Reviewed, UniProtKB/Swiss-Prot Q2KIH7 (PH4H_BOVIN)

Names and origin

Protein names

Recommended name:
    Phenylalanine-4-hydroxylase
      Short name=PAH
    EC=1.14.16.1
Alternative name(s):
    Phe-4-monooxygenase

Gene names

Name:

PAH

Organism

Bos taurus (Bovine)

Taxonomic identifier

9913 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Laurasiatheria › Cetartiodactyla › Ruminantia › Pecora › Bovidae › Bovinae › Bos

Protein attributes

Sequence length	451 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is not processed.
Protein existence	Evidence at transcript level.

General annotation (Comments)

Catalytic activity	L-phenylalanine + tetrahydrobiopterin + O(2) = L-tyrosine + 4a-hydroxytetrahydrobiopterin.
Cofactor	Fe(2+) ion By similarity.
Enzyme regulation	N-terminal region of PAH is thought to contain allosteric binding sites for phenylalanine and to constitute an "inhibitory" domain that regulates the activity of a catalytic domain in the C-terminal portion of the molecule By similarity.
Pathway	Amino-acid degradation; L-phenylalanine degradation; acetoacetic acid and fumarate from L-phenylalanine: step 1/6.
Subunit structure	Homodimer By similarity.
Sequence similarities	Belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Contains 1 ACT domain.

Ontologies

Keywords
Biological process	Phenylalanine catabolism
Ligand	Iron Metal-binding
Molecular function	Monooxygenase Oxidoreductase
PTM	Phosphoprotein
Technical term	Allosteric enzyme
Gene Ontology (GO)
Biological process	L-phenylalanine catabolic process Inferred from electronic annotation. Source: InterPro oxidation reduction Inferred from electronic annotation. Source: UniProtKB-KW
Molecular function	amino acid binding Inferred from electronic annotation. Source: InterPro iron ion binding Inferred from electronic annotation. Source: InterPro phenylalanine 4-monooxygenase activity Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 451	451	Phenylalanine-4-hydroxylase		PRO_0000281913
Regions
	Domain	34 – 109	76	ACT
Sites
	Metal binding	284	1	Iron By similarity
	Metal binding	289	1	Iron By similarity
	Metal binding	329	1	Iron By similarity
Amino acid modifications
	Modified residue	16	1	Phosphoserine; by PKA By similarity

Sequences

	Sequence		Length	Mass (Da)
	Q2KIH7-1 [UniParc]. Last modified March 7, 2006. Version 1. Checksum: 2D2791910652BD2E	FASTA	451	51,727
	10 20 30 40 50 60 MSALVLESRA LGRKLSDFGQ ETSYIEGNSD QNAVSLIFSL KEEVGALARV LRLFEENDIN 70 80 90 100 110 120 LTHIESRPSR LRKDEYEFFT NLDQRSVPAL ANIIKILRHD IGATVHELSR DKKKDTVPWF 130 140 150 160 170 180 PRTIQELDNF ANQVLSYGAE LDADHPGFKD PVYRARRKQF ADIAYNYRHG QPIPRVEYTE 190 200 210 220 230 240 EEKKTWGTVF RTLKSLYKTH ACYEHNHIFP LLEKYCGFRE DNIPQLEEVS QFLQSCTGFR 250 260 270 280 290 300 LRPVAGLLSS RDFLGGLAFR VFHCTQYIRH GSKPMYTPEP DICHELLGHV PLFSDRSFAQ 310 320 330 340 350 360 FSQEIGLASL GAPDEYIEKL ATIYWFTVEF GLCKQGDSIK AYGAGLLSSF GELQYCLSDK 370 380 390 400 410 420 PKLLPLELEK TAVQEYTITE FQPLYYVAES FNDAKEKVRN FAATIPRPFS VHYDPYTQRI 430 440 450 EVLDNTQQLK ILADSISSEV EILCSALQKL K

References

[1]	"Characterization of 954 bovine full-CDS cDNA sequences." Harhay G.P., Sonstegard T.S., Keele J.W., Heaton M.P., Clawson M.L., Snelling W.M., Wiedmann R.T., Van Tassell C.P., Smith T.P.L. BMC Genomics 6:166-166(2005) [PubMed: 16305752] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
[2]	NIH - Mammalian Gene Collection (MGC) project Submitted (JAN-2006) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Strain: Hereford. Tissue: Testis.

Cross-references

Sequence databases
EMBLGenBankDDBJ	BT030585 mRNA. Translation: ABQ13025.1. BC112633 mRNA. Translation: AAI12634.1.
RefSeq	NP_001039523.1.
UniGene	Bt.61435
3D structure databases
SMR	Q2KIH7. Positions 19-426.
ModBase	Search...
Genome annotation databases
Ensembl	ENSBTAG00000012794. Bos taurus. [Contig view]
GeneID	510583.
KEGG	bta:510583.
Phylogenomic databases
HOVERGEN	Q2KIH7.
Family and domain databases
InterPro	IPR001273. Aaa_hydroxylase. IPR002912. ACT_bd. IPR005961. Phe-4-hydroxylase_tetra. [Graphical view]
Gene3D	G3DSA:1.10.800.10. Aaa_hydroxylase. 1 hit.
PANTHER	PTHR11473. Aaa_hydroxylase. 1 hit.
Pfam	PF01842. ACT. 1 hit. PF00351. Biopterin_H. 1 hit. [Graphical view]
PRINTS	PR00372. FYWHYDRXLASE.
ProDom	PD002559. Aaa_hydroxylase. 1 hit. [Graphical view] [Entries sharing at least one domain]
TIGRFAMs	TIGR01268. Phe4hydrox_tetr. 1 hit.
PROSITE	PS00367. BIOPTERIN_HYDROXYL. 1 hit. [Graphical view]
ProtoNet	Search...

Entry information

Entry name

PH4H_BOVIN

Accession

Primary (citable) accession number: Q2KIH7
Secondary accession number(s): A5D9G6

Entry history

Integrated into UniProtKB/Swiss-Prot:	April 3, 2007
Last sequence update:	March 7, 2006
Last modified:	November 25, 2008
This is version 25 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)