Hereditary Fructose Intolerance

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Hereditary Fructose Intolerance Enzymes are involved in processes such as the breakdown of fructose because they speed the process up. These reactions would be a lot slower without them or may not happen at all. They act as a catalyst. Fructose is used as a source of energy for our bodies. Enzymes are needed to breakdown this sugar into energy. The enzyme fructokinase is the one that breaks this sugar down into fructose 1 phosphate. The next step is done by an enzyme called adolase B and this step produces DHAP and glyceraldehyde. Then these two products go through a process called glycolysis. Without these enzymes, our bodies cannot breakdown fructose into usable energy. A deficiency in aldolase B can be responsible for hereditary fructose intolerance by not allowing the second step of the process to happen. Hereditary fructose intolerance is a hereditary disorder caused by a genetic mutation. Because the enzyme adolase B is not functioning, the fructose 1 phosphate builds up in the liver and causes sequestration of F1P. This will lead to degradation of adenine nucleotides. This, then leads to concomitant hypoglycemia. This can lead to liver damage. The specific substrate acted on by adolase B is F1P (Fructose 1 phosphate). When the aldolase B is not functioning, we cannot process F1P, which causes an accumulation of F1P in the liver. The whole process is stopped and glycolysis cannot occur. The fructose will not be broken down for a source of energy. The role of aldolase B in the breakdown of fructose is in the second step. Aldolase B catalyzes F1P breakdown into glyceraldehyde and DHAP. These will become either glucose or pyruvate. Pyruvate is needed for the citric acid cycle to work. Without aldolase B, the body cannot process F1P. This will lead to an accumulation in body tissues.

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