Ketohexokinase
Function
Catalyzes the phosphorylation of the ketose sugar fructose to fructose-1-phosphate.
Involvement in disease
Fructosuria
FRUCT
Benign defect of intermediary metabolism.
None
The disease is caused by variants affecting the gene represented in this entry.
Pathway
Carbohydrate metabolism; fructose metabolism.
Sequence Similarities
Belongs to the carbohydrate kinase PfkB family.
Tissue Specificity
Most abundant in liver, kidney, gut, spleen and pancreas. Low levels also found in adrenal, muscle, brain and eye.
Alternative names
Ketohexokinase, Hepatic fructokinase, KHK