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Health, Medicine, Nursing
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Biochemistry Research Assignment: Fructose Metabolism

Essay Instructions:

Explain the reactions catalyzed by enzymes in the first two steps of fructose metabolism in the liver, including each of the following:
-the substrates acted on
-the enzyme catalysts
-the products generated
5. Discuss how a deficiency in aldolase B is responsible for HFI by doing the following:
a. Explain how the amount of the substrate of aldolase B is impacted by the deficiency.
b. Explain the role of the substrate in producing one condition of HFI (e.g., hypoglycemia, liver failure).
B. Explore how mitochondrial disease can occur at multiple levels in different mitochondrial processes by doing the following:
1. Explain what would hypothetically happen to the amount of ATP available to a cell if the entire Cori cycle were to occur and remain within a single cell (e.g., a muscle cell).
a. Identify specific numbers of ATP generated and used in the different parts of the cycle, in conjunction with your response from part B1.Explain where in the CAC a hypothetical defect of an enzyme could occur that would decrease the overall ATP production of the mitochondria, including each of the following:
-whether the entire cycle will continue to function
-what will happen to the cycle products
-why ATP production decreases
Note: This is a hypothetical defect, so the explanation should not include descriptions of any known disease.
4. Explain how a proton gradient is formed and used to make ATP during aerobic metabolism.
Note: The inclusion of a discussion of the electron transport chain and oxidative phosphorylation, including the role of oxygen, aids in the explanation of this process.

Essay Sample Content Preview:

Fructose metabolism
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The first two reactions in the liver take place when enzyme fructokinase converts fructose to fructose 1-phosphate. The substrate being acted upon is fructose, and the enzyme catalyst is fructokinase. ATP is produced by substrate level phosphorylation when fructose 1phosphate is transferred to ADP. The reaction generates fructose 1-phosphate which is then converted to the trioses dihydroxyacetone phosphate and glyceraldehyde in the presence of enzyme catalyst aldolase B.
Aldolase B deficiency is responsible for inability metabolize ingested fructose thus creating hereditary fructose intolerance (HFI). The absence of aldolase B activity increases the level of fructose one phosphate. This causes the inhibition of phosphorylase that prevents glycogenolysis. Glucose formation is further inhibited by fructose one phosphate inhibition of glucose one phosphate in the presence of catalyst phosphoglucomutase (Watson, 2014). As a result, hypoglycemia is seen any time fructose is ingested. Without aldolase B,the body struggles to transform F1P into DHAP and glyceraldehyde, which are vital ingredients in the formation of ATP.
HFI is mainly caused by the below optimum performance or levels of aldolase B,an enzyme that is found in the liver. The enzyme ,as initially noted, plays a vital role in breaking downfructose-1-phosphate into dihydroacetone and glyceraldehyde. Conditions such as hypoglycaemia are as a result of malfunctioning of the liver,which fails in controlling the breakdown of the sugar.
Cori cycle is a me...
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