Pah
entrez
18478
canonical
uniprot
P16331
canonical
ensembl
ENSMUSG00000020051
resolver
ensp
ENSMUSP00000020241
resolver
enst
ENSMUST00000020241
resolver
genesymbol
Pah
resolver
uniprot
Q3UEH8
resolver
uniprot
Q91WV1
resolver
uniprot_entry
PH4H_MOUSE
resolver
uniprot_entry
Q3UEH8_MOUSE
resolver
uniprot_sec
Q91WV1
resolver
activity_regulation
ACTIVITY 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. {ECO:0000250|UniProtKB:P00439}.
uniprot
proteins
amino_acid_sequence
MAAVVLENGVLSRKLSDFGQETSYIEDNSNQNGAVSLIFSLKEEVGALAKVLRLFEENEINLTHIESRPSRLNKDEYEFFTYLDKRSKPVLGSIIKSLRNDIGATVHELSRDKEKNTVPWFPRTIQELDRFANQILSYGAELDADHPGFKDPVYRARRKQFADIAYNYRHGQPIPRVEYTEEERKTWGTVFRTLKALYKTHACYEHNHIFPLLEKYCGFREDNIPQLEDVSQFLQTCTGFRLRPVAGLLSSRDFLGGLAFRVFHCTQYIRHGSKPMYTPEPDICHELLGHVPLFSDRSFAQFSQEIGLASLGAPDEYIEKLATIYWFTVEFGLCKEGDSIKAYGAGLLSSFGELQYCLSDKPKLLPLELEKTACQEYTVTEFQPLYYVAESFNDAKEKVRTFAATIPRPFSVRYDPYTQRVEVLDNTQQLKILADSINSEVGILCHALQKIKS
uniprot
proteins
ec_number
1.14.16.1
uniprot
proteins
function
FUNCTION: Catalyzes the hydroxylation of L-phenylalanine to L-tyrosine. {ECO:0000250|UniProtKB:P00439}.
uniprot
proteins
mass_dalton
51900
uniprot
proteins
mutagenesis
MUTAGEN 106; /note="V->A: Mutant mice have mild features of phenylketonuria."; /evidence="ECO:0000269|PubMed:9119379"; MUTAGEN 263; /note="F->S: Mutant mice have features of phenylketonuria."; /evidence="ECO:0000269|PubMed:9119379
uniprot
proteins
pathway_participation
PATHWAY: Amino-acid degradation; L-phenylalanine degradation; acetoacetate and fumarate from L-phenylalanine: step 1/6.
uniprot
proteins
post_translational_modification
PTM: Phosphorylation at Ser-16 increases basal activity and facilitates activation by the substrate phenylalanine. {ECO:0000250|UniProtKB:P00439}.
uniprot
proteins
protein_family
Biopterin-dependent aromatic amino acid hydroxylase family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
19468303
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
2334400
uniprot
proteins
pubmed
6098294
uniprot
proteins
pubmed
7387651
uniprot
proteins
pubmed
9119379
uniprot
proteins
sequence_length
453
uniprot
proteins