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Phenylalanine 4-hydroxylase (PAH) is an enzyme that catalyzes the conversion of L-phenylalanine to L-tyrosine. This reaction is a critical step in phenylalanine catabolism and is essential for preventing toxic accumulation of phenylalanine in the body. Deficiency or dysfunction in PAH leads to hyperphenylalaninemia or classic phenylketonuria (PKU).
Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine, requiring tetrahydrobiopterin (BH₄) and molecular oxygen.
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