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Hereditary fructose intolerance (HFI) or fructose poisoning is a hereditary condition caused by a deficiency of liver enzymes that metabolise fructose. It is also known as hereditary fructosemia, or fructose in the blood (-emia means in the blood).

The deficient enzyme is aldolase-B, which converts fructose-1-phosphate to DHAP and glyceraldehyde. This means that the fructose cannot be further metabolised beyond fructose-1-phosphate. This traps phosphates; which are needed to phosphorylate glycogen phosphorylase which carries on to release units of glucose-1-phosphate from glycogen. (Glucose-1-phosphate gets converted to glucose-6-phosphate and then dephosphorylated to form glucose).

In addition, Aldolase A plays an important role in gluconeogenesis, producing fructose-1,6-bisphosphate from glyceraldehyde-3-phosphate and DHAP. But, glucose may still be released through the breakdown of glycogen. Although, it cannot be synthesized from gluconeogenesis, resulting in severe hypoglycaemia.


Clinical Research on Fructotab
 
Fructotab is clinically proven to treat Hereditary Fructose Intolerance successfully. You may download a summary of our clinical study in Adobe's PDF format. Click here for details.
 
 

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