A. Demonstrate your understanding of the biochemical basis of hereditary fructose intolerance (HFI) by doing the following:
1. Describe two important features that make all enzymes catalysts.
2. Explain how enzymes act as protein catalysts in the first two steps of fructose metabolism in the liver. 4. Discuss the specific substrate acted on by aldolase B during the metabolism of fructose, including how the substrate is made.
5. Explain the role of aldolase B in the metabolism of fructose, including the products of the reaction.
a. Identify the different pathways the products of aldolase B can enter.
6. 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 the symptoms of HFI. 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 (glucose going to lactate and then back to glucose) were to occur and remain within that single cell (i.e., a muscle cell).
a. Justify your response to part B1 by providing specific numbers of ATP generated or used in the different parts of the cycle.3. Explain where in the CAC a hypothetical defect of an enzyme could occur that would decrease the overall ATP production of the mitochondriaa. Discuss what happens to the product of the enzyme from part B3, that includes each of the following points:
• whether the entire cycle will continue to function
• what will happen to the cycle products and their contribution to ATP production 4. Explain the specific role of coenzyme Q10 in the electron transport chain.
a. Explain how coenzyme Q10 leads to ATP synthesis.
i. Describe the electron transport chain and oxidative phosphorylation.
C. When you use sources, include all in-text citations and references in APA format.
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