ATP Depletion, a Possible Role in the Pathogenesis of Hyperuricemia in Glycogen Storage Disease Type I

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Abstract

Other investigators have shown that fructose infusion in normal man and rats acutely depletes hepatic ATP and Pi and increases the rate of uric acid formation by the degradation of preformed nucleotides. We postulated that a similar mechanism of ATP depletion might be present in patients with glucose-6-phosphatase deficiency (GSD-I) as a result of ATP consumption during glycogenolysis and resulting excess glycolysis. The postulate was tested by measurement of: (a) hepatic content of ATP, glycogen, phosphorylated sugars, and phosphorylase activities before and after increasing glycolysis by glucagon infusion and (b) plasma urate levels and urate excretion before and after therapy designed to maintain blood glucose levels above 70 mg/dl and thus prevent excess glycogenolysis and glycolysis.

Authors

Harry L. Greene, Frederick A. Wilson, Patrick Hefferan, Annie B. Terry, Jose Roberto Moran, Alfred E. Slonim, Thomas H. Claus, Ian M. Burr