2PAH: Tetrameric human phenylalanine hydroxylase

Name: PDB 2PAH: Tetrameric human phenylalanine hydroxylase
Published: 1999-10-06
License: https://creativecommons.org/publicdomain/zero/1.0/
Keywords: hydroxylase

Tetrameric human phenylalanine hydroxylase. Determined by X-ray diffraction at 3.1 Å resolution. Released 6 Oct 1999.

Method: X-ray diffraction
Resolution: 3.1 Å
Organism: Homo sapiens
Chains: 2
Atoms: 5,317
Mol. weight: 77.4 kDa
Ligands: FE
Released: 6 Oct 1999

Explore 2PAH in 3D Show helices and sheets RCSB PDB PDBe

Secondary structure: helices and β-sheets

2PAH contains 36 α-helices and 24 β-strands across 2 chains. Residue ranges use author residue numbering, as in the PDB file, from PDBe. To see them in 3D, choose the Cartoon representation with Secondary structure coloring: helices, sheets and coils get different colors.

Chain A: 18 helices, 12 β-strands

Element	Residues	Length	Sheet
β-strand	124	1	1
α-helix	125-130	6
α-helix	152-167	16
α-helix	173-176	4
α-helix	181-201	21
β-strand	202	1	2
α-helix	204-216	13
β-strand	220	1	3
β-strand	223	1	3
α-helix	224-226	3
α-helix	227-237	11
β-strand	241-244	4	4
α-helix	251-258	8
β-strand	262-265	4	4
α-helix	283-285	3
α-helix	286-290	5
α-helix	297-310	14
α-helix	315-325	11
α-helix	326-330	5
β-strand	333-336	4	2
β-strand	339-342	4	2
α-helix	345-348	4
α-helix	351-356	6
β-strand	365-366	2	2
α-helix	369-372	4
β-strand	387-389	3	2
α-helix	392-404	13
β-strand	411-415	5	1
β-strand	420-424	5	1
α-helix	426-450	25

Chain B: 18 helices, 12 β-strands

Element	Residues	Length	Sheet
β-strand	124	1	5
α-helix	125-129	5
α-helix	152-167	16
α-helix	173-176	4
α-helix	181-201	21
β-strand	202	1	6
α-helix	204-216	13
β-strand	220	1	7
β-strand	223	1	7
α-helix	224-226	3
α-helix	227-237	11
β-strand	241-244	4	8
α-helix	251-258	8
β-strand	262-265	4	8
α-helix	283-285	3
α-helix	286-290	5
α-helix	297-310	14
α-helix	315-325	11
α-helix	326-330	5
β-strand	333-336	4	6
β-strand	339-342	4	6
α-helix	345-348	4
α-helix	351-356	6
β-strand	365-366	2	6
α-helix	369-372	4
β-strand	387-389	3	6
α-helix	392-405	14
β-strand	411-415	5	5
β-strand	420-424	5	5
α-helix	427-449	23

Molecules and chains

Molecule	Chains	Type	Length	Organism	UniProt
Protein (phenylalanine hydroxylase)	A, B	protein	335	Homo sapiens	P00439 (AlphaFold model)

Sequence of entity 1 (A, B), FASTA

>2PAH_1 PROTEIN (PHENYLALANINE HYDROXYLASE) (chains A, B)
VPWFPRTIQELDRFANQILSYGAELDADHPGFKDPVYRARRKQFADIAYNYRHGQPIPRV
EYMEEEKKTWGTVFKTLKSLYKTHACYEYNHIFPLLEKYCGFHEDNIPQLEDVSQFLQTC
TGFRLRPVAGLLSSRDFLGGLAFRVFHCTQYIRHGSKPMYTPEPDICHELLGHVPLFSDR
SFAQFSQEIGLASLGAPDEYIEKLATIYWFTVEFGLCKQGDSIKAYGAGLLSSFGELQYC
LSEKPKLLPLELEKTAIQNYTVTEFQPLYYVAESFNDAKEKVRNFAATIPRPFSVRYDPY
TQRIEVLDNTQQLKILADSINSEIGILCSALQKIK

Ligands and cofactors

ID	Name	Formula	Copies
FE	FE (III) ion	Fe	2

Primary citation

Structure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuria. Fusetti, F., Erlandsen, H., Flatmark, T. et al. J Biol Chem (1998) 273:16962-16967. DOI 10.1074/jbc.273.27.16962 · PubMed

Other PDB entries of the same protein (UniProt P00439 (AlphaFold model), which also has an AlphaFold model), best resolution first:

1J8U 1.5 Å, Catalytic Domain of Human Phenylalanine Hydroxylase Fe(II) in Complex with…
6HPO 1.67 Å, Crystallographic structure of the catalytic domain of Human Phenylalanine Hydroxylase…
1J8T 1.7 Å, Catalytic Domain of Human Phenylalanine Hydroxylase Fe(II)
5FII 1.8 Å, Structure of a human aspartate kinase, chorismate mutase and TyrA domain.
9T1O 1.94 Å, Crystal structure of phenylalanine hydroxylase (PAH) with Belinostat
1DMW 2.0 Å, Crystal structure of double truncated human phenylalanine hydroxylase with bound…
1MMK 2.0 Å, Crystal structure of ternary complex of the catalytic domain of human phenylalanine…
1MMT 2.0 Å, Crystal structure of ternary complex of the catalytic domain of human phenylalanine…
1PAH 2.0 Å, Human phenylalanine hydroxylase dimer, residues 117-424
3PAH 2.0 Å, Human phenylalanine hydroxylase catalytic domain dimer with bound adrenaline inhibitor
4PAH 2.0 Å, Human phenylalanine hydroxylase catalytic domain dimer with bound nor-adrenaline inhibitor
1LRM 2.1 Å, Crystal structure of binary complex of the catalytic domain of human phenylalanine…

Browse structure collections

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