A. (2011). Chapter 20. Gluconeogenesis & the Control of Blood Glucose. In D.A. Bender, K.M. Botham, P.A. Weil, P.J. Kennelly, R.K. Murray, V.W. Rodwell (Eds), Harper's Illustrated Biochemistry, 29e.