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phenylalanine 4-monooxygenase


Definition: an enzyme that catalyzes oxidation of l-phenylalanine to l-tyrosine with O2 and tetrahydrobiopterin (the latter forming the dihydro derivative) which is, in turn, reduced by NADPH and dihydropteridine reductase to the active form); a deficiency of either phenylalanine 4-monooxyenase or dihydropteridine reductase will result in phenylketonuria.

Synonym(s): phenylalanine 4-hydroxylase

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.