Gene Symbol: Khk
Description: ketohexokinase
Alias: ketohexokinase, hepatic fructokinase
Species: mouse
Products:     Khk

Top Publications

  1. Diggle C, Shires M, Leitch D, Brooke D, Carr I, Markham A, et al. Ketohexokinase: expression and localization of the principal fructose-metabolizing enzyme. J Histochem Cytochem. 2009;57:763-74 pubmed publisher
    b>Ketohexokinase (KHK, also known as fructokinase) initiates the pathway through which most dietary fructose is metabolized. Very little is known about the cellular localization of this enzyme...
  2. Ishimoto T, Lanaspa M, Le M, Garcia G, Diggle C, MacLean P, et al. Opposing effects of fructokinase C and A isoforms on fructose-induced metabolic syndrome in mice. Proc Natl Acad Sci U S A. 2012;109:4320-5 pubmed publisher
    ..These studies provide insights into the mechanisms by which fructose causes obesity and metabolic syndrome. ..
  3. Hayward B, Bonthron D. Structure and alternative splicing of the ketohexokinase gene. Eur J Biochem. 1998;257:85-91 pubmed
    b>Ketohexokinase (fructokinase, KHK) catalyses the phosphorylation of fructose to fructose-l-phosphate. It thereby initiates the intracellular catabolism of a large proportion of dietary carbohydrate...
  4. Weninger S, Peters L, Majzoub J. Urocortin expression in the Edinger-Westphal nucleus is up-regulated by stress and corticotropin-releasing hormone deficiency. Endocrinology. 2000;141:256-63 pubmed
  5. Hagopian K, Ramsey J, Weindruch R. Fructose metabolizing enzymes from mouse liver: influence of age and caloric restriction. Biochim Biophys Acta. 2005;1721:37-43 pubmed
    ..We show here that the enzymes involved in fructose metabolism are influenced by CR and that this could contribute to alterations in gluconeogenesis and glycolysis observed with CR. ..
  6. Diggle C, Shires M, McRae C, Crellin D, Fisher J, Carr I, et al. Both isoforms of ketohexokinase are dispensable for normal growth and development. Physiol Genomics. 2010;42A:235-43 pubmed publisher
    ..of this epidemiologic correlation are incompletely defined, but high-flux fructose catabolism initiated by ketohexokinase (Khk, fructokinase) is believed to be important...
  7. Song 宋志林 Z, Roncal Jimenez C, Lanaspa Garcia M, Oppelt S, Kuwabara M, Jensen T, et al. Role of fructose and fructokinase in acute dehydration-induced vasopressin gene expression and secretion in mice. J Neurophysiol. 2017;117:646-654 pubmed publisher
    ..Further studies of the aldose reductase-fructokinase system in vasopressin regulation appear indicated. ..
  8. Roncal Jimenez C, Ishimoto T, Lanaspa M, Milagres T, Hernando A, Jensen T, et al. Aging-associated renal disease in mice is fructokinase dependent. Am J Physiol Renal Physiol. 2016;311:F722-F730 pubmed publisher
  9. Marek G, Pannu V, Shanmugham P, Pancione B, Mascia D, Crosson S, et al. Adiponectin resistance and proinflammatory changes in the visceral adipose tissue induced by fructose consumption via ketohexokinase-dependent pathway. Diabetes. 2015;64:508-18 pubmed publisher
    ..In mammalian cells, fructose is metabolized predominantly via phosphorylation to fructose-1 phosphate by ketohexokinase (KHK) or by alternative pathways...

More Information


  1. Patel C, Douard V, Yu S, Gao N, Ferraris R. Transport, metabolism, and endosomal trafficking-dependent regulation of intestinal fructose absorption. FASEB J. 2015;29:4046-58 pubmed publisher
    ..member 5 (GLUT5) is the primary fructose transporter and that fructose absorption via GLUT5, metabolism via ketohexokinase (KHK), as well as GLUT5 trafficking to the apical membrane via the Ras-related protein-in-brain 11 (Rab11)a-..
  2. Patel C, Douard V, Yu S, Tharabenjasin P, Gao N, Ferraris R. Fructose-induced increases in expression of intestinal fructolytic and gluconeogenic genes are regulated by GLUT5 and KHK. Am J Physiol Regul Integr Comp Physiol. 2015;309:R499-509 pubmed publisher
    ..test the hypothesis that fructose absorption via glucose transporter protein, member 5 (GLUT5), metabolism via ketohexokinase (KHK), as well as GLUT5 trafficking to the apical membrane via the Ras-related protein in brain 11a (Rab11a)-..
  3. Kishida K, Pearce S, Yu S, Gao N, Ferraris R. Nutrient sensing by absorptive and secretory progenies of small intestinal stem cells. Am J Physiol Gastrointest Liver Physiol. 2017;312:G592-G605 pubmed publisher
  4. Wightman P, Hayward B, Bonthron D. The genes encoding glucokinase regulatory protein and ketohexokinase co-localize to mouse chromosome 5. Mamm Genome. 1997;8:700-1 pubmed
  5. Patel C, Sugimoto K, Douard V, Shah A, Inui H, Yamanouchi T, et al. Effect of dietary fructose on portal and systemic serum fructose levels in rats and in KHK-/- and GLUT5-/- mice. Am J Physiol Gastrointest Liver Physiol. 2015;309:G779-90 pubmed publisher
    ..We hypothesized that diet-induced changes in blood fructose concentrations are regulated by ketohexokinase (KHK) and the fructose transporter GLUT5...
  6. Mirtschink P, Krishnan J, Grimm F, Sarre A, Hörl M, Kayikci M, et al. HIF-driven SF3B1 induces KHK-C to enforce fructolysis and heart disease. Nature. 2015;522:444-449 pubmed publisher
    ..The central fructose-metabolising enzyme is ketohexokinase (KHK), which exists in two isoforms: KHK-A and KHK-C, generated through mutually exclusive alternative ..
  7. Lanaspa M, Ishimoto T, Cicerchi C, Tamura Y, Roncal Jimenez C, Chen W, et al. Endogenous fructose production and fructokinase activation mediate renal injury in diabetic nephropathy. J Am Soc Nephrol. 2014;25:2526-38 pubmed publisher
    ..sorbitol can be converted to fructose, which is then metabolized largely by fructokinase, also known as ketohexokinase, leading to ATP depletion, proinflammatory cytokine expression, and oxidative stress...
  8. Roncal Jimenez C, Ishimoto T, Lanaspa M, Rivard C, Nakagawa T, Ejaz A, et al. Fructokinase activity mediates dehydration-induced renal injury. Kidney Int. 2014;86:294-302 pubmed publisher
    ..Access to sufficient water during the dehydration period can protect mice from developing renal injury. These studies provide a potential mechanism for Mesoamerican nephropathy. ..
  9. Ishimoto T, Lanaspa M, Rivard C, Roncal Jimenez C, Orlicky D, Cicerchi C, et al. High-fat and high-sucrose (western) diet induces steatohepatitis that is dependent on fructokinase. Hepatology. 2013;58:1632-43 pubmed publisher
    ..These studies emphasize the important role of fructose in the development of fatty liver and nonalcoholic steatohepatitis. ..