A 5 year old child was brought to the physician with a history of black urine . There is no history of fever or any other complaints. There is no growth retardation and all the developmental milestones are normal. The child is suspected to have an enzyme defect for metabolism of an aromatic amino acid. What is the enzyme deficient ?

A. Homogentistae oxidase
B. Homogentisate dehydrogenase
C. Tryptophan Hydroxylase
D. Tyrosine Transaminase

Answer – A: Homogentistae oxidase

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