Identification |
HMDB Protein ID
| CDBP00274 |
Secondary Accession Numbers
| Not Available |
Name
| Phenylalanine-4-hydroxylase |
Description
| Not Available |
Synonyms
|
- PAH
- Phe-4-monooxygenase
|
Gene Name
| PAH |
Protein Type
| Enzyme |
Biological Properties |
General Function
| Involved in amino acid binding |
Specific Function
| Not Available |
GO Classification
|
Biological Process |
cellular nitrogen compound metabolic process |
tetrahydrobiopterin metabolic process |
tyrosine biosynthetic process |
neurotransmitter biosynthetic process |
cellular amino acid biosynthetic process |
L-phenylalanine catabolic process |
catecholamine biosynthetic process |
protein hydroxylation |
Cellular Component |
cytosol |
Function |
monooxygenase activity |
carboxylic acid binding |
amino acid binding |
oxidoreductase activity |
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced pteridine as one donor, and incorporation of one atom of oxygen |
ion binding |
cation binding |
metal ion binding |
phenylalanine 4-monooxygenase activity |
binding |
catalytic activity |
transition metal ion binding |
iron ion binding |
Molecular Function |
phenylalanine 4-monooxygenase activity |
cofactor binding |
iron ion binding |
amino acid binding |
Process |
aromatic amino acid family metabolic process |
l-phenylalanine metabolic process |
l-phenylalanine catabolic process |
metabolic process |
cellular metabolic process |
oxidation reduction |
cellular amino acid and derivative metabolic process |
cellular amino acid metabolic process |
|
Cellular Location
|
Not Available
|
Pathways
|
Name | SMPDB/Pathwhiz | KEGG | Phenylalanine and Tyrosine Metabolism | | | L-phenylalanine degradation | Not Available | Not Available | Phenylalanine metabolism | Not Available | | Phenylalanine, tyrosine and tryptophan biosynthesis | Not Available | | Phenylketonuria | | Not Available |
|
Gene Properties |
Chromosome Location
| 12 |
Locus
| 12q22-q24.2 |
SNPs
| PAH |
Gene Sequence
|
>1359 bp
ATGTCCACTGCGGTCCTGGAAAACCCAGGCTTGGGCAGGAAACTCTCTGACTTTGGACAG
GAAACAAGCTATATTGAAGACAACTGCAATCAAAATGGTGCCATATCACTGATCTTCTCA
CTCAAAGAAGAAGTTGGTGCATTGGCCAAAGTATTGCGCTTATTTGAGGAGAATGATGTA
AACCTGACCCACATTGAATCTAGACCTTCTCGTTTAAAGAAAGATGAGTATGAATTTTTC
ACCCATTTGGATAAACGTAGCCTGCCTGCTCTGACAAACATCATCAAGATCTTGAGGCAT
GACATTGGTGCCACTGTCCATGAGCTTTCACGAGATAAGAAGAAAGACACAGTGCCCTGG
TTCCCAAGAACCATTCAAGAGCTGGACAGATTTGCCAATCAGATTCTCAGCTATGGAGCG
GAACTGGATGCTGACCACCCTGGTTTTAAAGATCCTGTGTACCGTGCAAGACGGAAGCAG
TTTGCTGACATTGCCTACAACTACCGCCATGGGCAGCCCATCCCTCGAGTGGAATACATG
GAGGAAGAAAAGAAAACATGGGGCACAGTGTTCAAGACTCTGAAGTCCTTGTATAAAACC
CATGCTTGCTATGAGTACAATCACATTTTTCCACTTCTTGAAAAGTACTGTGGCTTCCAT
GAAGATAACATTCCCCAGCTGGAAGACGTTTCTCAATTCCTGCAGACTTGCACTGGTTTC
CGCCTCCGACCTGTGGCTGGCCTGCTTTCCTCTCGGGATTTCTTGGGTGGCCTGGCCTTC
CGAGTCTTCCACTGCACACAGTACATCAGACATGGATCCAAGCCCATGTATACCCCCGAA
CCTGACATCTGCCATGAGCTGTTGGGACATGTGCCCTTGTTTTCAGATCGCAGCTTTGCC
CAGTTTTCCCAGGAAATTGGCCTTGCCTCTCTGGGTGCACCTGATGAATACATTGAAAAG
CTCGCCACAATTTACTGGTTTACTGTGGAGTTTGGGCTCTGCAAACAAGGAGACTCCATA
AAGGCATATGGTGCTGGGCTCCTGTCATCCTTTGGTGAATTACAGTACTGCTTATCAGAG
AAGCCAAAGCTTCTCCCCCTGGAGCTGGAGAAGACAGCCATCCAAAATTACACTGTCACG
GAGTTCCAGCCCCTGTATTACGTGGCAGAGAGTTTTAATGATGCCAAGGAGAAAGTAAGG
AACTTTGCTGCCACAATACCTCGGCCCTTCTCAGTTCGCTACGACCCATACACCCAAAGG
ATTGAGGTCTTGGACAATACCCAGCAGCTTAAGATTTTGGCTGATTCCATTAACAGTGAA
ATTGGAATCCTTTGCAGTGCCCTCCAGAAAATAAAGTAA
|
Protein Properties |
Number of Residues
| 452 |
Molecular Weight
| 51861.565 |
Theoretical pI
| 6.595 |
Pfam Domain Function
|
|
Signals
|
Not Available
|
Transmembrane Regions
|
Not Available
|
Protein Sequence
|
>Phenylalanine-4-hydroxylase
MSTAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFSLKEEVGALAKVLRLFEENDV
NLTHIESRPSRLKKDEYEFFTHLDKRSLPALTNIIKILRHDIGATVHELSRDKKKDTVPW
FPRTIQELDRFANQILSYGAELDADHPGFKDPVYRARRKQFADIAYNYRHGQPIPRVEYM
EEEKKTWGTVFKTLKSLYKTHACYEYNHIFPLLEKYCGFHEDNIPQLEDVSQFLQTCTGF
RLRPVAGLLSSRDFLGGLAFRVFHCTQYIRHGSKPMYTPEPDICHELLGHVPLFSDRSFA
QFSQEIGLASLGAPDEYIEKLATIYWFTVEFGLCKQGDSIKAYGAGLLSSFGELQYCLSE
KPKLLPLELEKTAIQNYTVTEFQPLYYVAESFNDAKEKVRNFAATIPRPFSVRYDPYTQR
IEVLDNTQQLKILADSINSEIGILCSALQKIK
|
External Links |
GenBank ID Protein
| 189937 |
UniProtKB/Swiss-Prot ID
| P00439 |
UniProtKB/Swiss-Prot Entry Name
| PH4H_HUMAN |
PDB IDs
|
|
GenBank Gene ID
| K03020 |
GeneCard ID
| PAH |
GenAtlas ID
| PAH |
HGNC ID
| HGNC:8582 |
References |
General References
| Not Available |