Title : Specific interaction of the diastereomers 7(R)- and 7(S)-tetrahydrobiopterin with phenylalanine hydroxylase: implications for understanding primapterinuria and vitiligo.

Pub. Date : 2006 Oct

PMID : 16935936






1 Functional Relationships(s)
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1 Mutations in PCD or its deactivation by hydrogen peroxide result in the generation of 7(R,S)BH4, which is a potent inhibitor of PAH that has been implicated in primapterinuria, a variant form of phenylketonuria, and in the skin depigmentation disorder vitiligo. Hydrogen Peroxide phenylalanine hydroxylase Homo sapiens