Confused and have questions? We’ve got answers. With Chegg Study, you can get step-by-step solutions to your questions from an expert in the field. If you rather get 1:1 study help, try 30 minutes of free online tutoring with Chegg Tutors.

Fructose intolerance

From Biology-Online Dictionary
Jump to: navigation, search

Fructose intolerance

An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (ec 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycaemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.