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EC Number
Natural Substrates
Commentary (Nat. Sub.)
4.2.1.96
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both PhhB and phenylalanine hydroxylase (PhhA) are induced coordinately in the presence of either L-tyrosine or L-phenylalanine, but PhhB exhibits a significant basal level of activity that is lacking for PhhA. PhhA and PhhB form a protein-protein complex
4.2.1.96
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dehydratase/DCoHalpha, has the kinetic properties necessary for regenerating tetrahydrobiopterin cofactor for phenylalanine hydroxylase. Properties of dehydratase/DCoHalpha are consistent with the hypothesis that the activity of this isozyme could account for the relatively mild symptoms reported for patients with a defect in dehydratase/DCoH
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