Protein Domain : IPR005961

Type:  Family Name:  Phenylalanine-4-hydroxylase, tetrameric form
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 []. Short Name:  Phe-4-hydroxylase_tetra
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0 Child Features

3 Contains

DB identifier Type Name
IPR002912 Domain ACT domain
IPR019774 Domain Aromatic amino acid hydroxylase, C-terminal
IPR018301 Binding_site Aromatic amino acid hydroxylase, iron/copper binding site

1 Cross References

Identifier
TIGR01268

0 Found In

3 GO Annotations

GO Term Gene Name
GO:0004505 IPR005961
GO:0006559 IPR005961
GO:0055114 IPR005961

3 Ontology Annotations

GO Term Gene Name
GO:0004505 IPR005961
GO:0006559 IPR005961
GO:0055114 IPR005961

1 Parent Features

DB identifier Type Name
IPR019773 Family Tyrosine 3-monooxygenase-like

0 Proteins

2 Publications

First Author Title Year Journal Volume Pages PubMed ID
            1371286
            16253218