CHEBI:16015
ChEBI
30572
ChemSpider
Phenylpyruvic acid
7722-84-1
CAS
Homogentisic acid
6140
PubChem-compound
Q96RQ9
UniProt
937
ChemSpider
CHEBI:16134
ChEBI
63-91-2
CAS
P-HYDROXY-PHENYLPYRUVATE
BioCyc
HMDB0003125
HMDB
Phenylalanine--tRNA ligase alpha subunit
P16930
UniProt
110-17-8
CAS
21883788
PubChem-compound
SMILES
[H][C@@]1(CNC2=NC(N)=NC(=O)[C@]2(O)N1)[C@@H](O)[C@H](C)O
SMILES
O
SMILES
OO
H3N
Ammonia
17.026548
L-Phenylalanine
C10H14N5O7P
Adenosine monophosphate
347.06308
50
ChemSpider
1.0
1.0
CHEBI:16027
ChEBI
L-amino-acid oxidase
5833
ChemSpider
HMDB0000538
HMDB
Phenylalanine--tRNA ligase beta subunit
ATP
BioCyc
Aspartate aminotransferase, cytoplasmic
Phenylalanine--tRNA ligase
SMILES
N[C@@H](CC1=CC=C(O)C=C1)C(O)=O
51
PubChem-compound
C8H8O6
Maleylacetoacetic acid
200.03209
Water
1.0
Phenylalanine-4-hydroxylase
Hydrogen peroxide
559142
ChemSpider
1.0
Tyrosine aminotransferase
HMDB0002281
HMDB
C4H6O3
Acetoacetic acid
102.03169
1.0
2.0
Q9Y285
UniProt
Lysosome
CHEBI:59560
ChEBI
1.0
CHEBI:44747
ChEBI
5742
ChemSpider
tRNA(Tyr)
954
ChemSpider
952
ChemSpider
Carbon dioxide
Phenylalanine-4-hydroxylase
4-Fumarylacetoacetic acid
274
ChemSpider
HMDB0002052
HMDB
C5H6O5
Oxoglutaric acid
146.02153
TYR
BioCyc
C9H8O3
Phenylpyruvic acid
164.04735
Citric Acid Cycle
SubPathway
5858
ChemSpider
CHEBI:47904
ChEBI
P32754
UniProt
Adenosine monophosphate
SMILES
OC(=O)C(=O)CC1=CC=C(O)C=C1
Maleylacetoacetate isomerase
CHEBI:16240
ChEBI
O43708
UniProt
HMDB0000205
HMDB
PPI
BioCyc
1.0
4444078
ChemSpider
Reaction79
false
L-Phenylalanine + Oxoglutaric acid ↔ L-Glutamic acid + Phenylpyruvic acid
REVERSIBLE
HMDB0000208
HMDB
L-Glutamic acid
SubPathwayInteraction90
SubPathwayReaction
SubPathway90Reaction
CPD-6641
BioCyc
217
ChemSpider
P00439
UniProt
SubPathwayInteraction89
SubPathway89Reaction
SubPathwayReaction
SMILES
OC(=O)C(=O)CC1=CC=CC=C1
Acetoacetic acid
SubPathwayInput
SubPathwayInteraction88
SubPathwayReaction
SubPathway88Reaction
2.6.1.5
false
2.6.1.5
L-Tyrosine + Oxoglutaric acid ↔ 4-Hydroxyphenylpyruvic acid + L-Glutamic acid
REVERSIBLE
Fumarylacetoacetase
SMILES
OC(=O)CCC(=O)C(O)=O
Tyrosine aminotransferase
CHEBI:30915
ChEBI
124-38-9
CAS
GO:0005764
GENE ONTOLOGY
94
ChemSpider
1.0
C00007
KEGG Compound
451-13-8
CAS
Aspartate aminotransferase, cytoplasmic
4-hydroxyphenylpyruvate dioxygenase
C00001
KEGG Compound
C00122
KEGG Compound
CHEBI:18361
ChEBI
4-Hydroxyphenylpyruvic acid
HMDB0059658
HMDB
1.0
C00002
KEGG Compound
L-Phenylalanine
4-FUMARYL-ACETOACETATE
BioCyc
Fumaric acid
SubPathwayInput
PHENYL-PYRUVATE
BioCyc
4-Hydroxyphenylpyruvic acid
2.0
GLT
BioCyc
7664-41-7
CAS
1.0
C4H4O4
Fumaric acid
116.010956
Tyrosine Metabolism
SubPathway
SMP0000057
SMPDB
CHEBI:30907
ChEBI
C8H8O4
Homogentisic acid
168.04225
Oxygen
Homogentisate 1,2-dioxygenase
Maleylacetoacetic acid
1.0
Adenosine triphosphate
C00011
KEGG Compound
SMILES
O=C=O
1.0
SMILES
N[C@@H](CC1=CC=CC=C1)C(O)=O
L-Tyrosine
SubPathwayInput
222
PubChem-compound
C00014
KEGG Compound
C00013
KEGG Compound
Catecholamine Biosynthesis
SubPathway
H4O7P2
Pyrophosphate
177.94322
40270
ChemSpider
Tyrosinemia Type 3 (TYRO3)
1.0
5957
PubChem-compound
4-hydroxyphenylpyruvate dioxygenase
6.1.1.1
false
6.1.1.1
Adenosine triphosphate + L-Tyrosine + tRNA(Tyr) → Adenosine monophosphate + L-Tyrosyl-tRNA(Tyr) + Pyrophosphate
LEFT_TO_RIGHT
CHEBI:15999
ChEBI
6.1.1.20
false
6.1.1.20
Adenosine triphosphate + L-Phenylalanine + tRNA(Phe) → Adenosine monophosphate + L-Phenylalanyl-tRNA(Phe) + Pyrophosphate
LEFT_TO_RIGHT
56-65-5
CAS
C00020
KEGG Compound
C00027
KEGG Compound
C00026
KEGG Compound
C00025
KEGG Compound
2.0
L-Tyrosyl-tRNA(Tyr)
2.0
Phenylalanine--tRNA ligase beta subunit
1.0
C00272
KEGG Compound
GO:0005737
GENE ONTOLOGY
Aspartate aminotransferase, cytoplasmic
C8H8O6
4-Fumarylacetoacetic acid
200.03209
1.0
Tyrosine--tRNA ligase, cytoplasmic
SMP0000006
SMPDB
3-KETOBUTYRATE
BioCyc
Homogentisate 1,2-dioxygenase
4-hydroxyphenylpyruvate dioxygenase
328-50-7
CAS
HMDB0000134
HMDB
Phenylpyruvic acid
AMP
BioCyc
HYDROGEN-PEROXIDE
BioCyc
1.0
C15522
KEGG Compound
Phenylalanine-4-hydroxylase
Q9NSD9
UniProt
44257
PubChem-compound
HMDB0000130
HMDB
HMDB0000250
HMDB
SMILES
OP(O)(=O)OP(O)(O)=O
tRNA(Phe)
14000-31-8
CAS
L-erythro-tetrahydrobiopterin
C9H8O4
4-Hydroxyphenylpyruvic acid
180.04225
997
PubChem-compound
CHEBI:29161
ChEBI
2.0
C00166
KEGG Compound
C00164
KEGG Compound
L-Tyrosine
Fumarylacetoacetase
SMP0000370
SMPDB
763
ChemSpider
L-Phenylalanyl-tRNA(Phe)
HMDB0000148
HMDB
L-Tyrosyl-tRNA(Tyr)
SMILES
OC(=O)CC(=O)CC(=O)\C=C\C(O)=O
tRNA(Tyr)
Cytoplasm
SMP0000012
SMPDB
Tyrosine--tRNA ligase, cytoplasmic
784
PubChem-compound
10197150
ChemSpider
CHEBI:15422
ChEBI
1.0
Homo sapiens
FUM
BioCyc
62989-33-7
CAS
HMDB0000707
HMDB
7732-18-5
CAS
780
PubChem-compound
CHEBI:18012
ChEBI
61-19-8
CAS
SMILES
NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1
O2
Oxygen
31.98983
HMDB0000158
HMDB
HMDB0000159
HMDB
AMMONIA
BioCyc
SMILES
N
56-86-0
CAS
SMILES
[H][C@@]1(CNC2=C(N1)C(=O)N=C(N)N2)[C@@H](O)[C@H](C)O
2.0
CHEBI:29153
ChEBI
ReactionCatalysis447
ACTIVATION
ReactionCatalysis445
ACTIVATION
C5H9NO4
L-Glutamic acid
147.05316
Phenylalanine--tRNA ligase alpha subunit
ReactionCatalysis446
ACTIVATION
156-06-9
CAS
33032
PubChem-compound
ReactionCatalysis443
ACTIVATION
60-18-4
CAS
ReactionCatalysis444
ACTIVATION
ReactionCatalysis441
ACTIVATION
Water
ReactionCatalysis442
ACTIVATION
1.0
C10H16N5O13P3
Adenosine triphosphate
506.99576
ReactionCatalysis440
ACTIVATION
1.0
129803
PubChem-compound
CHEBI:16526
ChEBI
CHEBI:17295
ChEBI
4444081
ChemSpider
4a-Hydroxytetrahydrobiopterin
7782-44-7
CAS
Pyrophosphate
C01036
KEGG Compound
HMDB0001377
HMDB
Tyrosine--tRNA ligase, cytoplasmic
HMDB0000045
HMDB
SMILES
CC(=O)CC(O)=O
1.0
SMILES
OC(=O)CC(=O)CC(=O)\C=C/C(O)=O
ReactionCatalysis438
ACTIVATION
PHE
BioCyc
ReactionCatalysis436
ACTIVATION
Reaction386
false
Maleylacetoacetic acid → 4-Fumarylacetoacetic acid
LEFT_TO_RIGHT
ReactionCatalysis437
ACTIVATION
3.7.1.2
false
3.7.1.2
4-Fumarylacetoacetic acid + Water → Acetoacetic acid + Fumaric acid
LEFT_TO_RIGHT
ReactionCatalysis435
ACTIVATION
9606
TAXONOMY
962
PubChem-compound
PW000142
PathWhiz
70110-58-6
CAS
1.0
P54577
UniProt
C9H11NO2
L-Phenylalanine
165.07898
2-KETOGLUTARATE
BioCyc
tRNA(Phe)
C9H11NO3
L-Tyrosine
181.0739
114908
ChemSpider
CO2
Carbon dioxide
43.98983
C00079
KEGG Compound
Fumarylacetoacetase
972
ChemSpider
SMILES
O=O
1.0
HMDB0001268
HMDB
Maleylacetoacetate isomerase
C00082
KEGG Compound
Oxoglutaric acid
HMDB0000051
HMDB
SMILES
N[C@@H](CCC(O)=O)C(O)=O
C9H15N5O3
L-erythro-tetrahydrobiopterin
241.1175
HMDB0002111
HMDB
1.4.3.2
false
1.4.3.2
L-Phenylalanine + Oxygen + Water → Ammonia + Hydrogen peroxide + Phenylpyruvic acid
LEFT_TO_RIGHT
1.4.3.2
false
1.4.3.2
L-Tyrosine + Oxygen + Water → 4-Hydroxyphenylpyruvic acid + Ammonia + Hydrogen peroxide
LEFT_TO_RIGHT
1.13.11.5
false
1.13.11.5
Homogentisic acid + Oxygen → Maleylacetoacetic acid
LEFT_TO_RIGHT
1.0
CHEBI:15377
ChEBI
6057
PubChem-compound
CHEBI:15379
ChEBI
PW000017
PathWhiz
5910
ChemSpider
1.0
SMILES
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
5280393
PubChem-compound
1.0
Q93099
UniProt
CHEBI:15374
ChEBI
C01179
KEGG Compound
5280398
PubChem-compound
L-Phenylalanyl-tRNA(Phe)
280
PubChem-compound
C01061
KEGG Compound
HMDB0000060
HMDB
96
PubChem-compound
2.0
1.13.11.27
false
1.13.11.27
4-Hydroxyphenylpyruvic acid + Oxygen → Carbon dioxide + Homogentisic acid
LEFT_TO_RIGHT
28613-33-4
CAS
1.0
CHEBI:15344
ChEBI
PW000121
PathWhiz
156-39-8
CAS
644102
PubChem-compound
PW000005
PathWhiz
6083
PubChem-compound
759
ChemSpider
2.0
2.0
CHEBI:30851
ChEBI
Maleylacetoacetate isomerase
C9H15N5O4
4a-Hydroxytetrahydrobiopterin
257.1124
H2O
Water
18.010565
H2O2
Hydrogen peroxide
34.005478
Oxygen
1.14.16.1
false
1.14.16.1
L-Phenylalanine + L-erythro-tetrahydrobiopterin + Oxygen → 4a-Hydroxytetrahydrobiopterin + L-Tyrosine
LEFT_TO_RIGHT
L-amino-acid oxidase
Ammonia
541-50-4
CAS
CHEBI:17895
ChEBI
1.0
977
PubChem-compound
5698-52-2
CAS
1.0
CHEBI:29182
ChEBI
HMDB0001967
HMDB
P17735
UniProt
C00544
KEGG Compound
1.0
Tyrosine aminotransferase
SMILES
OC(=O)\C=C\C(O)=O
1.0
1.0
CHEBI:29184
ChEBI
979
PubChem-compound
P17174
UniProt
SMILES
OC(=O)CC1=C(O)C=CC(O)=C1
4-MALEYL-ACETOACETATE
BioCyc