KEGG   Homo sapiens (human): 5053Help
Entry
5053              CDS       T01001                                 

Gene name
PAH, PH, PKU, PKU1
Definition
phenylalanine hydroxylase (EC:1.14.16.1)
Orthology
K00500  
phenylalanine-4-hydroxylase [EC:1.14.16.1]
Organism
hsa  Homo sapiens (human)
Pathway
Phenylalanine metabolism
Phenylalanine, tyrosine and tryptophan biosynthesis
Metabolic pathways
Biosynthesis of amino acids
Disease
H00167  
Phenylketonuria (PKU)
Brite
KEGG Orthology (KO) [BR:hsa00001]
 Metabolism
  Overview
   01230 Biosynthesis of amino acids
    5053 (PAH)
  Amino acid metabolism
   00360 Phenylalanine metabolism
    5053 (PAH)
   00400 Phenylalanine, tyrosine and tryptophan biosynthesis
    5053 (PAH)
Enzymes [BR:hsa01000]
 1. Oxidoreductases
  1.14  Acting on paired donors with incorporation of molecular oxygen
   1.14.16  With reduced pteridine as one donor, and incorporation of one atom of oxygen into the other donor
    1.14.16.1  phenylalanine 4-monooxygenase
     5053 (PAH)
BRITE hierarchy
SSDB OrthologParalogGFIT
Motif Motif
Other DBs
NCBI-GI: 
NCBI-GeneID: 
OMIM: 
HGNC: 
HPRD: 
Ensembl: 
Vega: 
UniProt: 
Structure
PDB: 

Jmol
Position
12q22-q24.2
AA seq 452 aa AA seqDB search
MSTAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFSLKEEVGALAKVLRLFEENDV
NLTHIESRPSRLKKDEYEFFTHLDKRSLPALTNIIKILRHDIGATVHELSRDKKKDTVPW
FPRTIQELDRFANQILSYGAELDADHPGFKDPVYRARRKQFADIAYNYRHGQPIPRVEYM
EEEKKTWGTVFKTLKSLYKTHACYEYNHIFPLLEKYCGFHEDNIPQLEDVSQFLQTCTGF
RLRPVAGLLSSRDFLGGLAFRVFHCTQYIRHGSKPMYTPEPDICHELLGHVPLFSDRSFA
QFSQEIGLASLGAPDEYIEKLATIYWFTVEFGLCKQGDSIKAYGAGLLSSFGELQYCLSE
KPKLLPLELEKTAIQNYTVTEFQPLYYVAESFNDAKEKVRNFAATIPRPFSVRYDPYTQR
IEVLDNTQQLKILADSINSEIGILCSALQKIK
NT seq 1359 nt NT seq  +upstreamnt  +downstreamnt
atgtccactgcggtcctggaaaacccaggcttgggcaggaaactctctgactttggacag
gaaacaagctatattgaagacaactgcaatcaaaatggtgccatatcactgatcttctca
ctcaaagaagaagttggtgcattggccaaagtattgcgcttatttgaggagaatgatgta
aacctgacccacattgaatctagaccttctcgtttaaagaaagatgagtatgaatttttc
acccatttggataaacgtagcctgcctgctctgacaaacatcatcaagatcttgaggcat
gacattggtgccactgtccatgagctttcacgagataagaagaaagacacagtgccctgg
ttcccaagaaccattcaagagctggacagatttgccaatcagattctcagctatggagcg
gaactggatgctgaccaccctggttttaaagatcctgtgtaccgtgcaagacggaagcag
tttgctgacattgcctacaactaccgccatgggcagcccatccctcgagtggaatacatg
gaggaagaaaagaaaacatggggcacagtgttcaagactctgaagtccttgtataaaacc
catgcttgctatgagtacaatcacatttttccacttcttgaaaagtactgtggcttccat
gaagataacattccccagctggaagacgtttctcaattcctgcagacttgcactggtttc
cgcctccgacctgtggctggcctgctttcctctcgggatttcttgggtggcctggccttc
cgagtcttccactgcacacagtacatcagacatggatccaagcccatgtatacccccgaa
cctgacatctgccatgagctgttgggacatgtgcccttgttttcagatcgcagctttgcc
cagttttcccaggaaattggccttgcctctctgggtgcacctgatgaatacattgaaaag
ctcgccacaatttactggtttactgtggagtttgggctctgcaaacaaggagactccata
aaggcatatggtgctgggctcctgtcatcctttggtgaattacagtactgcttatcagag
aagccaaagcttctccccctggagctggagaagacagccatccaaaattacactgtcacg
gagttccagcccctgtattacgtggcagagagttttaatgatgccaaggagaaagtaagg
aactttgctgccacaatacctcggcccttctcagttcgctacgacccatacacccaaagg
attgaggtcttggacaatacccagcagcttaagattttggctgattccattaacagtgaa
attggaatcctttgcagtgccctccagaaaataaagtaa

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