Biochem Task 4

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Biochemistry Task 4
GRT1 208.5.4-01, 03-05, 5.5-02, 04-07
Western Governors University

Biochemistry Task 4
GRT1 208.5.4-01, 03-05, 5.5-02, 04-07 A. Case 1: Hereditary Fructose Intolerance
A1. Role of Enzymes in Processes
Enzymes are proteins that carry out chemical reactions. They bind to substrates, which are basically substances that need to be broken down and changed into something else. When the enzyme and substrate bind, they form the enzyme-substrate complex. An enzyme will act in a specific way on the substrate that it is bound to in order to change it into a product, and at the end of the process, the enzyme is unchanged and ready to bind to the next substrate. An enzyme acts as a catalyst, something that lowers the energy required to complete a chemical reaction (activation energy) without itself being changed. (Hudon-Miller, 2012) In the case of fructose breakdown, an enzyme called fructokinase is responsible for splitting fructose into fructose 1-phosphate, a six-carbon fructose. Another enzyme called aldolase B splits fructose 1-phosphate into two three-carbon molecules, dihydroxyacetone phosphate (DHAP) and glyceraldehyde. These products are then able to enter the glycolysis pathway to be converted to pyruvate, which is essential for the citric acid cycle and the production of adenosine triphosphate (ATP) for cellular energy.
A2. Deficiency in Aldolase B
A hereditary deficiency in aldolase B could be caused by mutations in the ALDOB gene. An aldolase B deficiency will prevent the breakdown of fructose past the point of the fructose 1-phosphate stage. This causes fructose-1-phosphate to build up in the liver, and the depletion of phosphates that are needed for ATP production. Therefore, the synthesis of glucose (gluconeogenesis) cannot happen. Furthermore, glycogenolysis (the breakdown of glycogen into glucose) is prevented. If a person…...

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