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Transcript
Introduction: More and more researchers are discovering that many diseases are caused by
biochemical deficiencies or defects. That is, instead of indicating a disease is caused by a nutritional
deficiency, researchers recognize that an underlying cause may be a single molecular structure
within a cell that fails to turn on or turn off a cellular process. This is particularly true regarding
metabolism. For this task, you will consider the biochemical implications in a metabolic disorder
involving a normal component of food - fructose.
Requirements: Write a brief report (e.g., Microsoft Word) (suggested length of 3-4 pages) or
presentation (PowerPoint suggested length of 10-15 slides) that addresses the following:
A. To understand the issues behind hereditary fructose intolerance (HFI), address the following:
1) Explain how enzymes act as protein catalysts in biochemical processes (e.g., fructose
metabolism, glycolysis). Note: Describe at least two important features of all enzymes, and
then explaining the role of enzymes in the first two steps of fructose metabolism in the liver.
2) Explain how a deficiency in aldolase B is responsible for HFI. Note: In your explanation,
make sure to include the negative consequences of the substrate of Aldolase B, and how
this substrate leads to the symptoms of HFI.
3) Provide clearly labeled diagrams that demonstrate the following:
-The lock and key model or induced fit model of enzymatic activity. In your diagram, make
sure to label the entire enzymatic cycle, including product formation and release.
-The effect of enzymes on activation energy
Note: You may you use diagrams from the Internet or other resources to
demonstrate this concept; be sure to cite them in APA format.
4) Discuss the specific substrate acted on by Aldolase B during the metabolism of fructose.
5) Explain the role of aldolase B in the metabolism of fructose by discussing the products of
the reaction.
-Name the different pathways these products can enter.
B. Explore how mitochondrial disease can occur at multiple levels in different mitochondrial
processes by doing the following:
-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). Note: In your explanation, include
specific numbers of ATP generated/used in the different parts of the cycle to justify
your conclusion.
-Create an original dynamic diagram that shows why the citric acid cycle (CAC) is central to
aerobic metabolism and how it leads to ATP production. Note: This should include how
different metabolites enter the cycle, the entire CAC (including the names of all
intermediates, enzymes, and products), where and how of the products CAC make
ATP, and the role of oxygen in this process. A dynamic diagram is a clearly labeled
diagram that uses arrows to indicate movement and interactions.
-Explain where in the citric acid cycle a hypothetical defect of an enzyme could occur that
would decrease the overall ATP production of the mitochondria, including the consequences
of the defect. Note: This should also include the fate of the product of the chosen
enzyme, whether or not the entire cycle will continue to function, as well as the fate of
the cycle products and their contribution to ATP production. This is a hypothetical
defect, so the explanation should not include descriptions of any known disease.
C. Explain the specific role of coenzyme Q10 in ATP synthesis as part of the electron transport
chain. Note: In your explanation, provide a detailed description of the electron transport chain
and oxidative phosphorylation.
When you use sources, include all in-text citations and references in APA format. No more than a
combined total of 30% of a submission can be directly quoted or closely paraphrased from sources,
even if cited correctly.
Please have last slide as APA citation and include cited images on slides (besides created
diagrams) thank you.