Hereditary fructose intolerance

Fructose intolerance is a hereditary condition due to a deficiency of liver enzymes that metabolise fructose. Not to be confused with fructose malabsorption, a deficiency of fructose transporter enzyme in the enterocytes, which leads to abdominal bloating and diarrhea. the deficient enzyme is Fructose-1-phosphate aldolase, this means that the fructose cannot be further metabolised beyond fructose-1-phosphate. This traps phosphates; which are needed to phosphorolyse glycogen phosphorolase to carry on to make glucose. therefore glucose cannot be made through the breakdown of glycogen nor from gluconeogenesis, resulting in severe hypoglycaemia. If fructose is ingested, vomiting, hypoglycaemia and evetually kidney failure will follow. more...

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