Reactome | KHK dimer phosphorylates Fru to Fru 1-P

KHK dimer phosphorylates Fru to Fru 1-P

Stable Identifier

R-HSA-70333

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

ATP + beta-D-fructose => ADP + beta-D-fructose 1-phosphate + H(+), Ketohexokinase (KHK) phosphorylates beta-D-fructose to form fructose 1-phosphate, ATP + beta-D-fructose => ADP + D-fructose 1-phosphate

ReviewStatus

5/5

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Cytosolic ketohexokinase (KHK, also known as fructokinase) catalyzes the reaction of D-fructose (Fru) and ATP to form D-fructose 1-phosphate (Fru 1-P) and ADP. Two isoforms of the enzyme, A and C, are encoded by alternatively spliced forms of the gene; both form catalytically active dimers. The C isoform is predominant in liver and kidney tissues, has high affinity for fructose, and is probably responsible for the bulk of fructose phosphorylation in vivo (Asipu et al. 2003; Trinh et al. 2009). The A isoform is found in lower levels in many other tissues and may serve a role in fructose metabolism outside of liver and kidney (Funari et al. 2005). The physiological role of KHK has been established from metabolic and DNA sequencing studies of patients with essential fructosuria (Bonthron et al. 1994) and in mouse models for this disease (Diggle et al. 2010; Ishimoto et al. 2012).

Literature References

PubMed ID	Title	Journal	Year
22371574	Opposing effects of fructokinase C and A isoforms on fructose-induced metabolic syndrome in mice Maruyama, S, Diggle, CP, Ishimoto, T, Asipu, A, Kosugi, T, Bonthron, DT, Roncal-Jimenez, CA, Jackman, MR, Sautin, YY, Sánchez-Lozada, LG, Rodriguez-Iturbe, B, Garcia, GE, Rivard, CJ, Maclean, PS, Johnson, RJ, Le, MT, Lanaspa, MA	Proc. Natl. Acad. Sci. U.S.A.	2012
12941785	Properties of normal and mutant recombinant human ketohexokinases and implications for the pathogenesis of essential fructosuria Hayward, BE, O'Reilly, J, Asipu, A, Bonthron, DT	Diabetes	2003
19237742	Structures of alternatively spliced isoforms of human ketohexokinase Trinh, CH, Phillips, SE, Asipu, A, Bonthron, DT	Acta Crystallogr D Biol Crystallogr	2009
16266770	Genes required for fructose metabolism are expressed in Purkinje cells in the cerebellum Freeman, D, Funari, VA, Tolan, DR, Herrera, VL	Brain Res. Mol. Brain Res.	2005
20841500	Both isoforms of ketohexokinase are dispensable for normal growth and development McRae, C, Markham, AF, Hayward, BE, Crellin, D, Diggle, CP, Fisher, J, Asipu, A, Carr, IM, Shires, M, Bonthron, DT	Physiol. Genomics	2010
7833921	Molecular basis of essential fructosuria: molecular cloning and mutational analysis of human ketohexokinase (fructokinase) Donaldson, IA, Brady, N, Steinmann, B, Bonthron, DT	Hum Mol Genet	1994