Skip to main content
SpringerLink
Log in

Pathogenic mechanisms of disorders in fructose metabolism

  • Chapter
Inherited Disorders of Carbohydrate Metabolism

Abstract

Fructose is a normal constituent of the Western diet occurring in some fruits and vegetables and in honey. It is also a component of the disaccharide sucrose which is used extensively in the manufacture of confectionery. The usual daily intake has been estimated to be 50–100 grams. Three main types of metabolic defect involving fructose have been described in man:

  • essential fructosuria (EF)

  • hereditary fructose intolerance (HFI)

  • fructose-1,6-diphosphatase deficiency (FDD)

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. A. I. Mendeloff and T. E. Weichselbaum, (1953). Role of the human liver in the assimilation of intravenously administered fructose. Metabolism, 2, 450

    PubMed  CAS  Google Scholar 

  2. H. S. Deuel, (1936). The intermediary metabolism of fructose and galactose. Physiol. Rev., 16, 173

    CAS  Google Scholar 

  3. K. O. Raivio, M. P. Kekomaki and P. H. Maenpaa, (1969). Depletion of liver adenine nucleotides induced by D-fructose. Dose dependence and specificity of the fructose effect. Biochem. Pharmacol., 18, 2615

    Article  PubMed  CAS  Google Scholar 

  4. F. Leuthardt and E. Testa, (1951). Die phosphorylierung der fructose in der leber. Helv. Chim. Acta, 34, 931

    Article  CAS  Google Scholar 

  5. R. C. Adelman, F. J. Ballard and S. Weinhouse, (1967). Purification and properties of rat liver fructokinase. J. Biol. Chem., 242, 3360

    PubMed  CAS  Google Scholar 

  6. H. F. Woods, L. V. Eggleston and H. A. Krebs, (1970). The cause of hepatic accumulation of fructose-1-phosphate on fructose loading. Biochem. J., 119, 5

    CAS  Google Scholar 

  7. F. Heinz, W. Lamprecht and J. Kirsch, (1968). Enzymes of fructose metabolism in human liver. J. Clin. Invest., 47, 1826

    Article  PubMed  CAS  Google Scholar 

  8. R. E. Parks, E. Ben-Gershom and H. A. Lardy, (1957). Liver fructokinase. J. Biol. Chem., 227, 231

    PubMed  CAS  Google Scholar 

  9. H. G. Hers, (1952). La fructokinase de foie. Biochem. Biophys. Acta, 8, 416

    Article  PubMed  CAS  Google Scholar 

  10. H. G. Hers, (1952). Role du magnesium et du potassium dans la reaction fructokinasique. Biochim. Biophys. Acta, 8, 424

    Article  PubMed  CAS  Google Scholar 

  11. H. F. Woods, (1972). Hepatic accumulation of metabolites after fructose loading. Acta. Med. Scand., 542, 87

    CAS  Google Scholar 

  12. F. Leuthardt, E. Testa and H. P. Wolf, (1953). Der enzymatische Abban des fructose-1-phosphate in der leber. Helv. Chim. Acta, 36, 227

    Article  CAS  Google Scholar 

  13. M. A. G. Sillero, A. Sillero and A. Sols, (1969). Enzymes involved in fructose metabolism in liver and the glyceraldehyde metabolic crossroads. Eur. J. Biochem., 10, 345

    Article  PubMed  CAS  Google Scholar 

  14. L. Hue and H. G. Hers, (1972). 4-3H fructose and of 4-3H) glucose to liver glycogen in the mouse. An investigation of the glyceraldehyde crossroads. Eur. J. Biochem., 29, 268

    Article  PubMed  CAS  Google Scholar 

  15. J. Bergstrom, P. Fiirst, F. Gallyas, E. Hultman, L. H. SonNilsson, A. E. RochNorlund and E. Vinnas (1972). Aspects of fructose metabolism in normal man. Acta Med. Scand., 542, 57

    CAS  Google Scholar 

  16. K. Kjerulf-Jensen, (1942). The phosphate esters formed in the liver tissue of rats and rabbits during assimilation of hexoses and glycerol. Acta Physiol. Scand., 4, 249

    Article  CAS  Google Scholar 

  17. H. B. Burch, P. Max, K. Chyu and O. H. Lowry, (1969). Metabolic intermediates in liver of rats given large amounts of fructose or dihydroxyacetone. Biochem. Biophys. Res. Commun., 34, 619

    Article  PubMed  CAS  Google Scholar 

  18. H. G. Hers, (1955). The conversion of fructose-1-C14 and sorbitol-1-C14 to liver and muscle glycogen in the rat. J. Biol. Chem., 214, 373

    PubMed  CAS  Google Scholar 

  19. A. Sols and r. K. Crane, (1954). Substrate specificity of brain hexokinase. J. Biol. Chem., 210, 581

    PubMed  CAS  Google Scholar 

  20. J. Nissel and L. Linden, (196). Fructose-1-phosphate aldolase and fructose-1,6-diphosphate aldolase activity in the mucosa of the intestine in hereditary fructose intolerance. Scand. J. Gastroenterol., 3, 80

    Article  Google Scholar 

  21. J. F. Kranold, D. Loh and R. C. Morris Jr, (1969). Renal fructose-metabolizing enzymes: significance in hereditary fructose intolerance. Science, 165, 402

    Article  Google Scholar 

  22. J. Perheentupa, K. O. Raivio and E. A. Nikkila, (1972). Hereditary fructose intolerance. Acta Med. Scand., 542, 65

    CAS  Google Scholar 

  23. H. G. Hers, (1970). Misuses for fructose. Nature, 227, 421

    Article  PubMed  CAS  Google Scholar 

  24. H. F. Woods and K. G. M. M. Alberti, (1972). Dangers of intravenous fructose. Lancet, 2, 1354

    Article  PubMed  CAS  Google Scholar 

  25. G. Van den Berghe, (1975). Fructose metabolism. In F. A. Hommes and C. J. Van den Berghe (eds.). tiNormal and Pathological Development of Energy Metabolism, p. 211 (Academic Press London)

    Google Scholar 

  26. P. H. Maenpaa, K. O. Raivio and M. P. Kekomaki, (1968). Liver adenine nucleotides: fructose induced depletion and its effect on protein synthesis. Science, 161, 1253

    Article  PubMed  CAS  Google Scholar 

  27. A. L. Lehninger, J. Sice and E. V. Jensen, (1955). Effect of substrate structure on the aldolase equilibrium. Biochim. Biophys. Acta, 17, 285

    Article  PubMed  CAS  Google Scholar 

  28. H. B. Burch, O. H. Lowry, L. Meinhardt, P. Max and K. Chyu, (1970). Effect of fructose, dihydroxyacetone, glycerol and glucose on metabolites and related compounds in liver and kidney. J. Biol. Chem., 245, 2092

    PubMed  CAS  Google Scholar 

  29. H. F. Woods and H. A. Krebs, (1973). The effect of glycerol and dihydroxyacetone on hepatic adenine nucleotides. Biochem. J., 132, 55

    PubMed  CAS  Google Scholar 

  30. H. F. Woods and H. A. Krebs, (1973). Xylitol metabolism in the isolated perfused rat liver. Biochem. J., 134, 437

    PubMed  CAS  Google Scholar 

  31. D. T. Yu and M. J. Phillips, (1971). Hepatic ultrastructural changes in acute fructose overload. Ultrastructure Res. 36, 222

    Article  CAS  Google Scholar 

  32. P. H. Maenpaa, (1972). Fructose induced alterations in liver polysome profiles and Mg2+ levels. FEBS Lett., 24, 37

    Article  PubMed  CAS  Google Scholar 

  33. J. Ch. Bode, O. Zelder, H. J. Rumpell and U. Wittkamp, (1973). [atDepletion of liver adenosine phosphates and metabolic effects of intravenous infusion of fructose or sorbitol in man and in the rat. Eur. J. Clin. Invest., 3, 436

    Article  PubMed  CAS  Google Scholar 

  34. R. C. Morris Jr., E. McSherry and A. Sebastian, (1973). On the metabolic pathogenesis of the renal disorder of hereditary fructose intolerance ( HFI). J. Clin. Invest., 52, 57

    Google Scholar 

  35. G. Van den Berghe, (1973). M’emoires de l’Academie Royale de Medecine de Belgique, 47, 93

    Google Scholar 

  36. W. Schumer, (1971). Adverse effects of xylitol in parenteral alimentation. Metabolism, 20, 345

    Article  PubMed  CAS  Google Scholar 

  37. I. G. Walton, H. F. Woods and T. D. R. Hockaday, (1973). Inhibition by sucrose of triglyceride production by the perfused rat. Proceedings of Spring Meeting of the Medical and Scientific Sections of the British Diabetic Association

    Google Scholar 

Download references

Authors
  1. H. F. Woods
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Bristol Royal Hospital for Sick Children, Bristol, UK

    D. Burman

  2. Department of Clinical Chemistry, Southmead Hospital, Bristol, UK

    J. B. Holton

  3. Clinical Chemistry Department, Bristol Maternity Hospital, Bristol, UK

    C. A. Pennock

Rights and permissions

Reprints and permissions

Copyright information

© 1980 The Society for the Study of Inborn Errors of Metabolism

About this chapter

Cite this chapter

Woods, H.F. (1980). Pathogenic mechanisms of disorders in fructose metabolism. In: Burman, D., Holton, J.B., Pennock, C.A. (eds) Inherited Disorders of Carbohydrate Metabolism. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-9215-3_12

Download citation

  • DOI: https://doi.org/10.1007/978-94-009-9215-3_12

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-009-9217-7

  • Online ISBN: 978-94-009-9215-3

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation