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The metabolism of phenylalanine will now be taken in some detail, as two inborn errors of metabolism are known which affect this pathway. The Phenylalanine is 1st hydroxylated by phenylalanine hydroxylase to form another aromatic amino acid tyrosine. The coenzyme for this reaction is the reductant tetrahydrobiopterin that is oxidized to dihydrobiopterin.
Figure: The metabolism of phenylalanine
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