Changes of liver metabolite concentrations in adults with disorders of fructose metabolism after intravenous fructose by 31P magnetic resonance spectroscopy.

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  • Additional Information
    • Source:
      Publisher: Nature Publishing Group Country of Publication: United States NLM ID: 0100714 Publication Model: Print Cited Medium: Print ISSN: 0031-3998 (Print) Linking ISSN: 00313998 NLM ISO Abbreviation: Pediatr Res Subsets: MEDLINE
    • Publication Information:
      Publication: 2012- : New York : Nature Publishing Group
      Original Publication: Basel ; New York : Karger.
    • Subject Terms:
      Fructose/*metabolism ; Fructose Metabolism, Inborn Errors/*metabolism ; Liver/*metabolism ; Magnetic Resonance Spectroscopy/*methods ; Muscles/*metabolism; Adenosine Triphosphate/metabolism ; Adult ; Fasting ; Fructose/administration & dosage ; Fructose/pharmacology ; Fructose Intolerance/metabolism ; Fructose-1,6-Diphosphatase Deficiency/metabolism ; Fructosephosphates/metabolism ; Humans ; Hydrogen-Ion Concentration ; Kinetics ; Organophosphates/metabolism ; Phosphates/metabolism ; Phosphocreatine/metabolism ; Phosphorus ; Reference Values ; Time Factors
    • Abstract:
      A novel 31P magnetic resonance spectroscopy procedure allows the estimation of absolute concentrations of certain phosphorus-containing compounds in liver. We have validated this approach by measuring ATP, phosphomonesters, and inorganic phosphate (Pi) during fasting and after an i.v. fructose bolus in healthy adults and in three adults with disorders of fructose metabolism and by comparing results with known metabolic concentrations measured chemically. During fasting, the ATP concentration averaged 2.7 +/- 0.3 (SD, n = 9) mmol/L, which, after due correction for other nucleoside triphosphates, was 2.1 mmol/L and corresponded well with known concentrations. Fructose-1-phosphate (F-1-P) could not be measured during fasting; its concentration after fructose was calculated from the difference of the phosphomonester signals before (2.9 +/- 0.2 mmol/L) and after fructose. Pi was 1.4 +/- 0.3 mmol/L and represented the one fourth of Pi visible in magnetic resonance spectra. In the three healthy controls after fructose (200 mg/kg, 20% solution, 2.5 min), the fructokinase-mediated increase of F-1-P was rapid, reaching 4.9 mmol/L within 3 min, whereas the uncorrected ATP decreased from 2.7 to 1.8 mmol/L and the Pi from 1.4 to 0.3 mmol/L. The subsequent decrease of F-1-P, mediated by fructaldolase, was accompanied by an overshooting rise of Pi to 2.7 mmol/L. In the patient with essential fructosuria, the concentrations of F-1-P, ATP, and Pi remained unchanged, confirming that fructokinase was indeed inactive. In the patient with hereditary fructose intolerance, initial metabolic changes were the same as in the controls, but baseline concentrations were not yet reestablished after 7 h, indicating weak fructaldolase activity.(ABSTRACT TRUNCATED AT 250 WORDS)
    • Accession Number:
      0 (Fructosephosphates)
      0 (Organophosphates)
      0 (Phosphates)
      020IUV4N33 (Phosphocreatine)
      15978-08-2 (fructose-1-phosphate)
      27YLU75U4W (Phosphorus)
      30237-26-4 (Fructose)
      8L70Q75FXE (Adenosine Triphosphate)
    • Publication Date:
      Date Created: 19941001 Date Completed: 19950208 Latest Revision: 20131121
    • Publication Date:
      20231215
    • Accession Number:
      10.1203/00006450-199410000-00004
    • Accession Number:
      7816517