Hereditary Fructose Lab Report

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Hereditary Fructose Intolerance and Mitochondrial Disease Sara James Western Governor's University Hereditary Fructose Intolerance Enzymes in Breakdown of Fructose Enzymes are proteins that carry out chemical reactions. They will bind to a substrate and then end up releasing a product. The enzymes do it by a process of lock and key. The lock is considered the substrate and the enzyme is considered the key. Only the enzyme will fix the substrate because of the active sites on the enzyme. Once locked together, the enzyme can break down the substrate (Hudon-Miller, 2012c). An example of this is Sucrose. Sucrose is considered a regular table sugar. It is composed of two monosaccharaides: glucose and fructose. When fructose is broken down,…show more content…
The specific substrate in this case that is acted by Aldolase B is fructose-1-phosphate. This is later converted into DHAP and glyceraldehyde. The product, once finished, will then enter the glycolysis cycle to form ATP (Hudon-Miller, 2012c). Role of Aldolase B in the Breakdown of Fructose Aldolase B is found mostly in the liver but can also be present at lower levels in the kidney and intestinal cells. It is involved in breaking down a simple sugar called fructose. This is mostly found in fruits and can be used in the body for energy. Aldolase B is responsible in the second step of the metabolism of fructose. At this stage, the enzyme will break down the molecule fructose-1-phosphate into DHAP (dihydrozyacteone phosphate) and glyceraldehyde. Without aldolase B, this cannot be done (Hudon-Miller, 2012c) Mitochondrial Disease Cori Cycle If the amount of energy available to a cell would remain in that single cell during a Cori cycle, there would not be enough energy to convert the lactate back to glucose. Normally during the cycle, glucose is converted to lactate in the muscles. This will make two ATP. Lactate is then transported to the liver and converted back to glucose. This will require the liver to use six ATP. If this was all done in a single cell, the energy made is not enough to cover what is needed to convert it back to glucose. This will put the amount of energy in the negative range (Hudon-Miller,

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