This disorder is inherited in an autosomal recessive pattern.
Essential fructosuria is caused by a deficiency of the enzyme fructokinase which catalyzes the reaction fructose to fructose-1-phosphate.
Fructokinase is the first enzyme involved in the degradation of fructose to fructose-1-phosphate.
Unlike other defects in fructose metabolism, essential fructosuria causes a clinically benign condition.
Although fructose accumulates in blood and urine, it cannot enter cells and therefore causes a clinically benign condition. Fructose is excreted through the urine, causing a positive test for reducing substances.
Although fructose accumulates in blood and urine, it cannot enter cells and therefore causes a clinically benign condition.
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