Question

3. (36 points total) A doctor from Cairo, Egypt is perplexed by two twin infants that he is treating for deficiencies in energy production. He has many theories on what could be the issue, but he wants you to help. He presented their case to your lab. You sequence these infants DNA and try to uncover what metabolic problem they may have that is causing this energy-deficient illness they have. A. (10 points) You characterize the enzymes for glycolysis, PDH, and TCA cycle and everything seems fine, except for one thing: you discover that these infants have a very strange pathway never discovered before. This pathway takes oxaloacetate from the body and branches it into a pathway that breaks it in down into seemingly non-biologically relevant intermediates. Why could this explain the energy deficiency the Egyptian doctor discovered? (50 words max). ANSWER: B. (10 points) You discover that the enzyme that controls this weird oxaloacetate pathway is very similar to Pyruvate Dehydrogenase. Additionally, this pathway can be regulated by reversible covalent modification very similar to that of PDH. These infants have a problem with this regulation, but if this regulation was normal, how could you regulate this weird pathway to solve their problem? Make sure you address ALL possible ways you can regulate, with the knowledge that this is very similar to PDH. (50 words max) ANSWER:

Answer 1

3.a) As oxaloacetate and acetyl co A becomes attached and formed citrate by citrate synthase and that finally make many intermediate and produces energy by making ATP FADH2 AND NADH2 .

BUT WHEN THIS oxaloacetate is being used and making a new intermediates which is never biological significance. Then the pathway will not produces any energy through TCA cycle.

But the glucose and many other food components break down product like pyruvate succcinyl COa will be used up.

B)as kinases inhibit the PDH complex and in the patient this weird pathway is regulated by reversible of the PDH regulation, then phosphatases enzyme will inhibit the activity of this pathway.

And kinase will stimulate this pathway so

Components like

NADH, ACETYL COA AND INSULIN CAN CONTROL THIS PATHWAY.

Thank you