Tetrahydrobiopterin or BH4 is a cofactor in the synthesis of nitric oxide. It is also important in the conversion of phenylalanine to tyrosine by the enzyme phenylalanine-4-hydroxylase. A defect in BH4 production and/or a defect in the enzyme dihydropteridine reductase (DHPR) causes phenylketonuria type IV.
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