Anti-Ketohexokinase Isoform C Antibody, clone 5F1-3F5 clone 5F1-3F5, from mouse

Ketohexokinase (UniProt: P50053; also known as EC: 2.7.1.3, Hepatic fructokinase) is encoded by the KHK gene (Gene ID: 3795) in human. Ketohexokinase is a homodimeric cytosolic enzyme in that catalyzes the conversion of fructose to fructose 1 phosphate. It is most abundant in the liver, where it may constitute up to 0.6% of the total liver protein. It has also been reported in kidney, spleen, gut, and pancreas. Very low levels are also found in adrenal, muscle, brain, and eyes. It has multiple fructose binding sites. There are two major isoforms of this enzyme ketofructokinase C and ketofructokinase A. Due to its high Km (~ 28 mM), fructose is preferentially metabolized by the C isoform. Ketohexokinase -C is unique among sugar kinases in that its metabolism of fructose is associated with a rapid depletion of intracellular ATP. Unlike glucokinase, in which excessive phosphorylation of glucose is prevented by a negative feedback system, the metabolism of fructose by ketohexokinase-C results in rapid phosphorylation with a fall in intracellular phosphate and ATP. If the ATP levels fall significantly then the protein synthesis is transiently stopped and cells may develop features that are consistent with ischemia. Mutations in KHK gene has been linked to fructosuria, a clinically benign condition that is characterized nu the incomplete metabolism of fructose in the liver, leading to its urinary excretion. (Ref.: Diggle, CP et al., (2009). J. Histochem. Cytochem. 57(8): 763-774; Hayward, BE., and Bonthron, DT (1998). Eur. J. Biochem. 257(1); 85-91).