Alkaptonuria results when an individual has no functional HGD enzyme. Without functional HGD, breakdown of excess tyrosine stops at homogentisate. The accumulated homogentisate is polymerized by the enzyme polyphenol oxidase to a complex set of molecules called “ochronotic pigment” that stain the patient’s urine and tissues dark brown or black. Through an unknown mechanism, it is believed that the accumulated ochronotic pigment leads to arthritis and other symptoms of alkaptonuria.

Question

Interestingly, none of the other intermediates in this pathway are toxic or lead to any observed disease symptoms.

The drug NTBC inhibits the enzyme 4-hydroxyphenyl pyruvate hydroxylase. NTBC has been used successfully to reduce the symptoms of alkaptonuria.

ii) In an alkaptonuria patient (one that consumes excess tyrosine) being treated with NTBC, which, if any, of the following compounds would accumulate?

4-hydroxy phenylpyruvate
4-maleyl-acetoacetate

iii) If a normal individual that consumes excess tyrosine were to take a therapeutic dose of NTBC, which, if any, of the following compounds would accumulate?

4-hydroxy phenylpyruvate
homogentisate

I believe it's a and a. Can somebody tell me for sure?

Answer 1

what is answer

Answer 2

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Answer 3

Ans ii)4-hydroxy phenylpyruvate iii)4-hydroxy phenylpyruvate