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Pathway Description
Fructosuria
Homo sapiens
Disease Pathway
Fructosuria, full name essential fructosuria, is a condition that presents no symptoms and is benign. Patients with this condition exhibit a mutation in the KHK gene, which leads to fructose metabolism not being completed. This is because of a lack of the enzyme fructokinase, found in the liver. It is identified by the presence of fructose in the urine, which in people without the condition should not be present.
References
Fructosuria References
[Metagen: FRUCTOSURIA](http://metagene.de/program/d.prg?id_d=130)
[OMIM: 229800](http://omim.org/entry/229800})
[Fructosuria](http://en.wikipedia.org/wiki/Fructosuria)
Herman RH, Zakim D: Fructose metabolism. IV. Enzyme deficiencies: essential fructosuria, fructose intolerance, and glycogen-storage disease. Am J Clin Nutr. 1968 Jun;21(6):693-8. doi: 10.1093/ajcn/21.6.693.
Pubmed: 4875257
Miller CO, Yang X, Lu K, Cao J, Herath K, Rosahl TW, Askew R, Pavlovic G, Zhou G, Li C, Akiyama TE: Ketohexokinase knockout mice, a model for essential fructosuria, exhibit altered fructose metabolism and are protected from diet-induced metabolic defects. Am J Physiol Endocrinol Metab. 2018 Sep 1;315(3):E386-E393. doi: 10.1152/ajpendo.00027.2018. Epub 2018 Jun 5.
Pubmed: 29870677
Fructose and Mannose Degradation References
Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.
Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.
Sharma V, Ichikawa M, Freeze HH: Mannose metabolism: more than meets the eye. Biochem Biophys Res Commun. 2014 Oct 17;453(2):220-8. doi: 10.1016/j.bbrc.2014.06.021. Epub 2014 Jun 12.
Pubmed: 24931670
Sun SZ, Empie MW: Fructose metabolism in humans - what isotopic tracer studies tell us. Nutr Metab (Lond). 2012 Oct 2;9(1):89. doi: 10.1186/1743-7075-9-89.
Pubmed: 23031075
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