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Protein Page:
PAH (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
PAH phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. Note: This description may include information from UniProtKB.
Protein type: EC 1.14.16.1; Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis; Oxidoreductase
Chromosomal Location of Human Ortholog: 12q22-q24.2
Cellular Component: cytosol
Molecular Function: amino acid binding; iron ion binding; phenylalanine 4-monooxygenase activity
Biological Process: L-phenylalanine catabolic process; catecholamine biosynthetic process; amino acid biosynthetic process; neurotransmitter biosynthetic process
Reference #:  P00439 (UniProtKB)
Alt. Names/Synonyms: PAH; PH; PH4H; Phe-4-monooxygenase; phenylalanine hydroxylase; Phenylalanine-4-hydroxylase; PKU; PKU1
Gene Symbols: PAH
Molecular weight: 51,862 Da
Basal Isoelectric point: 6.15  Predict pI for various phosphorylation states
Select Structure to View Below

PAH

Protein Structure Not Found.


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Sites Implicated In
enzymatic activity, induced: S16‑p
molecular association, regulation: S16‑p
protein conformation: S16‑p

Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 S2-p ______MstAVLENP
0 1 T3-p _____MstAVLENPG
0 3 G12 VLENPGLGRKLsDFG
7 41 S16-p PGLGRKLsDFGQETS
0 1 T22 LsDFGQETSYIEDNC
0 2 Y24 DFGQETSYIEDNCNQ
0 1 C29 TSYIEDNCNQNGAIS
0 2 K50 EEVGALAKVLRLFEE
0 1 K74 SRPSRLKKDEYEFFT
0 1 K74 SRPSRLKKDEYEFFT
0 1 K85 EFFTHLDKRsLPALt
0 1 K85 EFFTHLDKRsLPALt
0 1 S87-p FTHLDKRsLPALtNI
0 1 L88 THLDKRsLPALtNII
0 2 T92-p KRsLPALtNIIKILR
0 2 K96 PALtNIIKILRHDIG
0 1 I97 ALtNIIKILRHDIGA
0 1 K115 ELSRDKKKDtVPWFP
0 1 T117-p SRDKKKDtVPWFPRT
0 1 K150 DADHPGFKDPVYRAR
0 2 K150 DADHPGFKDPVYRAR
0 1 K159 PVYRARRKQFADIAY
0 1 Y166 KQFADIAYNYRHGQP
0 1 K185 EYMEEEKKTWGTVFK
0 1 K199 KTLKSLYKTHACYEY
0 1 K274 QYIRHGSKPMYTPEP
0 1 K361 LQYCLSEKPKLLPLE
0 1 K363 YCLSEKPKLLPLELE
0 1 S411 ATIPRPFSVRYDPYT
0 3 S436-p QLKILADsINSEIGI
  mouse

 
A2 ______MAAVVLENG
A3 _____MAAVVLENGV
S12-p VLENGVLsRKLsDFG
S16-p GVLsRKLsDFGQEtS
T22-p LsDFGQEtSyIEDNs
Y24-p DFGQEtSyIEDNsNQ
S29-p tSyIEDNsNQNGAVS
K50-ub EEVGALAkVLRLFEE
K74-ub SRPSRLNkDEYEFFT
K74-sc SRPSRLNkDEYEFFT
K85-ub EFFTYLDkRSkPVLG
K85-sc EFFTYLDkRSkPVLG
S87 FTYLDkRSkPVLGSI
K88-ub TYLDkRSkPVLGSII
G92 kRSkPVLGSIIksLR
K96-ub PVLGSIIksLRNDIG
S97-p VLGSIIksLRNDIGA
K115-ub ELSRDKEkNTVPWFP
T117 SRDKEkNTVPWFPRT
K150-ac DADHPGFkDPVYRAR
K150-ub DADHPGFkDPVYRAR
K159-ub PVYRARRkQFADIAy
Y166-p kQFADIAyNYRHGQP
K185-ub EYTEEERkTWGTVFR
K199-ub RTLKALYkTHACYEH
K274-ub QYIRHGSkPMYTPEP
K361-ub LQYCLSDkPkLLPLE
K363-ub YCLSDkPkLLPLELE
S411-p ATIPRPFsVRYDPYT
S436-p QLKILADsINSEVGI
  rat

 
A2 ______MAAVVLENG
A3 _____MAAVVLENGV
S12 VLENGVLSRKLsDFG
S16-p GVLSRKLsDFGQETS
T22 LsDFGQETSYIEDNS
Y24 DFGQETSYIEDNSNQ
S29 TSYIEDNSNQNGAIS
K50 EEVGALAKVLRLFEE
K74 SRPSRLNKDEYEFFT
K74 SRPSRLNKDEYEFFT
K85 EFFTYLDKRTKPVLG
K85 EFFTYLDKRTKPVLG
T87 FTYLDKRTKPVLGSI
K88 TYLDKRTKPVLGSII
G92 KRTKPVLGSIIKSLR
K96 PVLGSIIKSLRNDIG
S97 VLGSIIKSLRNDIGA
K115 ELSRDKEKNTVPWFP
T117 SRDKEKNTVPWFPRT
K150 DADHPGFKDPVYRAR
K150 DADHPGFKDPVYRAR
K159 PVYRARRKQFADIAY
Y166 KQFADIAYNYRHGQP
Q185 EYTEEEKQTWGTVFR
K199 RTLKALYKTHACYEH
K274 QYIRHGSKPMYTPEP
K361 LQYCLSDKPKLLPLE
K363 YCLSDKPKLLPLELE
S411 ATIPRPFSVRYDPYT
S436 QLKILADSINSEVGI
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