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Sugar kinases

Unless otherwise stated all data on this page refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).


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These are enzymes that catalyse the phosphorylation of sugar molecules. Shared three-dimensional structures identify distinct subfamilies; hexokinases, ribokinases and the eukaryotic mevalonate and phosphomevalonate kinases [2].


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ketohexokinase Show summary »

Target Id 3236
Nomenclature ketohexokinase
Previous and unofficial names fructokinase
Genes KHK (Hs), Khk (Mm), Khk (Rn)
Ensembl ID ENSG00000138030 (Hs), ENSMUSG00000029162 (Mm), ENSRNOG00000008047 (Rn)
UniProtKB AC P50053 (Hs), P97551 (Rn)
EC number
PF-06835919 pIC50 8.0 [4]
LY3522348 pIC50 7.6 – 7.7 [3], 7.4 [3]
Comment KHK catalyses the first step of metabolism of dietary fructose, converting fructose to fructose-1-phosphate. Two catalytically active isoforms of human KHK have been reported: KHK-C and KHK-A [1]. KHK-C is expressed predominantly in liver, kidney, and small intestine, whilst KHK-A is more widely distributed in other tissues. Inherited KHK deficiency causes essential fructosuria, a benign condition in which the excess fructose is excreted into the urine.

KHK inhibitors are expected to provide clinical benefit by reducing the effects of excess dietary fructose on promoting metabolic dysfunction. In particular KHK inhibitors are proposed to reduce the hepatic manifestations of metabolic syndrome, i.e. hepatic steatosis, fibrosis and inflammation in conditions such as non-alcoholic fatty liver disease (NAFLD) and non-alcoholic steatohepatitis (NASH). This mechanistic approach may also provide beneficial effects in hyperglycemia and insulin resistance.


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How to cite this family page

Database page citation:

Sugar kinases. Accessed on 20/05/2024. IUPHAR/BPS Guide to PHARMACOLOGY,

Concise Guide to PHARMACOLOGY citation:

Alexander SPH, Fabbro D, Kelly E, Mathie AA, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Davies JA et al. (2023) The Concise Guide to PHARMACOLOGY 2023/24: Enzymes. Br J Pharmacol. 180 Suppl 2:S289-373.