A. Demonstrate your understanding of the biochemical basis of hereditary fructose intolerance (HFI) by doing the following: 1. Insert your description of two important features that make all enzymes catalysts here. (Insert in-text citation here). 2. Insert your explanation of how enzymes act as protein catalysts in the first two steps of fructose metabolism in the liver. (Insert in-text citation here). 3. Provide an original, clearly labeled diagram, or series of diagrams, that illustrates the following: Insert the lock and key model OR the induced fit model of enzymatic activity here. (Insert in-text citation here). Note: The diagram must show the entire enzymatic cycle, including product formation and release. Click here to learn how to insert images into a Google Document. Insert the activation energy of a reaction in the presence and absence of an enzyme. (Insert in-text citation here). 4. Insert your discussion of the specific substrate acted on by aldolase B during the metabolism of fructose, including how the substrate is made. (Insert in-text citation here). 5. Insert your explanation of the role of aldolase B in the metabolism of fructose, including the products of the reaction here. a. Identify the different pathways the products of aldolase B can enter here. (Insert in-text citation here). 6. Discuss how a deficiency in aldolase B is responsible for HFI by doing the following: a. Insert your explanation of how the amount of the substrate of aldolase B is impacted by the deficiency. b. Insert your explanation of the role of the substrate in producing the symptoms of HFI. (Insert in-text citation here). B. Explore how mitochondrial disease can occur at multiple levels in different mitochondrial processes by doing the following: 1. Insert your explanation of 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. (Insert in-text citation here). 2. Insert your original dynamic diagram that shows how the citric acid cycle (CAC) is central to aerobic metabolism here. a. Be sure your diagram includes the following elements: how different metabolites enter the cycle the entire CAC, including the names of all intermediates, enzymes, and products where the products of the CAC go in order to make ATP the role of oxygen in this process (Insert in-text citation here). 3. Insert your explanation of where in the CAC a hypothetical defect of an enzyme could occur that would decrease the overall ATP production of the mitochondria. (Insert in-text citation here). a. Insert your discussion of 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 (Insert in-text citation here). 4. Insert your explanation of the specific role of coenzyme Q10 in the electron transport chain here. a. Insert your explanation of how coenzyme Q10 leads to ATP synthesis here. i. Insert your description of the electron transport chain and oxidative phosphorylation here. (Insert in-text citation here).

A. Demonstrate your understanding of the biochemical basis of hereditary fructose intolerance (HFI) by doing the following:

1. Insert your description of two important features that make all enzymes catalysts here.



(Insert in-text citation here).


2. Insert your explanation of how enzymes act as protein catalysts in the first two steps of fructose metabolism in the liver.

(Insert in-text citation here).


3. Provide an original, clearly labeled diagram, or series of diagrams, that illustrates the following:


Insert the lock and key model OR the induced fit model of enzymatic activity here.



(Insert in-text citation here).


Note: The diagram must show the entire enzymatic cycle, including product formation and release.


Click here to learn how to insert images into a Google Document.




Insert the activation energy of a reaction in the presence and absence of an enzyme.



(Insert in-text citation here).


4. Insert your discussion of the specific substrate acted on by aldolase B during the metabolism of fructose, including how the substrate is made.


(Insert in-text citation here).


5. Insert your explanation of the role of aldolase B in the metabolism of fructose, including the products of the reaction here.


a. Identify the different pathways the products of aldolase B can enter here.


(Insert in-text citation here).


6. Discuss how a deficiency in aldolase B is responsible for HFI by doing the following:


a. Insert your explanation of how the amount of the substrate of aldolase B is impacted by the deficiency.


b. Insert your explanation of the role of the substrate in producing the symptoms of HFI.



(Insert in-text citation here).



B. Explore how mitochondrial disease can occur at multiple levels in different mitochondrial processes by doing the following:

1. Insert your explanation of 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.


(Insert in-text citation here).


2. Insert your original dynamic diagram that shows how the citric acid cycle (CAC) is central to aerobic metabolism here.

a. Be sure your diagram includes the following elements:

how different metabolites enter the cycle

the entire CAC, including the names of all intermediates, enzymes, and products

where the products of the CAC go in order to make ATP

the role of oxygen in this process



(Insert in-text citation here).


3. Insert your explanation of where in the CAC a hypothetical defect of an enzyme could occur that would decrease the overall ATP production of the mitochondria.



(Insert in-text citation here).


a. Insert your discussion of 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


(Insert in-text citation here).


4. Insert your explanation of the specific role of coenzyme Q10 in the electron transport chain here.


a. Insert your explanation of how coenzyme Q10 leads to ATP synthesis here.

i. Insert your description of the electron transport chain and oxidative phosphorylation here.


(Insert in-text citation here).