Hepatic fructokinase

ketohexokinase (fructokinase)
Ketohexokinase homodimer, Human
Identifiers
SymbolKHK
NCBI gene3795
HGNC6315
OMIM229800
RefSeqNM_006488
UniProtP50053
Other data
EC number2.7.1.3
LocusChr. 2 p23.3-23.2
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StructuresSwiss-model
DomainsInterPro
Ketohexokinase
Identifiers
EC no.2.7.1.3
CAS no.9030-50-6
Databases
IntEnzIntEnz view
BRENDABRENDA entry
ExPASyNiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
PRIAMprofile
PDB structuresRCSB PDB PDBe PDBsum
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NCBIproteins

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

ATP +    ADP +
ATP + D-fructose → ADP + D-fructose-1-phosphate[1]

Isoforms

In humans, ketohexokinase is encoded by the KHK gene, which produces two isoforms, KHK-A and KHK-C, through alternative splicing.

  • KHK-C is primarily expressed in the liver, kidney, and intestine. It has a high affinity for fructose (low Km) and is responsible for the majority of fructose metabolism.
  • KHK-A is ubiquitously expressed in many tissues at low levels. It has a significantly lower affinity for fructose (high Km) compared to the C isoform.[2]

References

  1. ^ Bais R, James HM, Rofe AM, Conyers RA (1985). "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. 230 (1): 53–60. doi:10.1042/bj2300053. PMC 1152585. PMID 2996495.
  2. ^ Diggle CP, Shires M, Leitch D, Brooke D, Carr IM, Markham AF, Hayward BE, Asipu A, Bonthron DT (2009). "Ketohexokinase: Expression and Localization of the Principal Fructose-metabolizing Enzyme". Journal of Histochemistry & Cytochemistry. 57 (8): 763–774. doi:10.1369/jhc.2009.953190. PMC 2713076. PMID 19365088.