A Metabolic Basis for Treatment of Type I Glycogen Storage Disease

  • Harry L. Greene
Part of the Hepatology book series (H, volume 5)


Most diseases associated with excess hepatic glycogen can be traced to deficient activity of one of the enzymes involved in synthesis or degradation of glycogen (1–3). Exceptions to this are deficient activity of the glycolytic enzyme, phosphofructokinase in muscle (4), and deficient activity of the hepatic gluconeogenic enzyme glucose-6-phosphatase (G-6-Pase). The fact that glycogen synthesis and degradation can proceed unimpaired, leads to a number of clinical and chemical features unique to patients with G-6-Pase deficiency or type I glycogen storage (GSD-I).


Uric Acid Glycogen Storage Disease Glycogen Storage Disease Type Portacaval Shunt Purine Synthesis 
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Copyright information

© Plenum Press, New York 1983

Authors and Affiliations

  • Harry L. Greene
    • 1
  1. 1.Department of PediatricsVanderbilt Medical CenterNashvilleUSA

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