Ketohexokinase (IPR034093)

Short name: KHK

Overlapping homologous superfamilies

Family relationships



Ketohexokinase (fructokinase, KHK) catalyzes the phosphorylation of fructose to fructose-1-phosphate (F1P), the first step in the metabolism of dietary fructose. KHK can also phosphorylate several other furanose sugars [PMID: 9799106]. It is found in higher eukaryotes where it is believed to function as a dimer and requires K(+) and ATP to be active [PMID: 11264590]. In humans, hepatic KHK deficiency causes fructosuria, a benign inborn error of metabolism [PMID: 12941785, PMID: 7833921].

GO terms

Biological Process

GO:0006000 fructose metabolic process

Molecular Function

GO:0004454 ketohexokinase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.