HUMAN:PH4H

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Species (Taxon ID)	Homo sapiens (Human). (taxon:9606)
Gene Name(s)	PAH
Protein Name(s)	Phenylalanine-4-hydroxylase PAH Phe-4-monooxygenase
External Links
UniProt Identifier	PH4H_HUMAN
UniProt Accessions	P00439, Q16717, Q8TC14,
EMBL	K03020, U49897, S61296, BC026251,
PIR	A00508,
RefSeq	NP_000268.1,
PDB	1DMW, 1J8T, 1J8U, 1KW0, 1LRM, 1MMK, 1MMT, 1PAH, 1TDW, 1TG2, 2PAH, 3PAH, 4PAH, 5PAH, 6PAH,
IntAct	P00439,
Ensembl	ENST00000307000,
Pfam	PF01842, PF00351,

Annotations

Qualifier	GO ID	GO term name	Reference	Evidence Code	with/from	Aspect	Notes	Status
	GO:0003824	catalytic activity	GO_REF:0000004	IEA: Inferred from Electronic Annotation	SP_KW:KW-0021	F
	GO:0004497	monooxygenase activity	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR001273	F
	GO:0004497	monooxygenase activity	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR018301	F
	GO:0004497	monooxygenase activity	GO_REF:0000004	IEA: Inferred from Electronic Annotation	SP_KW:KW-0503	F
	GO:0004505	phenylalanine 4-monooxygenase activity	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR005961	F
	GO:0004505	phenylalanine 4-monooxygenase activity	GO_REF:0000003	IEA: Inferred from Electronic Annotation	EC:1.14.16.1	F
	GO:0004505	phenylalanine 4-monooxygenase activity	PMID:3856322^[1]	TAS: Traceable Author Statement		F
	GO:0004505	phenylalanine 4-monooxygenase activity	Reactome:REACT_2	EXP: Inferred from Experiment		F
	GO:0005506	iron ion binding	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR001273	F
	GO:0005506	iron ion binding	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR018301	F
	GO:0005829	cytosol	Reactome:REACT_2	TAS: Traceable Author Statement		C
	GO:0006559	L-phenylalanine catabolic process	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR005961	P
	GO:0006559	L-phenylalanine catabolic process	GO_REF:0000004	IEA: Inferred from Electronic Annotation	SP_KW:KW-0585	P
	GO:0006559	L-phenylalanine catabolic process	Reactome:REACT_1786	TAS: Traceable Author Statement		P
	GO:0008152	metabolic process	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR002912	P
	GO:0008152	metabolic process	GO_REF:0000004	IEA: Inferred from Electronic Annotation	SP_KW:KW-0021	P
	GO:0008652	cellular amino acid biosynthetic process	PMID:3856322^[1]	TAS: Traceable Author Statement		P
	GO:0009072	aromatic amino acid family metabolic process	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR001273	P
	GO:0009072	aromatic amino acid family metabolic process	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR018301	P
	GO:0016491	oxidoreductase activity	GO_REF:0000004	IEA: Inferred from Electronic Annotation	SP_KW:KW-0560	F
	GO:0016597	amino acid binding	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR002912	F
	GO:0016714	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	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR019773	F
	GO:0016714	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	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR019774	F
	GO:0034641	cellular nitrogen compound metabolic process	Reactome:REACT_13	TAS: Traceable Author Statement		P
	GO:0042136	neurotransmitter biosynthetic process	PMID:2872999^[2]	NAS: Non-traceable Author Statement		P
	GO:0042423	catecholamine biosynthetic process	PMID:2872999^[2]	NAS: Non-traceable Author Statement		P
	GO:0046872	metal ion binding	GO_REF:0000004	IEA: Inferred from Electronic Annotation	SP_KW:KW-0479	F
	GO:0055114	oxidation-reduction process	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR001273	P
	GO:0055114	oxidation-reduction process	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR005961	P
	GO:0055114	oxidation-reduction process	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR018301	P
	GO:0055114	oxidation-reduction process	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR019773	P
	GO:0055114	oxidation-reduction process	GO_REF:0000002	IEA: Inferred from Electronic Annotation	InterPro:IPR019774	P
	GO:0055114	oxidation-reduction process	GO_REF:0000004	IEA: Inferred from Electronic Annotation	SP_KW:KW-0560	P
	GO:0004505	phenylalanine 4-monooxygenase activity	PMID:20667834^[3]	IDA: Inferred from Direct Assay		F	Figure 1- Shows measurements of PAH activity based on different levels of substrate and cofactors.	complete
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Notes

References

See Help:References for how to manage references in GONUTS.

↑ ^1.0 ^1.1 Ledley FD et al. (1985) Gene transfer and expression of human phenylalanine hydroxylase. Science 228: 77-9 PubMed GONUTS page
↑ ^2.0 ^2.1 Craig SP et al. (1986) Localization of the human tyrosine hydroxylase gene to 11p15: gene duplication and evolution of metabolic pathways. Cytogenet Cell Genet 42: 29-32 PubMed GONUTS page
↑ Gersting SW et al. (2010) Activation of phenylalanine hydroxylase induces positive cooperativity toward the natural cofactor. J Biol Chem 285: 30686-97 PubMed GONUTS page

[PMID:3856322-0] 1.0 ^1.1 Ledley FD et al. (1985) Gene transfer and expression of human phenylalanine hydroxylase. Science 228: 77-9 PubMed GONUTS page

[PMID:2872999-1] 2.0 ^2.1 Craig SP et al. (1986) Localization of the human tyrosine hydroxylase gene to 11p15: gene duplication and evolution of metabolic pathways. Cytogenet Cell Genet 42: 29-32 PubMed GONUTS page

[PMID:20667834-2] Gersting SW et al. (2010) Activation of phenylalanine hydroxylase induces positive cooperativity toward the natural cofactor. J Biol Chem 285: 30686-97 PubMed GONUTS page

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HUMAN:PH4H

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