CATH Domain 2phmA00

CATH Classification

Level	CATH Code	Description
	1	Mainly Alpha
	1.10	Orthogonal Bundle
	1.10.800	Phenylalanine Hydroxylase
	1.10.800.10	Aromatic amino acid hydroxylase

Domain Context

CATH Clusters

Superfamily	Aromatic amino acid hydroxylase
Functional Family	Phenylalanine-4-hydroxylase

Enzyme Information

1.14.16.1

Phenylalanine 4-monooxygenase.

based on mapping to UniProt P04176

L-phenylalanine + tetrahydrobiopterin + O(2) = L-tyrosine + 4a-hydroxytetrahydrobiopterin.

-!- The reaction involves an arene oxide which rearranges to give the phenolic hydroxy group. -!- This results in the hydrogen at C-4 migrating to C-3 and in part being retained, a process known as the NIH-shift. -!- The 4a-hydroxytetrahydrobiopterin formed can dehydrate to 6,7- dihydrobiopterin, both spontaneously and by the action of EC 4.2.1.96. -!- The 6,7-dihydrobiopterin can be enzymically reduced back to tetrahydrobiopterin, by EC 1.5.1.34, or slowly rearranges into the more stable compound 7,8-dihydrobiopterin. -!- Formerly EC 1.14.3.1 and EC 1.99.1.2.

UniProtKB Entries (1)

P04176

PH4H_RAT

Rattus norvegicus

Phenylalanine-4-hydroxylase

PDB Structure

PDB	2PHM
External Links	PDBSum Proteopedia
Method	X-RAY DIFFRACTION
Organism	Spodoptera
Primary Citation	Structural basis of autoregulation of phenylalanine hydroxylase. Kobe, B., Jennings, I.G., House, C.M., Michell, B.J., Goodwill, K.E., Santarsiero, B.D., Stevens, R.C., Cotton, R.G., Kemp, B.E. Nat.Struct.Biol.