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Ketohexokinase

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{{STRUCTURE_3nbv| PDB=3nbv | SIZE=400| SCENE= |right|CAPTION=Ketohexokinase dimer complex with fructose and AMPPNP (stick model) [[3nbv]] }}
{{STRUCTURE_3nbv| PDB=3nbv | SIZE=400| SCENE= |right|CAPTION=Ketohexokinase dimer complex with fructose and AMPPNP (stick model) [[3nbv]] }}
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<StructureSection load='2q92' size='350' side='right' caption='Human α-defensin 1 (PDB entry [[2pm4]])' scene=''>
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== Function ==
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'''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<ref>PMID:2996495</ref>.
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== Disease ==
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HKH deficiency causes the essential fructosuria disorder<ref>PMID:12941785</ref>.
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'''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. HKH deficiency causes the essential fructosuria disorder. KHK acts in the first step of fructose metabolism.
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== Structural highlights ==
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<ref>PMID:8528769</ref>
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</StructureSection>
==3D structuresm of ketohexokinase==
==3D structuresm of ketohexokinase==
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[[3nbw]] – hKHK + pyrazole derivative<br />
[[3nbw]] – hKHK + pyrazole derivative<br />
[[3nc2]], [[3nc9]], [[3nca]], [[3ro4]], [[3q92]], [[3qa2]], [[3qai]] – hKHK + inhibitor
[[3nc2]], [[3nc9]], [[3nca]], [[3ro4]], [[3q92]], [[3qa2]], [[3qai]] – hKHK + inhibitor
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== References ==
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<references/>
[[Category:Topic Page]]
[[Category:Topic Page]]

Revision as of 09:30, 6 April 2016

Template:STRUCTURE 3nbv

Human α-defensin 1 (PDB entry 2pm4)

Drag the structure with the mouse to rotate

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

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Michal Harel, Alexander Berchansky

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