| Type: | Domain | Name: | Aromatic amino acid hydroxylase, C-terminal |
| Description: | Phenylalanine, tyrosine and tryptophan hydroxylases constitute a family of tetrahydrobiopterin-dependent aromatic amino acid hydroxylases, all of which are rate-limiting catalysts for important metabolic pathways []. The proteins are structurally and functionally related, each containing iron, and catalysing ring hydroxylation of aromatic amino acids, using tetra-hydrobiopterin (BH4) as a substrate. All are regulated by phosphorylation at serines in their N-termini. It has been suggested that the proteins each contain a conserved C-terminal catalytic (C) domain and an unrelated N-terminal regulatory (R) domain. It is possible that the R domains arose from genes that were recruited from different sources to combine with the common gene for the catalytic core. Thus, by combining with the same C domain, the proteins acquired the unique regulatory properties of the separate R domains.A variety of enzymes belong to this family that includes, phenylalanine-4-hydroxylase from Chromobacterium violaceumwhere it is copper-dependent; it is iron-dependent in Pseudomonas aeruginosa, phenylalanine-4-hydroxylase catalyzes the conversion of phenylalanine to tyrosine. In humans, deficiencies are the cause of phenylketonuria, the most common inborn error of amino acid metabolism [], tryptophan 5-hydroxylase catalyzes the rate-limiting step in serotonin biosynthesis: the conversion of tryptophan to 3-hydroxy-anthranilate and tyrosine 3-hydroxylase catalyzes the rate limiting step in catecholamine biosynthesis: the conversion of tyrosine to 3,4-dihydroxy-L-phenylalanine. | Short Name: | Aromatic-AA_hydroxylase_C |
| DB identifier | Type | Name |
|---|---|---|
| IPR018301 | Binding_site | Aromatic amino acid hydroxylase, iron/copper binding site |
| DB identifier | Type | Name |
|---|---|---|
| IPR001273 | Family | Aromatic amino acid hydroxylase |
| IPR005961 | Family | Phenylalanine-4-hydroxylase, tetrameric form |
| IPR005963 | Family | Tryptophan 5-monooxygenase |
| IPR019773 | Family | Tyrosine 3-monooxygenase-like |
| IPR005962 | Family | Tyrosine 3-monooxygenase |
| IPR005960 | Family | Phenylalanine-4-hydroxylase, monomeric form |
