6. Orotic aciduria is characterized by an excess in orotic acid (at this point y

Question

6. Orotic aciduria is characterized by an excess in orotic acid (at this point you should get your notes and find the degradation pathway for purines). If you could give only one or two compounds to patients that suffer from orotic aciduria, which one would you chose? Justify your choice clearly. Note: if you google the answer you will learn zilch 7. Purine bases can be salvaged rather than synthesized de novo. What is the advantage of this metabolic strategy? Hint: Be specific; I would like to see numbers in your answer.

Explanation / Answer

Question 6.

Answer. Orotic aciduria is an autosomal recessive disease in which there is a severe deficiency in the activity of the de novo pyrimidine pathway enzyme uridine 5'-monophosphate (UMP). Hence people suffering from this disease can not synthesize Cytidine and Uridine. Orotic acid is an intermediate compound in pyrimidine synthesis pathway, which helps in conversion between dihydrofolate and tetrahydrofolate. Orotic aciduria results in megaloblastic anemia due to decreased pyrimidine synthesis, that can result in decreased nucleotide-lipid cofactors needed for erythrocyte membrane synthesis in the bone marrow.

Question 7.

Answer.  Salvage pathways are considerably more energyefficient than de novo pathways, which requires 6 moles of ATP for each mole of nucleotide produced.

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