CHEBI:16015
ChEBI
30572
ChemSpider
P25093
UniProt
Phenylpyruvic acid
7722-84-1
CAS
Homogentisic acid
6140
PubChem-compound
ReactionCatalysis168877
ACTIVATION
937
ChemSpider
CHEBI:16134
ChEBI
63-91-2
CAS
P-HYDROXY-PHENYLPYRUVATE
BioCyc
HMDB0003125
HMDB
110-17-8
CAS
21883788
PubChem-compound
SMILES
O
SMILES
OO
SMILES
CC(O)C(O)C1CNC2=NC(N)=NC(=O)C2(O)N1
Q6P721
UniProt
H3N
Ammonia
17.026548
L-Phenylalanine
C10H14N5O7P
Adenosine monophosphate
347.06308
50
ChemSpider
1.0
1.0
CHEBI:16027
ChEBI
ReactionCatalysis168867
ACTIVATION
Tyrosinemia Type 2 (or Richner-Hanhart Syndrome)
5833
ChemSpider
HMDB0000538
HMDB
2.0
ATP
BioCyc
SMILES
N[C@@H](CC1=CC=C(O)C=C1)C(O)=O
51
PubChem-compound
C8H8O6
Maleylacetoacetic acid
200.03209
Water
1.0
Hydrogen peroxide
559142
ChemSpider
1.0
HMDB0002281
HMDB
Q4KM49
UniProt
C4H6O3
Acetoacetic acid
102.03169
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
P04176
UniProt
1.0
SubPathwayInteraction108539
SubPathway108539Reaction
SubPathwayReaction
Q505J8
UniProt
Lysosome
CHEBI:59560
ChEBI
1.0
CHEBI:44747
ChEBI
5742
ChemSpider
tRNA(Tyr)
954
ChemSpider
952
ChemSpider
Tyrosine aminotransferase
Amine oxidase [flavin-containing] A
Carbon dioxide
4-Fumarylacetoacetic acid
274
ChemSpider
HMDB0002052
HMDB
C5H6O5
Oxoglutaric acid
146.02153
TYR
BioCyc
C9H8O3
Phenylpyruvic acid
164.04735
SubPathwayInteraction108540
SubPathwayReaction
SubPathway108540Reaction
Citric Acid Cycle
SubPathway
SubPathwayInteraction108541
SubPathway108541Reaction
SubPathwayReaction
5858
ChemSpider
CHEBI:47904
ChEBI
3.7.1.2
false
3.7.1.2
4-Fumarylacetoacetic acid + Water → Acetoacetic acid + Fumaric acid
LEFT_TO_RIGHT
Reaction147281
false
Maleylacetoacetic acid → 4-Fumarylacetoacetic acid
LEFT_TO_RIGHT
Adenosine monophosphate
1.13.11.5
false
1.13.11.5
Homogentisic acid + Oxygen → Maleylacetoacetic acid
LEFT_TO_RIGHT
SMILES
OC(=O)C(=O)CC1=CC=C(O)C=C1
CHEBI:16240
ChEBI
Rattus norvegicus
Tyrosine aminotransferase
HMDB0000205
HMDB
PPI
BioCyc
1.13.11.27
false
1.13.11.27
4-Hydroxyphenylpyruvic acid + Oxygen → Carbon dioxide + Homogentisic 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
2.6.1.5
false
2.6.1.5
L-Tyrosine + Oxoglutaric acid ↔ 4-Hydroxyphenylpyruvic acid + L-Glutamic acid
REVERSIBLE
1.14.16.1
false
1.14.16.1
L-Phenylalanine + L-erythro-tetrahydrobiopterin + Oxygen → 4a-Hydroxytetrahydrobiopterin + L-Tyrosine
LEFT_TO_RIGHT
1.4.3.2
false
1.4.3.2
L-Phenylalanine + Oxygen + Water → Ammonia + Hydrogen peroxide + Phenylpyruvic acid
LEFT_TO_RIGHT
1.0
Reaction147272
false
L-Phenylalanine + Oxoglutaric acid ↔ L-Glutamic acid + Phenylpyruvic acid
REVERSIBLE
4444078
ChemSpider
HMDB0000208
HMDB
L-Glutamic acid
CPD-6641
BioCyc
217
ChemSpider
SMILES
OC(=O)C(=O)CC1=CC=CC=C1
Acetoacetic acid
SubPathwayInput
SMILES
OC(=O)CCC(=O)C(O)=O
CHEBI:30915
ChEBI
124-38-9
CAS
GO:0005764
GENE ONTOLOGY
94
ChemSpider
C00007
KEGG Compound
1.0
451-13-8
CAS
C00001
KEGG Compound
Phenylalanine--tRNA ligase alpha subunit
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
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
ReactionCatalysis137510
ACTIVATION
ReactionCatalysis137513
ACTIVATION
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
ReactionCatalysis137514
ACTIVATION
222
PubChem-compound
ReactionCatalysis137515
ACTIVATION
C00014
KEGG Compound
ReactionCatalysis137516
ACTIVATION
C00013
KEGG Compound
Catecholamine Biosynthesis
SubPathway
O7P2
Pyrophosphate
173.91193
Q68FT7
UniProt
40270
ChemSpider
PW121992
PathWhiz
1.0
5957
PubChem-compound
4-hydroxyphenylpyruvate dioxygenase
2.0
CHEBI:15999
ChEBI
56-65-5
CAS
C00020
KEGG Compound
C00027
KEGG Compound
C00026
KEGG Compound
C00025
KEGG Compound
Q5EBB6
UniProt
L-Tyrosyl-tRNA(Tyr)
1.0
Maleylacetoacetate isomerase
C00272
KEGG Compound
GO:0005737
GENE ONTOLOGY
2.0
C8H8O6
4-Fumarylacetoacetic acid
200.03209
B0BNI1
UniProt
Aspartate aminotransferase, cytoplasmic
1.0
2.0
Maleylacetoacetate isomerase
Homogentisate 1, 2-dioxygenase
SMP0000006
SMPDB
3-KETOBUTYRATE
BioCyc
10116
TAXONOMY
Q63817
UniProt
328-50-7
CAS
2.0
HMDB0000134
HMDB
Fumarylacetoacetase
Phenylpyruvic acid
AMP
BioCyc
HYDROGEN-PEROXIDE
BioCyc
1.0
C15522
KEGG Compound
44257
PubChem-compound
HMDB0000130
HMDB
HMDB0000250
HMDB
SMILES
[O-]P([O-])(=O)OP([O-])([O-])=O
tRNA(Phe)
14000-31-8
CAS
L-erythro-tetrahydrobiopterin
C9H8O4
4-Hydroxyphenylpyruvic acid
180.04225
Phenylalanine-4-hydroxylase
997
PubChem-compound
CHEBI:29161
ChEBI
Fumarylacetoacetase
C00166
KEGG Compound
ReactionCatalysis137507
ACTIVATION
ReactionCatalysis137508
ACTIVATION
Homogentisate 1, 2-dioxygenase
ReactionCatalysis137509
ACTIVATION
C00164
KEGG Compound
L-Tyrosine
ReactionCatalysis137505
ACTIVATION
ReactionCatalysis137506
ACTIVATION
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
Phenylalanyl-tRNA synthetase subunit beta
Maleylacetoacetate isomerase
SMP0000012
SMPDB
784
PubChem-compound
2.0
10197150
ChemSpider
CHEBI:15422
ChEBI
1.0
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
FUM
BioCyc
Phenylalanine--tRNA ligase
7732-18-5
CAS
62989-33-7
CAS
HMDB0000707
HMDB
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)NC(N)=N2)[C@@H](O)[C@H](C)O
CHEBI:29153
ChEBI
C5H9NO4
L-Glutamic acid
147.05316
156-06-9
CAS
33032
PubChem-compound
60-18-4
CAS
Water
1.0
C10H16N5O13P3
Adenosine triphosphate
506.99576
1.0
4-hydroxyphenylpyruvate dioxygenase
129803
PubChem-compound
CHEBI:16526
ChEBI
CHEBI:17295
ChEBI
4444081
ChemSpider
4a-Hydroxytetrahydrobiopterin
2.0
7782-44-7
CAS
Pyrophosphate
C01036
KEGG Compound
HMDB0001377
HMDB
HMDB0000045
HMDB
SMILES
CC(=O)CC(O)=O
1.0
SMILES
OC(=O)CC(=O)CC(=O)\C=C/C(O)=O
PHE
BioCyc
O88655
UniProt
2.0
962
PubChem-compound
PW000142
PathWhiz
70110-58-6
CAS
1.0
C9H11NO2
L-Phenylalanine
165.07898
2-KETOGLUTARATE
BioCyc
Amine oxidase [flavin-containing] A
tRNA(Phe)
C9H11NO3
L-Tyrosine
181.0739
114908
ChemSpider
CO2
Carbon dioxide
43.98983
C00079
KEGG Compound
972
ChemSpider
SMILES
O=O
1.0
HMDB0001268
HMDB
C00082
KEGG Compound
Oxoglutaric acid
Tyrosine--tRNA ligase, cytoplasmic
HMDB0000051
HMDB
SMILES
N[C@@H](CCC(O)=O)C(O)=O
HMDB0002111
HMDB
C9H15N5O3
L-erythro-tetrahydrobiopterin
241.1175
1.0
CHEBI:15377
ChEBI
Tyrosine aminotransferase
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
CHEBI:15374
ChEBI
C01179
KEGG Compound
SMP0120733
SMPDB
5280398
PubChem-compound
L-Phenylalanyl-tRNA(Phe)
280
PubChem-compound
C01061
KEGG Compound
HMDB0000060
HMDB
Aspartate aminotransferase, cytoplasmic
96
PubChem-compound
Phenylalanine-4-hydroxylase
28613-33-4
CAS
Phenylalanine--tRNA ligase alpha subunit
1.0
CHEBI:15344
ChEBI
156-39-8
CAS
Tyrosine--tRNA ligase, cytoplasmic
644102
PubChem-compound
PW000005
PathWhiz
6083
PubChem-compound
759
ChemSpider
CHEBI:30851
ChEBI
2.0
H2O
Water
18.010565
H2O2
Hydrogen peroxide
34.005478
C9H15N5O4
4a-Hydroxytetrahydrobiopterin
257.1124
Oxygen
Fumarylacetoacetase
Ammonia
541-50-4
CAS
Tyrosine--tRNA ligase, cytoplasmic
Aspartate aminotransferase, cytoplasmic
4-hydroxyphenylpyruvate dioxygenase
G3V6C2
UniProt
1.0
CHEBI:17895
ChEBI
Phenylalanyl-tRNA synthetase subunit beta
977
PubChem-compound
5698-52-2
CAS
1.0
CHEBI:29182
ChEBI
HMDB0001967
HMDB
C00544
KEGG Compound
1.0
SMILES
OC(=O)\C=C\C(O)=O
1.0
1.0
CHEBI:29184
ChEBI
979
PubChem-compound
Phenylalanine-4-hydroxylase
SMILES
OC(=O)CC1=C(O)C=CC(O)=C1
4-MALEYL-ACETOACETATE
BioCyc