Hepatic fructokinase

Hepatic fructokinase

protein
Name=ketohexokinase (fructokinase)
caption=


width=
HGNCid=6315
Symbol=KHK
AltSymbols=
EntrezGene=3795
OMIM=229800
RefSeq=NM_006488
UniProt=P50053
PDB=
ECnumber=2.7.1.3
Chromosome=2
Arm=p
Band=23.3
LocusSupplementaryData=-23.2

Hepatic fructokinase (or ketohexokinase ) is an enzyme that catalyzes the phosphorylation of fructose to produce fructose-1-phosphate.

:ATP + D-fructose → ADP + D-fructose-1-phosphatecite journal |author=Bais R, James HM, Rofe AM, Conyers RA |title=The purification and properties of human liver ketohexokinase. A role for ketohexokinase and fructose-bisphosphate aldolase in the metabolic production of oxalate from xylitol |journal=Biochem. J. |volume=230 |issue=1 |pages=53–60 |year=1985 |pmid=2996495 |doi=]

Pathology

A deficiency is associated with essential fructosuria.

References

External links

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  • essential fructosuria — a benign, asymptomatic, autosomal recessive disorder of carbohydrate metabolism due to deficiency of hepatic fructokinase resulting from mutations in the KHK gene (locus: 2p23.3 23.2); the only manifestations are fructosemia and fructosuria. See… …   Medical dictionary

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