| 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 |
| protein hydroxylation |
| 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 |
| Cellular Component |
| cytosol |
| Function |
| metal ion binding |
| phenylalanine 4-monooxygenase activity |
| binding |
| catalytic activity |
| transition metal ion binding |
| iron ion binding |
| 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 |
| Molecular Function |
| phenylalanine 4-monooxygenase activity |
| cofactor binding |
| iron ion binding |
| amino acid binding |
| Process |
| l-phenylalanine catabolic process |
| metabolic process |
| cellular metabolic process |
| oxidation reduction |
| cellular amino acid and derivative metabolic process |
| cellular amino acid metabolic process |
| aromatic amino acid family metabolic process |
| l-phenylalanine 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 |