Estimation of glucose carbon recycling in children with glycogen storage disease: A sup 13 C NMR study using (U- sup 13 C)glucose

Journal Article · Thu Jun 01 00:00:00 EDT 1989 · Proceedings of the National Academy of Sciences of the United States of America; (USA)

DOI:https://doi.org/10.1073/pnas.86.12.4690· OSTI ID:5352891

Kalderon, B; Korman, S H; Gutman, A; Lapidot, A ^[1]

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1. Weizmann Institute of Science, Rehovot (Israel)

A stable isotope procedure to estimate hepatic glucose carbon recycling and thereby elucidate the mechanism by which glucose is produced in patients lacking glucose 6-phosphatase is described. A total of 10 studies was performed in children with glycogen storage disease type I (GSD-I) and type III (GSD-III) and control subjects. A primed dose-constant nasogastric infusion of D-(U-{sup 13}C)glucose or an infusion diluted with nonlabeled glucose solution was administered following different periods of fasting. Hepatic glucose carbon recycling was estimated from {sup 13}C NMR spectra. The values obtained for GSD-I patients coincided with the standard (U-{sup 13}C)glucose dilution curve. These results indicate that the plasma glucose of GSD-I subjects comprises only a mixture of 99% {sup 13}C-enriched D-(U-{sup 13}C)glucose and unlabeled glucose but lacks any recycled glucose. Significantly different glucose carbon recycling values were obtained for two GSD-III patients in comparison to GSD-I patients. The results eliminate a mechanism for glucose production in GSD-I children involving gluconeogenesis. However, glucose release by amylo-1,6-glucosidase activity would result in endogenous glucose production of non-{sup 13}C-labeled and nonrecycled glucose carbon, as was found in this study. In GSD-III patients gluconeogenesis is suggested as the major route for endogenous glucose synthesis. The contribution of the triose-phosphate pathway in these patients has been determined.

• OSTI ID: 5352891
• Journal Information: Proceedings of the National Academy of Sciences of the United States of America; (USA), Journal Name: Proceedings of the National Academy of Sciences of the United States of America; (USA) Vol. 86:12; ISSN 0027-8424; ISSN PNASA
• Country of Publication: United States
• Language: English

Related Subjects

550201* -- Biochemistry-- Tracer Techniques
59 BASIC BIOLOGICAL SCIENCES
AGE GROUPS
ALDEHYDES
BARYONS
BIOLOGICAL PATHWAYS
CARBOHYDRATES
CARBON 13
CARBON ISOTOPES
CHILDREN
DIAGNOSIS
DISEASES
ELEMENTARY PARTICLES
EVEN-ODD NUCLEI
FERMIONS
GLUCOSE
HADRONS
HEXOSES
ISOTOPES
LIGHT NUCLEI
MAGNETIC RESONANCE
METABOLIC DISEASES
METABOLISM
MONOSACCHARIDES
NUCLEAR MAGNETIC RESONANCE
NUCLEI
NUCLEONS
ORGANIC COMPOUNDS
PATHOGENESIS
PROTONS
RESONANCE
SACCHARIDES
STABLE ISOTOPES