Fructokinase
Fructokinase is an enzyme that facilitates the phosphorylation of fructose to fructose 1-phosphate. This enzyme is a key player in the metabolism of fructose, particularly in the liver and kidney.
Function[edit | edit source]
Fructokinase catalyzes the first step in the metabolism of dietary fructose. It converts fructose, a monosaccharide, into fructose 1-phosphate. This reaction consumes one molecule of adenosine triphosphate (ATP), producing adenosine diphosphate (ADP).
Clinical significance[edit | edit source]
Deficiency in fructokinase leads to a benign condition known as essential fructosuria. This is characterized by the excretion of unmetabolized fructose in the urine. Because fructokinase is not available to metabolize fructose, the fructose is not converted into fructose 1-phosphate and instead is excreted in the urine.
In contrast, an overactivity of fructokinase can lead to hereditary fructose intolerance, a potentially fatal condition if left untreated. This is due to the accumulation of fructose 1-phosphate in the body, which can lead to liver and kidney damage.
See also[edit | edit source]
References[edit | edit source]
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