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. Author manuscript; available in PMC: 2013 Nov 1.
Published in final edited form as: J Clin Lipidol. 2012 Aug 30;6(6):10.1016/j.jacl.2012.08.005. doi: 10.1016/j.jacl.2012.08.005

Figure 1. Overview of the Interrelationships of Glucose, Lactate, Triglyceride, Uric Acid, and Glygogen Metabolism in the Liver.

Figure 1

UDP-glucose is uridine diphosphoglucose. Steps in the process are catalyzed by: 1. hexokinase/glucokinase, 2. glucose-6-phosphatase (G6Pase), 3. phosphoglucomutase, 4. glycogen synthase, 5. branching enzyme, 6. glycogen phosphorylase, and 7. debranching enzyme (figure as modified from references 7 and 8). In glycogen storage disease type Ia there is a block in the conversion of glucose-6-phosphate to glucose due to a deficiency of glucose-6-phosphatase. This block results in markedly enhanced conversion of glucose-6-phosphate to: 1) to pyruvate and lactate; 2) to pyruvate and then to acetyl-CoA, fatty acids and finally to triglycerides; 3) to ribose-5-phosphate and then to uric acid; or to 4) to glucose-1-phosphate, then to UDP-glucose, then to amylopectin, and then to glycogen. The excess production of lactate, triglycerides, uric acid, and glycogen can be diminished by the administration of exogenous cornstarch (glucose polymer) on a regular and prescribed basis.