2PAH

TETRAMERIC HUMAN PHENYLALANINE HYDROXYLASE

External Resource: Annotation

Domain Annotation: SCOP/SCOPe Classification
Domain Annotation: SCOP2 Classification
Domain Annotation: ECOD Classification
Domain Annotation: CATH
Protein Family Annotation
Gene Ontology: Gene Product Annotation
InterPro: Protein Family Classification
Pharos: Disease Associations

Domain Annotation: SCOP/SCOPe Classification SCOP-e Database Homepage

Chains	Domain Info	Class	Fold	Superfamily	Family	Domain	Species	Provenance Source (Version)
A	d2paha_	Alpha and beta proteins (a+b)	Aromatic aminoacid monoxygenases, catalytic and oligomerization domains	Aromatic aminoacid monoxygenases, catalytic and oligomerization domains	Aromatic aminoacid monoxygenases, catalytic and oligomerization domains	Phenylalanine hydroxylase, PAH	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)
B	d2pahb_	Alpha and beta proteins (a+b)	Aromatic aminoacid monoxygenases, catalytic and oligomerization domains	Aromatic aminoacid monoxygenases, catalytic and oligomerization domains	Aromatic aminoacid monoxygenases, catalytic and oligomerization domains	Phenylalanine hydroxylase, PAH	human (Homo sapiens ) [TaxId: 9606 ],	SCOPe (2.08)

Domain Annotation: SCOP2 Classification SCOP2 Database Homepage

Chains	Type	Family Name	Domain Identifier	Family Identifier	Provenance Source (Version)
A	SCOP2B Superfamily	Aromatic aminoacid monoxygenases catalytic and oligomerization domains	8039871	3001457	SCOP2B (2022-06-29)
B	SCOP2B Superfamily	Aromatic aminoacid monoxygenases catalytic and oligomerization domains	8039871	3001457	SCOP2B (2022-06-29)

Domain Annotation: ECOD Classification ECOD Database Homepage

Chains	Family Name	Domain Identifier	Architecture	Possible Homology	Homology	Topology	Family	Provenance Source (Version)
A	Biopterin_H	e2pahA1	A: a+b complex topology	X: Aromatic aminoacid monoxygenases, catalytic and oligomerization domains (From Topology)	H: Aromatic aminoacid monoxygenases, catalytic and oligomerization domains (From Topology)	T: Aromatic aminoacid monoxygenases, catalytic and oligomerization domains	F: Biopterin_H	ECOD (1.6)
B	Biopterin_H	e2pahB1	A: a+b complex topology	X: Aromatic aminoacid monoxygenases, catalytic and oligomerization domains (From Topology)	H: Aromatic aminoacid monoxygenases, catalytic and oligomerization domains (From Topology)	T: Aromatic aminoacid monoxygenases, catalytic and oligomerization domains	F: Biopterin_H	ECOD (1.6)

Domain Annotation: CATH CATH Database Homepage

Chain	Domain	Class	Architecture	Topology	Homology	Provenance Source (Version)
A	1.10.800.10	Mainly Alpha	Orthogonal Bundle	Phenylalanine Hydroxylase	Aromatic amino acid hydroxylase	CATH (4.3.0)
B	1.10.800.10	Mainly Alpha	Orthogonal Bundle	Phenylalanine Hydroxylase	Aromatic amino acid hydroxylase	CATH (4.3.0)

Protein Family Annotation Pfam Database Homepage

Chains	Accession	Name	Description	Comments	Source
A, B	PF00351	Biopterin-dependent aromatic amino acid hydroxylase (Biopterin_H)	Biopterin-dependent aromatic amino acid hydroxylase	This family includes phenylalanine-4-hydroxylase, the phenylketonuria disease protein.	Domain

Gene Ontology: Gene Product Annotation Gene Ontology Database Homepage

Chains	Polymer	Molecular Function	Biological Process	Cellular Component
A, B	PROTEIN (PHENYLALANINE HYDROXYLASE)	cation binding transition metal ion binding iron ion binding ion binding binding metal ion binding small molecule binding oxidoreductase activity monooxygenase activity oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen 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 phenylalanine 4-monooxygenase activity catalytic activity	catabolic process oxoacid metabolic process aromatic amino acid family catabolic process amino acid metabolic process organic acid catabolic process organic acid metabolic process alpha-amino acid metabolic process cellular catabolic process organic substance metabolic process carboxylic acid catabolic process small molecule metabolic process amino acid catabolic process organonitrogen compound catabolic process erythrose 4-phosphate/phosphoenolpyruvate family amino acid metabolic process catabolic process oxoacid metabolic process aromatic amino acid family catabolic process amino acid metabolic process organic acid catabolic process organic acid metabolic process alpha-amino acid metabolic process cellular catabolic process organic substance metabolic process carboxylic acid catabolic process small molecule metabolic process amino acid catabolic process organonitrogen compound catabolic process erythrose 4-phosphate/phosphoenolpyruvate family amino acid metabolic process organonitrogen compound metabolic process L-phenylalanine catabolic process aromatic amino acid metabolic process cellular metabolic process carboxylic acid metabolic process L-amino acid catabolic process primary metabolic process organic cyclic compound catabolic process L-amino acid metabolic process L-phenylalanine metabolic process organic cyclic compound metabolic process proteinogenic amino acid metabolic process erythrose 4-phosphate/phosphoenolpyruvate family amino acid catabolic process alpha-amino acid catabolic process proteinogenic amino acid catabolic process metabolic process cellular process small molecule catabolic process organic substance catabolic process amino acid biosynthetic process biosynthetic process organic substance biosynthetic process cellular biosynthetic process organonitrogen compound biosynthetic process organic acid biosynthetic process carboxylic acid biosynthetic process tyrosine metabolic process aromatic amino acid family biosynthetic process small molecule biosynthetic process L-amino acid biosynthetic process aromatic amino acid family biosynthetic process, prephenate pathway organic cyclic compound biosynthetic process proteinogenic amino acid biosynthetic process erythrose 4-phosphate/phosphoenolpyruvate family amino acid biosynthetic process tyrosine biosynthetic process alpha-amino acid biosynthetic process organic hydroxy compound metabolic process catechol-containing compound metabolic process amine metabolic process biogenic amine biosynthetic process catecholamine metabolic process amine biosynthetic process phenol-containing compound metabolic process organic hydroxy compound biosynthetic process catecholamine biosynthetic process phenol-containing compound biosynthetic process catechol-containing compound biosynthetic process biogenic amine metabolic process	intracellular anatomical structure cytoplasm cellular anatomical entity cytosol

InterPro: Protein Family Classification InterPro Database Homepage

Chains	Accession	Name	Type
A, B	IPR019773	Tyrosine 3-monooxygenase-like	Family
A, B	IPR005961	Phenylalanine-4-hydroxylase, tetrameric form	Family
A, B	IPR019774	Aromatic amino acid hydroxylase, C-terminal	Domain
A, B	IPR045865	ACT-like domain	Homologous Superfamily
A, B	IPR002912	ACT domain	Domain
A, B	IPR041912	Eukaryotic phenylalanine-4-hydroxylase, catalytic domain	Domain
A, B	IPR036329	Aromatic amino acid monoxygenase, C-terminal domain superfamily	Homologous Superfamily
A, B	IPR036951	Aromatic amino acid hydroxylase superfamily	Homologous Superfamily
A, B	IPR001273	Aromatic amino acid hydroxylase	Family
A, B	IPR018301	Aromatic amino acid hydroxylase, iron/copper binding site	Binding Site

Pharos: Disease Associations Pharos Homepage Annotation

Chains	Drug Target	Associated Disease
A, B	Pharos: P00439	phenylketonuria Classical phenylketonuria pancreatic ductal adenocarcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia atypical teratoid rhabdoid tumor glioblastoma interstitial cystitis lung carcinoma medulloblastoma pancreatic intraductal papillary-mucinous carcinoma pilocytic astrocytoma schizophrenia bipolar disorder drug-induced liver injury hyperphenylalaninemia due to tetrahydrobiopterin deficiency phenylketonuria Classical phenylketonuria pancreatic ductal adenocarcinoma pancreatic intraductal papillary-mucinous neoplasm with low grade dysplasia atypical teratoid rhabdoid tumor glioblastoma interstitial cystitis lung carcinoma medulloblastoma pancreatic intraductal papillary-mucinous carcinoma pilocytic astrocytoma schizophrenia bipolar disorder drug-induced liver injury hyperphenylalaninemia due to tetrahydrobiopterin deficiency inborn errors of metabolism large cell medulloblastoma malignant ependymoma nephrosclerosis pancreatic intraductal papillary-mucinous neoplasm primitive neuroectodermal tumor Sjogren syndrome classic phenylketonuria intellectual disability lung cancer malignant glioma

RCSB PDB Core Operations are funded by the National Science Foundation (DBI-1832184), the US Department of Energy (DE-SC0019749), and the National Cancer Institute, National Institute of Allergy and Infectious Diseases, and National Institute of General Medical Sciences of the National Institutes of Health under grant R01GM133198.