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ENTRY	nt06016
Name	Phenylalanine and tyrosine metabolism
Category	Pathway view; Amino acid metabolism
Pathway	hsa00790 Folate biosynthesis hsa00400 Phenylalanine, tyrosine and tryptophan biosynthesis hsa00350 Tyrosine metabolism
Disease	H00167 Phenylketonuria H00165 Tyrosinemia H00163 Alkaptonuria
Display	drug-target relation   disease type

N00700    Phe+BH4 − PAH → Tyr+BH4OH       D2  |     Phenylketonuria PAH     PAH*                   D3  | N00702    GTP − GCH1 ⇉ PTS ⇉ SPR → BH4     Hyperphenylalaninemia, BH4-deficient, B     GCH1*     Hyperphenylalaninemia, BH4-deficient, C         PTS*               D3  | N00705    BH4OH − PCBD1/2 ⇉ QDPR → BH4     Hyperphenylalaninemia, BH4-deficient, D     PCBD1*     Hyperphenylalaninemia, BH4-deficient, A         QDPR*           D1  |                 N00708    Tyr − TAT ⇉ HPD ⇉ HGD ⇉ GSTZ1 ⇉ FAH → Fumarate+Acetoacet..     Tyrosinemia, type II Tyr↗   TAT*     Tyrosinemia, type III Tyr↗       HPD*     Alkaptonuria             HGD*     Tyrosinemia, type I Tyr↗                   FAH*

		Disease name	Disease category
Phenylketonuria PAH	H00167	Phenylketonuria	Inherited metabolic disorder
Hyperphenylalaninemia, BH4-deficient, B	H00167	Phenylketonuria	Inherited metabolic disorder
Hyperphenylalaninemia, BH4-deficient, C	H00167	Phenylketonuria	Inherited metabolic disorder
Hyperphenylalaninemia, BH4-deficient, D	H00167	Phenylketonuria	Inherited metabolic disorder
Hyperphenylalaninemia, BH4-deficient, A	H00167	Phenylketonuria	Inherited metabolic disorder
Tyrosinemia, type II	H00165	Tyrosinemia	Inherited metabolic disorder
Tyrosinemia, type III	H00165	Tyrosinemia	Inherited metabolic disorder
Alkaptonuria	H00163	Alkaptonuria	Inherited metabolic disorder
Tyrosinemia, type I	H00165	Tyrosinemia	Inherited metabolic disorder

		Drug name
D1	D05177	Nitisinone (JAN/USAN/INN)
D2	D11077	Pegvaliase (INN)
D3	D01798	Sapropterin dihydrochloride (USAN)