HFI: Hereditary Fructose Intolerance

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HFI (Hereditary Fructose Intolerance) is a disorder related to the enzyme fructose-1-phosphate aldolase, or aldolase B. It is characterized by a deficiency of this enzyme. As previously discussed, this enzyme is utilized in the second step of metabolizing fructose in the liver. Furthermore, a deficiency of this enzyme will subsequently create an abundance of the products from step one of fructose metabolism as well an overall loss of atp. The significance here is that the net loss of atp and the loss of the phosphate group, which is still attached to fructose from step one, create an unfavorable environment for other reactions. This ultimately becomes responsible for the symptoms related to the disorder, like hypoglycemia. Hypoglycemia, or

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