Phenylalanine Hydroxylase
Also known as: PAH; Phenylalanine 4-Monooxygenase; Phenylalaninase; Phenylalanine Carboxylase; Phenylalanine Monooxygenase
Description
Phenylalanine Hydroxylase is a type of Hydroxylase Enzyme. Its main site of activity is the Liver, although small quantities are also present in Fibroblasts and the Brain.
Biological Function of Phenylalanine Hydroxylase
Amino Acids
Phenylalanine Hydroxylase catalyzes the endogenous conversion of Phenylalanine to Tyrosine (primarily in the Liver). [1]
These Substances may Enhance the Function of Phenylalanine Hydroxylase
Coenzymes
Tetrahydrobiopterin is an essential cofactor for the activation of Phenylalanine Hydroxylase.
Minerals
Iron is an essential cofactor for Phenylalanine Hydroxylase.
Vitamins
Folic Acid (after its conversion to Tetrahydrobiopterin) is an essential cofactor for Phenylalanine Hydroxylase. [2]
These Ailments Interfere with Phenylalanine Hydroxylase
Nutrient Metabolism Ailments
Phenylketonuria (PKU) prevents the manufacture of Phenylalanine Hydroxylase within the body.
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