Function
Ketohexokinase (KHK) also known as hepatic fructokinase catalyzes the phosphorylation of fructose to fructose-1-phosphate using ATP as phosphate source in the liver. The HKH gene is spliced to HKH-A and the more active HKH-C. KHK acts in the first step of fructose metabolism[1].
Disease
HKH deficiency causes the essential fructosuria disorder[2].
Structural highlights
Human KHK inhibitor interacts with the 2 monomers[3]. Water molecule shown as red sphere.
- ↑ Bais R, James HM, Rofe AM, Conyers RA. The purification and properties of human liver ketohexokinase. A role for ketohexokinase and fructose-bisphosphate aldolase in the metabolic production of oxalate from xylitol. Biochem J. 1985 Aug 15;230(1):53-60. PMID:2996495
- ↑ Asipu A, Hayward BE, O'Reilly J, Bonthron DT. Properties of normal and mutant recombinant human ketohexokinases and implications for the pathogenesis of essential fructosuria. Diabetes. 2003 Sep;52(9):2426-32. PMID:12941785
- ↑ Maryanoff BE, O'Neill JC, McComsey DF, Yabut SC, Luci DK, Jordan AD Jr, Masucci JA, Jones WJ, Abad MC, Gibbs AC, Petrounia I. Inhibitors of Ketohexokinase: Discovery of Pyrimidinopyrimidines with Specific Substitution that Complements the ATP-Binding Site. ACS Med Chem Lett. 2011 Apr 18;2(7):538-43. doi: 10.1021/ml200070g. eCollection, 2011 Jul 14. PMID:24900346 doi:https://dx.doi.org/10.1021/ml200070g