Ketohexokinase

From Proteopedia

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spinqualitylabelsall models Human α-defensin 1 (PDB entry 2pm4) Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image 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 [3]

3D structuresm of ketohexokinase

2hlz, 2hqq, 3b3l – hKHK – human
2hw1, 3nbv – hKHK + AMPPNP + fructose
3nbw – hKHK + pyrazole derivative
3nc2, 3nc9, 3nca, 3ro4, 3q92, 3qa2, 3qai – hKHK + inhibitor

References

1. ↑ 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
2. ↑ 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
3. ↑ White SH, Wimley WC, Selsted ME. Structure, function, and membrane integration of defensins. Curr Opin Struct Biol. 1995 Aug;5(4):521-7. PMID:8528769