Determination of fructose metabolic pathways in normal and fructose-intolerant children: A sup 13 C NMR study using (U- sup 13 C)fructose

Journal Article · Sun Jul 01 00:00:00 EDT 1990 · Proceedings of the National Academy of Sciences of the United States of America; (USA)

DOI:https://doi.org/10.1073/pnas.87.14.5449· OSTI ID:6108441

Gopher, A; Lapidot, A ^[1]; Vaisman, N ^[2]; Mandel, H ^[3]

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1. Weizmann Institute of Science, Rehovot (Israel)
2. Kaplan Hospital, Rehovot (Israel)
3. Rambam Hospital, Haifa (Israel)

An inborn deficiency in the ability of aldolase B to split fructose 1-phosphate is found in humans with hereditary fructose intolerance (HFI). A stable isotope procedure to elucidate the mechanism of conversion of fructose to glucose in normal children and in HFI children has been developed. A constant infusion of D-(U-{sup 13}C)fructose was given nasogastrically to control and to HFI children. Hepatic fructose conversion to glucose was estimated by examination of {sup 13}C NMR spectra of plasma glucose. Significantly lower values ({approx}3-fold) for fructose conversion to glucose were obtained for the HFI patients as compared to the controls. A quantitative determination of the metabolic pathways of fructose conversion to glucose was derived from {sup 13}C NMR measurement of plasma ({sup 13}C)glucose isotopomer populations. The finding of isotopomer populations of three adjacent {sup 13}C atoms at glucose C-4 ({sup 13}C{sub 3}-{sup 13}C{sub 4}-{sup 13}C{sub 5}) suggests that there is a direct pathway from fructose, by-passing fructose-1-phosphate aldolase, to fructose 1,6-bisphosphate. The metabolism of fructose by fructose-1-phosphate aldolase activity accounts for only {approx}50% of the total amount of hepatic fructose conversion to glucose. In view of the marked decline by 67% in synthesis of glucose from fructose in HFI subjects found in this study, the extent of ({sup 13}C)glucose formation from a trace amount of (U-{sup 13}C)fructose infused into the patient can be used as a safe and noninvasive diagnostic test for inherent faulty fructose metabolism.

• OSTI ID: 6108441
• Journal Information: Proceedings of the National Academy of Sciences of the United States of America; (USA), Vol. 87:14; ISSN 0027-8424
• Country of Publication: United States
• Language: English

Related Subjects

59 BASIC BIOLOGICAL SCIENCES
BLOOD PLASMA
NUCLEAR MAGNETIC RESONANCE
FRUCTOSE
BIOLOGICAL PATHWAYS
GLUCOSE
LABELLED COMPOUNDS
INFUSION
METABOLIC DISEASES
DIAGNOSIS
ALDOLASES
CARBON 13
NMR SPECTRA
PATIENTS
ALDEHYDES
BIOLOGICAL MATERIALS
BLOOD
BODY FLUIDS
CARBOHYDRATES
CARBON ISOTOPES
CARBON-CARBON LYASES
DISEASES
ENZYMES
EVEN-ODD NUCLEI
HEXOSES
INTAKE
ISOTOPES
KETONES
LIGHT NUCLEI
LYASES
MAGNETIC RESONANCE
MATERIALS
MONOSACCHARIDES
NUCLEI
ORGANIC COMPOUNDS
RESONANCE
SACCHARIDES
SPECTRA
STABLE ISOTOPES
550201* - Biochemistry- Tracer Techniques