Essential fructosuria, caused by a deficiency of the enzyme hepatic fructokinase, is a clinically benign condition characterized by the incomplete metabolism of fructose in the liver, leading to its excretion in urine.[2] Fructokinase (sometimes called ketohexokinase) is the first enzyme involved in the degradation of fructose to fructose-1-phosphate in the liver.[3]
This defective degradation does not cause any clinical symptoms, fructose is either excreted unchanged in the urine or metabolized to fructose-6-phosphate by alternate pathways in the body, most commonly by hexokinase in adipose tissue and muscle.[2]
^"Essential fructosuria". Orphanet. Retrieved 11 April 2019.
^ abSteinmann B, Santer R (2012). "Disorders of Fructose Metabolism". In Saudubray JM, van den Berghe G, Walter JH (eds.). Inborn Metabolic Diseases: Diagnosis and Treatment (5th ed.). New York: Springer. pp. 157–165. ISBN 978-3-642-15719-6.
^Online Mendelian Inheritance in Man (OMIM): 229800
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→ ADP + D-fructose-1-phosphate A deficiency is associated with essentialfructosuria. Bais R, James HM, Rofe AM, Conyers RA (1985). "The purification...
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