Glucose infusion paradoxically accelerates degradation of adenine nucleotide in working muscle of patients with glycogen storage disease type VII.

Abstract

We investigated the effect of glucose infusion on adenosine triphosphate degradation in skeletal muscle of patients with glycogen storage disease type VII. Three patients and six healthy subjects exercised on a bicycle ergometer twice, once with 20% glucose infusion and once with saline infusion. The glucose infusion increased plasma glucose levels to 170 to 182 mg/dl and serum insulin levels to 30 to 50 microU/ml, while it markedly decreased plasma free fatty acid levels. The exercise-induced increases in plasma ammonia, inosine, and hypoxanthine were much larger with glucose than with saline infusion in the patients. Urinary excretion of inosine and hypoxanthine with glucose infusion was twice as high as that with saline infusion. No such differences were present between glucose and saline infusion in the healthy subjects. Glucose infusion therefore accelerates the energy crisis in working muscle of patients with glycogen storage disease type VII, probably due to a decrease in fatty acid utilization.

• Authors: A Ono, M Kuwajima, N Kono, I Mineo, C Nakagawa, S Tarui, Y Matsuzawa
• Journal: Neurology (Neurology) Vol. 45 Issue 1 Pg. 161-4 (Jan 1995) ISSN: 0028-3878 [Print] United States
• PMID: 7824108 (Publication Type: Clinical Trial, Comparative Study, Controlled Clinical Trial, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Adenine Nucleotides
• Blood Glucose
• Fatty Acids, Nonesterified
• Hypoxanthines
• Insulin
• Hypoxanthine
• Inosine
• Ammonia
• Glucose

Topics

• Adenine Nucleotides (metabolism)
• Adult
• Ammonia (blood)
• Blood Glucose (metabolism)
• Exercise Test
• Fatty Acids, Nonesterified (blood)
• Female
• Glucose (administration & dosage, pharmacology)
• Glycogen Storage Disease Type VII (metabolism, physiopathology)
• Humans
• Hypoxanthine
• Hypoxanthines (blood, urine)
• Inosine (blood, urine)
• Insulin (blood)
• Male
• Muscle, Skeletal (metabolism, physiology, physiopathology)
• Physical Exertion
• Reference Values
• Time Factors