TM Cox, University of Cambridge, Cambridge, UK
Published online: March 2002
DOI: 10.1038/npg.els.0002271
Fructose is an important macronutrient and fructose esters participate critically in intermediary metabolism; thus, inherited defects of enzymes involved in handling of dietary fructose may have profound metabolic effects. Examples are essential fructosuria, hereditary fructose intolerance and fructose-1,6-bisphosphatase deficiency.
Keywords: inborn error; aldolase B; fructokinase; fructose; sucrose; metabolism; 1,6 bisphosphatase
| References | |
| Ali M, Rellos P and Cox TM (1998) Hereditary fructose intolerance. Journal of Medical Genetics 35: 353–365. | |
| Burrant CF, Takeda J, Brot-Laroche E, Bell GI and Davidson NO (1992) Fructose transporter in human spermatozoa and small intestine is GLUT 5. Journal of Biological Chemistry 267: 14523–14256. | |
| Cox TM (1994) Aldolase B and fructose intolerance. FASEB Journal 8: 62–71. | |
| book El-Maghrabi MR, Colosia AD and Lange AJ (1990) "Liver 6-phosphofructo-2-kinase/fructose 2,6-bisphosphatase". In: Pilkis SJ (ed.) Fructose-2,6-Bisphosphate, pp. 87–124. Boca Raton, FL: CRC Press. | |
| book Hers H-G (1957) Le Metabolisme du Fructose. Brussels: Editions Arsica. | |
| Hommes FA (1993) Inborn errors of fructose metabolism. American Journal of Clinical Nutrition 58(supplement): 788S–795S. | |
| Park YK and Yetley EA (1993) Intakes and food sources in the United States. American Journal of Clinical Nutrition 58(supplement): 737S–747S. | |
| Pilkis SJ, El-Maghrabi MR, Pilkis J and Claus T (1981) Inhibition of fructose-1,6-bisphosphatase by fructose 2,6-bisphosphate. Journal of Biological Chemistry 256: 3619–3622. | |
| book Putnam JJ and Allhouse JE (1991) Food Consumption, Prices and Expenditures, 1968–1989. Statistical Bulletin no. 825. Washington, DC: US Department of Agriculture/Economic Research Service. | |
| book Steinmann B, Gitzelmann R and Van den Berghe G (2001) "Disorders of fructose metabolism". In: Scriver CR, Beaudet A, Valle D and Sly WS (eds) The Metabolic and Molecular Bases of Inherited Disease, pp 1489–1520. New York: McGraw-Hill. | |
| Van Schaftingen E (1987) Fructose 2,6-bisphosphate Advances in Enzymology 59: 315–395. | |
| book Van Schaftingen E (1990) "Fructose 2,6-bisphosphate in the regulation of hepatic carbohydrate metabolism". In: Pilkis SJ (ed.) Fructose-2,6-Bisphosphate, pp. 65–85. Boca Raton, FL: CRC Press. | |
| Van Schaftingen E, Jett MF, Hue L and Hers H-G (1981) Control of liver 6-phosphofructokinase by fructose 2,6-bisphosphate and other effectors. Proceedings of the National Academy of Sciences of the USA 78: 3483–3488. | |
| Van Schaftingen E, Betheux M and Veiga da Cunha M (1994) Short-term control of glucokinase activity: role of a regulatory protein. FASEB Journal 8: 414–419. | |
| Further Reading | |
| Ali M, Rellos P and Cox TM (1998) Hereditary fructose intolerance. Journal of Medical Genetics 35: 353–365. | |
| Bell L and Sherwood W (1987) Current practices and improved recommendations for treating hereditary fructose intolerance. Journal of the American Dietetic Association 87: 721–728. | |
| Burlina AB, Paletto M, Shin YS and Zacchello F (1990) Clinical and biochemical observations on three cases of fructose-1,6-diphosphatase deficiency. Journal of Inherited Metabolic Disease 13: 263–266. | |
| book Cornblath M and Schwartz R (1991) Disorders of Carbohydrate Metabolism in Infancy, 3rd edn, pp. 325–351, 355–358. Boston: Blackwell Scientific. | |
| Forbes AL and Bowman BA (eds) (1993) Health effects of dietary fructose. American Journal of Clinical Nutrition 58(supplement): 721S-823S. | |
| Hommes FA (1993) Inborn errors of fructose metabolism. American Journal of Clinical Nutrition 58(supplement): 788S–795S. | |
| book Steinmann B, Gitzelmann R and Van den Berghe G (2001) "Disorders of fructose metabolism", In: Scriver CR, Beaudet A, Valle D and Sly WS (eds) The Metabolic and Molecular Bases of Inherited Disease, 8th edn, pp. 1489–1520. New York: McGraw-Hill. | |
Copyright © 2000-2013 by John Wiley & Sons, Inc., or related companies. All rights reserved.
