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1. XXXXXXXXXXXXXXXXXXXA doctor from Cairo, Egypt is perplexed bytwo twin infants that he is treating for deficiencies in energyproduction.   He has many theories on what could be theissue, but he wants you to help. He presented their case to yourlab. You sequence these infants DNA and try to uncover whatmetabolic problem they may have that is causing thisenergy-deficient illness they have.

1. (10 points) You characterize the enzymes for glycolysis, PDH,and TCA cycle and everything seems fine, except for one thing: youdiscover that these infants have a very strange pathway neverdiscovered before. This pathway takes oxaloacetate from the bodyand branches it into a pathway that breaks it in down intoseemingly non-biologically relevant intermediates. Why could thisexplain the energy deficiency the Egyptian doctor discovered? (50words max).

ANSWER:

                                                  

1. (10 points) You discover that the enzyme that controls thisweird oxaloacetate pathway is very similar to PyruvateDehydrogenase. Additionally, this pathway can be regulated byreversible covalent modification very similar to that of PDH. Theseinfants have a problem with this regulation, but if this regulationwas normal, how could you regulate this weird pathway to solvetheir problem? Make sure you address ALL possibleways you can regulate, with the knowledge that this is very similarto PDH. (50 words max)

ANSWER:

1. (8 points) You also discover another problem in the infants: akey enzyme responsible for the production of Coenzyme Q (i.e.Ubiquinone) is hugely mutated to work very, veryslowly.   This causes very low levels of Coenzyme Q inthe infants. Why could this also explain their energy-productionproblem? (40 words max).

ANSWER:

1. (8 points) You email the doctor and he asks if it is possibleto just add Coenzyme Q in the diet if the infant twins. Why maythis work, in theory, but why might this be a problem to actuallydo in the body? Consider the structure of Coenzyme Q and where itis located in the mitochondria? (30 words max).

ANSWER: