InterPro : IPR005961

Name  Phenylalanine-4-hydroxylase, tetrameric form Short Name  Phe-4-hydroxylase_tetra
Type  Family Description  This entry represents the large, tetrameric form of phenylalanine-4-hydroxylase (PAH; ), as found in metazoans. PAH irreversibly converts phenylalanine to tyrosine, and is known to be the rate-limiting step in phenylalanine catabolism in some systems. It is closely related to metazoan tyrosine 3-monooxygenase and tryptophan 5-monoxygenase, and more distantly to monomeric phenylalanine-4-hydroxylases of some Gram-negative bacteria. The member of this family from Drosophila has been described as having both phenylalanine-4-hydroxylase and tryptophan 5-monoxygenase activity []. However, a Drosophila member of the tryptophan 5-monoxygenase clade has subsequently been discovered.A deficiency in PAH can lead to phenylketonuria (PKU), an autosomal recessive genetic disorder characterised by an inability to metabolise phenylalanine, resulting in elevated Phe levels in the bloodstream that can lead to mental retardation if left untreated [].

Sequence Features

GO Displayer


InterPro protein domain ID --> Contigs



0 Child Features

3 Contains

Id Name Short Name Type
IPR019774 Aromatic amino acid hydroxylase, C-terminal Aromatic-AA_hydroxylase_C Domain
IPR018301 Aromatic amino acid hydroxylase, iron/copper binding site ArAA_hydroxylase_Fe/CU_BS Binding_site
IPR002912 ACT domain ACT_dom Domain

0 Found In

1 Parent Features

Id Name Short Name Type
IPR019773 Tyrosine 3-monooxygenase-like Tyrosine_3-monooxygenase-like Family

2 Publications

First Author Title Year Journal Volume Pages
Neckameyer WS A single locus encodes both phenylalanine hydroxylase and tryptophan hydroxylase activities in Drosophila. 1992 J Biol Chem 267 4199-206
Kim SW Structural and functional analyses of mutations of the human phenylalanine hydroxylase gene. 2006 Clin Chim Acta 365 279-87

To cite PlanMine, please refer to the following publication:

Rozanski, A., Moon, H., Brandl, H., Martín-Durán, J. M., Grohme, M., Hüttner, K., Bartscherer, K., Henry, I., & Rink, J. C.
PlanMine 3.0—improvements to a mineable resource of flatworm biology and biodiversity
Nucleic Acids Research, gky1070. doi:10.1093/nar/gky1070 (2018)