Reference/ChEBI CHEBI 16015
type: UnificationXref
id: CHEBI:16015
db: ChEBI

Compound/PW C040095 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-erythro-tetrahydrobiopterin
stoichiometric coefficient: 1.0

Reference/CAS 7722-84-1
type: UnificationXref
id: 7722-84-1
db: CAS

Reference/PubChem-compound 6140
type: UnificationXref
id: 6140
db: PubChem-compound

Phenylalanine-4-hydroxylase
type: Protein
cellular location: Cytosol
entity reference: Phenylalanine-4-hydroxylase

Reference/ChemSpider 937
type: UnificationXref
id: 937
db: ChemSpider

Reference/ChEBI CHEBI 16134
type: UnificationXref
id: CHEBI:16134
db: ChEBI

Reference/CAS 63-91-2
type: UnificationXref
id: 63-91-2
db: CAS

Reference/HMDB HMDB0003125
type: UnificationXref
id: HMDB0003125
db: HMDB

Phenylalanine--tRNA ligase alpha subunit
type: ProteinReference
xref: Reference/UniProt Q9Y285
organism: BiologicalState26

Compound/PW C000103 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Tyrosine
stoichiometric coefficient: 1.0

Reference/CAS 110-17-8
type: UnificationXref
id: 110-17-8
db: CAS

L-Phenylalanine
type: SmallMolecule
cellular location: Lysosome
entity reference: L-Phenylalanine

L-Tyrosine
type: SmallMolecule
cellular location: Cytosol
entity reference: L-Tyrosine

Reference/ChemSpider 50
type: UnificationXref
id: 50
db: ChemSpider

Compound/PW C000981 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Fumarylacetoacetic acid
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 16027
type: UnificationXref
id: CHEBI:16027
db: ChEBI

Reference/ChemSpider 5833
type: UnificationXref
id: 5833
db: ChemSpider

Reference/BioCyc ATP
type: UnificationXref
id: ATP
db: BioCyc

Aspartate aminotransferase, cytoplasmic
type: ProteinReference
xref: Reference/UniProt P17174
organism: BiologicalState26

Compound/PW C000103 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Tyrosine
stoichiometric coefficient: 1.0

Hydrogen peroxide
type: SmallMolecule
cellular location: Lysosome
entity reference: Hydrogen peroxide

Phenylalanine-4-hydroxylase
type: ProteinReference
xref: Reference/UniProt P00439
organism: BiologicalState26

Reference/ChemSpider 559142
type: UnificationXref
id: 559142
db: ChemSpider

Reference/PathWhiz PW127169
type: UnificationXref
id: PW127169
db: PathWhiz

Aspartate aminotransferase, cytoplasmic
type: Complex
component stoichiometry: Protein/277 Cytosol/Stoichiometry/2.0
cellular location: Cytosol
component: Aspartate aminotransferase, cytoplasmic

Tyrosine aminotransferase
type: ProteinReference
xref: Reference/UniProt P17735
organism: BiologicalState26

Reference/HMDB HMDB0002281
type: UnificationXref
id: HMDB0002281
db: HMDB

4-Fumarylacetoacetic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: 4-Fumarylacetoacetic acid

Reference/UniProt Q9Y285
type: UnificationXref
id: Q9Y285
db: UniProt

Reference/BRENDA TISSUE ONTOLOGY BTO 0000759
type: UnificationXref
id: BTO:0000759
db: BRENDA TISSUE ONTOLOGY

Tyrosine aminotransferase
type: Protein
cellular location: Cytosol
entity reference: Tyrosine aminotransferase

Protein/268 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: Phenylalanine--tRNA ligase alpha subunit
stoichiometric coefficient: 2.0

Reference/ChEBI CHEBI 44747
type: UnificationXref
id: CHEBI:44747
db: ChEBI

Reference/ChemSpider 5742
type: UnificationXref
id: 5742
db: ChemSpider

4F2 cell-surface antigen heavy chain
type: Protein
entity reference: 4F2 cell-surface antigen heavy chain

Reference/ChemSpider 954
type: UnificationXref
id: 954
db: ChemSpider

Reference/ChemSpider 952
type: UnificationXref
id: 952
db: ChemSpider

Aspartate aminotransferase, cytoplasmic
type: Protein
cellular location: Cytosol
entity reference: Aspartate aminotransferase, cytoplasmic

Y+L amino acid transporter 1
type: Protein
entity reference: Y+L amino acid transporter 1

Reference/HMDB HMDB0002052
type: UnificationXref
id: HMDB0002052
db: HMDB

Reference/BioCyc TYR
type: UnificationXref
id: TYR
db: BioCyc

Reference/ChemSpider 5858
type: UnificationXref
id: 5858
db: ChemSpider

Reference/ChEBI CHEBI 47904
type: UnificationXref
id: CHEBI:47904
db: ChEBI

Reference/UniProt P32754
type: UnificationXref
id: P32754
db: UniProt

Adenosine monophosphate
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Adenosine monophosphate

Compound/PW C000104/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Phenylalanine
stoichiometric coefficient: 1.0

SLC3A2/4F2hc Y+L amino acid transporter 1
type: Complex
component: Y+L amino acid transporter 1
component: 4F2 cell-surface antigen heavy chain

Tyrosinemia Type 3 (TYRO3)
type: Pathway
pathway order: PathwayTransport/2513110
pathway order: PathwayEnzymeReaction/2513100
pathway order: PathwayEnzymeReaction/2513101
pathway order: PathwayEnzymeReaction/2513098
pathway order: PathwayEnzymeReaction/2513099
pathway order: PathwayEnzymeReaction/2513096
pathway order: PathwayEnzymeReaction/2513097
pathway order: PathwayEnzymeReaction/2513108
pathway order: PathwayEnzymeReaction/2513106
pathway order: PathwayEnzymeReaction/2513107
pathway order: PathwayEnzymeReaction/2513104
pathway order: PathwaySubPathway/118574
pathway order: PathwayEnzymeReaction/2513105
pathway order: PathwaySubPathway/118573
pathway order: PathwayEnzymeReaction/2513102
pathway order: PathwayEnzymeReaction/2513103
pathway order: PathwayTransport/2513109
pathway order: PathwaySubPathway/118575
pathway component: Reaction79
pathway component: TransportCatalysis5409
pathway component: Catecholamine Biosynthesis
pathway component: Reaction80
pathway component: 1.14.16.1
pathway component: ReactionCatalysis208490
pathway component: 1.4.3.2
pathway component: ReactionCatalysis447
pathway component: 1.13.11.5
pathway component: ReactionCatalysis445
pathway component: ReactionCatalysis446
pathway component: Citric Acid Cycle
pathway component: ReactionCatalysis443
pathway component: ReactionCatalysis444
pathway component: ReactionCatalysis441
pathway component: ReactionCatalysis442
pathway component: 6.1.1.1
pathway component: ReactionCatalysis440
pathway component: 6.1.1.20
pathway component: Transport14617
pathway component: Transport14593
pathway component: ReactionCatalysis438
pathway component: TransportCatalysis5430
pathway component: Tyrosine Metabolism
pathway component: ReactionCatalysis436
pathway component: 1.13.11.27
pathway component: Reaction386
pathway component: ReactionCatalysis437
pathway component: 3.7.1.2
pathway component: ReactionCatalysis435
xref: Reference/SMPDB SMP0125605
xref: Reference/PathWhiz PW127169
organism: Homo sapiens

SubPathwayInteraction118573
type: Interaction
name: SubPathwayReaction
name: SubPathway118573Reaction
participant: L-Tyrosine

Reference/ChEBI CHEBI 16240
type: UnificationXref
id: CHEBI:16240
db: ChEBI

Transport14617
type: Transport
participant stoichiometry: Compound/PW C000103 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000103/Stoichiometry/1.0
right: L-Tyrosine
spontaneous: false
left: L-Tyrosine
name: L-Tyrosine (→) Transport: Homo sapiens, Liver, Hepatocyte, Cytosol to Homo sapiens, Blood Vessel
conversion direction: LEFT_TO_RIGHT

SubPathwayInteraction118574
type: Interaction
name: SubPathway118574Reaction
name: SubPathwayReaction
participant: L-Tyrosine

SubPathwayInteraction118575
type: Interaction
name: SubPathway118575Reaction
name: SubPathwayReaction
participant: Acetoacetic acid
participant: Fumaric acid

Reference/UniProt O43708
type: UnificationXref
id: O43708
db: UniProt

Reference/BioCyc PPI
type: UnificationXref
id: PPI
db: BioCyc

Compound/PW C000414 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Adenosine triphosphate
stoichiometric coefficient: 1.0

Cytosol
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005829

Compound/PW C000170 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyrophosphate
stoichiometric coefficient: 1.0

Maleylacetoacetic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Maleylacetoacetic acid

Reference/ChemSpider 94
type: UnificationXref
id: 94
db: ChemSpider

Compound/PW C000084 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Homogentisic acid
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00007
type: UnificationXref
id: C00007
db: KEGG Compound

Reference/CAS 451-13-8
type: UnificationXref
id: 451-13-8
db: CAS

Reference/KEGG Compound C00001
type: UnificationXref
id: C00001
db: KEGG Compound

Reference/KEGG Compound C00122
type: UnificationXref
id: C00122
db: KEGG Compound

Reference/ChEBI CHEBI 18361
type: UnificationXref
id: CHEBI:18361
db: ChEBI

Reference/KEGG Compound C00002
type: UnificationXref
id: C00002
db: KEGG Compound

Fumaric acid
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Fumaric acid
name: SubPathwayInput

4-Hydroxyphenylpyruvic acid
type: SmallMolecule
cellular location: Lysosome
entity reference: 4-Hydroxyphenylpyruvic acid

4-hydroxyphenylpyruvate dioxygenase
type: Protein
cellular location: Cytosol
entity reference: 4-hydroxyphenylpyruvate dioxygenase

Compound/PW C000170 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyrophosphate
stoichiometric coefficient: 1.0

Adenosine triphosphate
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Adenosine triphosphate

Reference/KEGG Compound C00011
type: UnificationXref
id: C00011
db: KEGG Compound

L-Tyrosine
type: SmallMolecule
cellular location: Cytoplasm
entity reference: L-Tyrosine
name: SubPathwayInput

Reference/KEGG Compound C00014
type: UnificationXref
id: C00014
db: KEGG Compound

Reference/KEGG Compound C00013
type: UnificationXref
id: C00013
db: KEGG Compound

Fumarylacetoacetase
type: Protein
cellular location: Cytosol
entity reference: Fumarylacetoacetase

Protein/677 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: Fumarylacetoacetase
stoichiometric coefficient: 2.0

Tyrosine aminotransferase
type: Complex
component stoichiometry: Protein/271 Cytosol/Stoichiometry/2.0
cellular location: Cytosol
component: Tyrosine aminotransferase

Reference/CAS 56-65-5
type: UnificationXref
id: 56-65-5
db: CAS

Reference/KEGG Compound C00020
type: UnificationXref
id: C00020
db: KEGG Compound

L-Glutamic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: L-Glutamic acid

Reference/KEGG Compound C00027
type: UnificationXref
id: C00027
db: KEGG Compound

Reference/KEGG Compound C00026
type: UnificationXref
id: C00026
db: KEGG Compound

Reference/KEGG Compound C00025
type: UnificationXref
id: C00025
db: KEGG Compound

Protein/573 Cytoplasm/Stoichiometry/2.0
type: Stoichiometry
physical entity: Tyrosine--tRNA ligase, cytoplasmic
stoichiometric coefficient: 2.0

L-Tyrosyl-tRNA(Tyr)
type: Rna
cellular location: Cytoplasm
entity reference: L-Tyrosyl-tRNA(Tyr)

Phenylalanine--tRNA ligase beta subunit
type: ProteinReference
xref: Reference/UniProt Q9NSD9
organism: BiologicalState26

Compound/PW C001065 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: Oxygen
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00272
type: UnificationXref
id: C00272
db: KEGG Compound

4-Fumarylacetoacetic acid
type: SmallMoleculeReference
xref: Reference/BioCyc 4-FUMARYL-ACETOACETATE
xref: Reference/HMDB HMDB0001268
xref: Reference/PubChem-compound 5280398
xref: Reference/KEGG Compound C01061
xref: Reference/ChemSpider 4444081
xref: Reference/ChEBI CHEBI 30907
xref: Reference/CAS 28613-33-4
chemical formula: C8H8O6
molecular weight: 200.03209
structure: ChemicalStructure/PW C000981

L-erythro-tetrahydrobiopterin
type: SmallMolecule
cellular location: Cytosol
entity reference: L-erythro-tetrahydrobiopterin

Compound/PW C000103/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Tyrosine
stoichiometric coefficient: 1.0

BiologicalState26
type: BioSource
cell type: Hepatocyte
xref: Reference/TAXONOMY 9606
name: Homo sapiens, Liver, Hepatocyte
tissue: Liver

4a-Hydroxytetrahydrobiopterin
type: SmallMolecule
cellular location: Cytosol
entity reference: 4a-Hydroxytetrahydrobiopterin

Homogentisate 1,2-dioxygenase
type: ProteinReference
xref: Reference/UniProt Q93099
organism: BiologicalState26

4-hydroxyphenylpyruvate dioxygenase
type: ProteinReference
xref: Reference/UniProt P32754
organism: BiologicalState26

TransportCatalysis5409
type: Catalysis
controller: SLC3A2/4F2hc Y+L amino acid transporter 1
controlled: Transport14593
control type: ACTIVATION

Reference/CAS 328-50-7
type: UnificationXref
id: 328-50-7
db: CAS

Reference/HMDB HMDB0000134
type: UnificationXref
id: HMDB0000134
db: HMDB

Phenylpyruvic acid
type: SmallMolecule
cellular location: Lysosome
entity reference: Phenylpyruvic acid

Reference/BioCyc AMP
type: UnificationXref
id: AMP
db: BioCyc

Reference/BioCyc HYDROGEN-PEROXIDE
type: UnificationXref
id: HYDROGEN-PEROXIDE
db: BioCyc

Reference/KEGG Compound C15522
type: UnificationXref
id: C15522
db: KEGG Compound

Reference/HMDB HMDB0000130
type: UnificationXref
id: HMDB0000130
db: HMDB

Reference/HMDB HMDB0000250
type: UnificationXref
id: HMDB0000250
db: HMDB

ChemicalStructure/PW C000170
type: ChemicalStructure
structure format: SMILES
structure data: OP(O)(=O)OP(O)(O)=O

tRNA(Phe)
type: RnaReference
xref: Reference/ChEBI CHEBI 29184
organism: BiologicalState26

4-Hydroxyphenylpyruvic acid
type: SmallMoleculeReference
xref: Reference/BioCyc P-HYDROXY-PHENYLPYRUVATE
xref: Reference/CAS 156-39-8
xref: Reference/ChEBI CHEBI 15999
xref: Reference/PubChem-compound 979
xref: Reference/HMDB HMDB0000707
xref: Reference/ChemSpider 954
xref: Reference/KEGG Compound C01179
chemical formula: C9H8O4
molecular weight: 180.04225
structure: ChemicalStructure/PW C000558

Reference/PubChem-compound 997
type: UnificationXref
id: 997
db: PubChem-compound

Reference/ChEBI CHEBI 29161
type: UnificationXref
id: CHEBI:29161
db: ChEBI

Protein/496 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: Phenylalanine--tRNA ligase beta subunit
stoichiometric coefficient: 2.0

Protein/762 Cytoplasm/Stoichiometry/2.0
type: Stoichiometry
physical entity: Maleylacetoacetate isomerase
stoichiometric coefficient: 2.0

Reference/KEGG Compound C00166
type: UnificationXref
id: C00166
db: KEGG Compound

L-Phenylalanyl-tRNA(Phe)
type: Rna
cellular location: Cytosol
entity reference: L-Phenylalanyl-tRNA(Phe)

Reference/KEGG Compound C00164
type: UnificationXref
id: C00164
db: KEGG Compound

L-Tyrosine
type: SmallMolecule
cellular location: Lysosome
entity reference: L-Tyrosine

Fumarylacetoacetase
type: ProteinReference
xref: Reference/UniProt P16930
organism: BiologicalState26

Reference/ChemSpider 763
type: UnificationXref
id: 763
db: ChemSpider

L-Phenylalanyl-tRNA(Phe)
type: RnaReference
xref: Reference/ChEBI CHEBI 29153
organism: BiologicalState26

Compound/PW C000134 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Oxoglutaric acid
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000148
type: UnificationXref
id: HMDB0000148
db: HMDB

L-Tyrosyl-tRNA(Tyr)
type: RnaReference
xref: Reference/ChEBI CHEBI 29161
organism: Homo sapiens

tRNA(Phe)
type: Rna
cellular location: Cytosol
entity reference: tRNA(Phe)

tRNA(Tyr)
type: RnaReference
xref: Reference/ChEBI CHEBI 29182
organism: Homo sapiens

Tyrosine--tRNA ligase, cytoplasmic
type: Protein
cellular location: Cytoplasm
entity reference: Tyrosine--tRNA ligase, cytoplasmic

L-Phenylalanine
type: SmallMolecule
cellular location: Cytosol
entity reference: L-Phenylalanine

Reference/PubChem-compound 784
type: UnificationXref
id: 784
db: PubChem-compound

Reference/ChemSpider 10197150
type: UnificationXref
id: 10197150
db: ChemSpider

Compound/PW C001783 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydrogen peroxide
stoichiometric coefficient: 1.0

Reference/BioCyc FUM
type: UnificationXref
id: FUM
db: BioCyc

Reference/CAS 7732-18-5
type: UnificationXref
id: 7732-18-5
db: CAS

Reference/CAS 62989-33-7
type: UnificationXref
id: 62989-33-7
db: CAS

Reference/PubChem-compound 780
type: UnificationXref
id: 780
db: PubChem-compound

Reference/ChEBI CHEBI 18012
type: UnificationXref
id: CHEBI:18012
db: ChEBI

Reference/CAS 61-19-8
type: UnificationXref
id: 61-19-8
db: CAS

4-Hydroxyphenylpyruvic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: 4-Hydroxyphenylpyruvic acid

ChemicalStructure/PW C000032
type: ChemicalStructure
structure format: SMILES

Reference/HMDB HMDB0000158
type: UnificationXref
id: HMDB0000158
db: HMDB

Reference/HMDB HMDB0000159
type: UnificationXref
id: HMDB0000159
db: HMDB

Reference/BioCyc AMMONIA
type: UnificationXref
id: AMMONIA
db: BioCyc

ChemicalStructure/PW C000035
type: ChemicalStructure
structure format: SMILES
structure data: N

Reference/CAS 56-86-0
type: UnificationXref
id: 56-86-0
db: CAS

Reference/ChEBI CHEBI 29153
type: UnificationXref
id: CHEBI:29153
db: ChEBI

Reference/CAS 156-06-9
type: UnificationXref
id: 156-06-9
db: CAS

Adenosine triphosphate
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15422
xref: Reference/PubChem-compound 5957
xref: Reference/HMDB HMDB0000538
xref: Reference/CAS 56-65-5
xref: Reference/ChemSpider 5742
xref: Reference/BioCyc ATP
xref: Reference/KEGG Compound C00002
chemical formula: C10H16N5O13P3
molecular weight: 506.99576
structure: ChemicalStructure/PW C000414

Reference/ChEBI CHEBI 17295
type: UnificationXref
id: CHEBI:17295
db: ChEBI

Reference/ChemSpider 4444081
type: UnificationXref
id: 4444081
db: ChemSpider

Pyrophosphate
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Pyrophosphate

Reference/KEGG Compound C01036
type: UnificationXref
id: C01036
db: KEGG Compound

Reference/HMDB HMDB0001377
type: UnificationXref
id: HMDB0001377
db: HMDB

Tyrosine--tRNA ligase, cytoplasmic
type: ProteinReference
xref: Reference/UniProt P54577
organism: Homo sapiens

Reference/HMDB HMDB0000045
type: UnificationXref
id: HMDB0000045
db: HMDB

ChemicalStructure/PW C000042
type: ChemicalStructure
structure format: SMILES
structure data: CC(=O)CC(O)=O

RNA/20 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Phenylalanyl-tRNA(Phe)
stoichiometric coefficient: 1.0

ChemicalStructure/PW C001376
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CC(=O)CC(=O)\C=C/C(O)=O

Reference/BioCyc PHE
type: UnificationXref
id: PHE
db: BioCyc

TransportCatalysis5430
type: Catalysis
controller: SLC3A2/4F2hc Y+L amino acid transporter 1
controlled: Transport14617
control type: ACTIVATION

Reference/PubChem-compound 962
type: UnificationXref
id: 962
db: PubChem-compound

RNA/22 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Tyrosyl-tRNA(Tyr)
stoichiometric coefficient: 1.0

L-Phenylalanine
type: SmallMoleculeReference
xref: Reference/PubChem-compound 6140
xref: Reference/HMDB HMDB0000159
xref: Reference/ChemSpider 5910
xref: Reference/ChEBI CHEBI 17295
xref: Reference/BioCyc PHE
xref: Reference/CAS 63-91-2
xref: Reference/KEGG Compound C00079
chemical formula: C9H11NO2
molecular weight: 165.07898
structure: ChemicalStructure/PW C000104

L-Tyrosine
type: SmallMoleculeReference
xref: Reference/CAS 60-18-4
xref: Reference/PubChem-compound 6057
xref: Reference/HMDB HMDB0000158
xref: Reference/ChEBI CHEBI 17895
xref: Reference/KEGG Compound C00082
xref: Reference/ChemSpider 5833
xref: Reference/BioCyc TYR
chemical formula: C9H11NO3
molecular weight: 181.0739
structure: ChemicalStructure/PW C000103

Reference/ChemSpider 114908
type: UnificationXref
id: 114908
db: ChemSpider

Carbon dioxide
type: SmallMoleculeReference
xref: Reference/PubChem-compound 280
xref: Reference/ChEBI CHEBI 16526
xref: Reference/ChemSpider 274
xref: Reference/HMDB HMDB0001967
xref: Reference/CAS 124-38-9
xref: Reference/KEGG Compound C00011
chemical formula: CO2
molecular weight: 43.98983
structure: ChemicalStructure/PW C001316

Reference/KEGG Compound C00079
type: UnificationXref
id: C00079
db: KEGG Compound

Reference/ChemSpider 972
type: UnificationXref
id: 972
db: ChemSpider

ChemicalStructure/PW C001065
type: ChemicalStructure
structure format: SMILES
structure data: O=O

Reference/HMDB HMDB0001268
type: UnificationXref
id: HMDB0001268
db: HMDB

Maleylacetoacetate isomerase
type: Complex
component stoichiometry: Protein/762 Cytoplasm/Stoichiometry/2.0
cellular location: Cytoplasm
component: Maleylacetoacetate isomerase

Reference/KEGG Compound C00082
type: UnificationXref
id: C00082
db: KEGG Compound

Protein/274 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: Phenylalanine-4-hydroxylase
stoichiometric coefficient: 2.0

Reference/HMDB HMDB0000051
type: UnificationXref
id: HMDB0000051
db: HMDB

ChemicalStructure/PW C000095
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CCC(O)=O)C(O)=O

Reference/HMDB HMDB0002111
type: UnificationXref
id: HMDB0002111
db: HMDB

L-erythro-tetrahydrobiopterin
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00272
xref: Reference/ChemSpider 40270
xref: Reference/ChEBI CHEBI 59560
xref: Reference/PubChem-compound 44257
xref: Reference/CAS 62989-33-7
xref: Reference/HMDB HMDB0059658
chemical formula: C9H15N5O3
molecular weight: 241.1175
structure: ChemicalStructure/PW C040095

Phenylalanine-4-hydroxylase
type: Complex
component stoichiometry: Protein/274 Cytosol/Stoichiometry/2.0
cellular location: Cytosol
component: Phenylalanine-4-hydroxylase

Reference/PubChem-compound 6057
type: UnificationXref
id: 6057
db: PubChem-compound

Reference/UniProt Q9UM01
type: UnificationXref
id: Q9UM01
db: UniProt

Compound/PW C000104 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Phenylalanine
stoichiometric coefficient: 1.0

Reference/ChemSpider 5910
type: UnificationXref
id: 5910
db: ChemSpider

Compound/PW C000035 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: Ammonia
stoichiometric coefficient: 1.0

Reference/PubChem-compound 5280393
type: UnificationXref
id: 5280393
db: PubChem-compound

Reference/UniProt Q93099
type: UnificationXref
id: Q93099
db: UniProt

Reference/KEGG Compound C01179
type: UnificationXref
id: C01179
db: KEGG Compound

Reference/PubChem-compound 5280398
type: UnificationXref
id: 5280398
db: PubChem-compound

Reference/KEGG Compound C01061
type: UnificationXref
id: C01061
db: KEGG Compound

Reference/HMDB HMDB0000060
type: UnificationXref
id: HMDB0000060
db: HMDB

Reference/PubChem-compound 96
type: UnificationXref
id: 96
db: PubChem-compound

Homogentisic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Homogentisic acid

Reference/CAS 28613-33-4
type: UnificationXref
id: 28613-33-4
db: CAS

Reference/CAS 156-39-8
type: UnificationXref
id: 156-39-8
db: CAS

Reference/PubChem-compound 644102
type: UnificationXref
id: 644102
db: PubChem-compound

Acetoacetic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Acetoacetic acid

Reference/PubChem-compound 6083
type: UnificationXref
id: 6083
db: PubChem-compound

Reference/ChemSpider 759
type: UnificationXref
id: 759
db: ChemSpider

Maleylacetoacetate isomerase
type: ProteinReference
xref: Reference/UniProt O43708
organism: Homo sapiens

Water
type: SmallMoleculeReference
xref: Reference/PubChem-compound 962
xref: Reference/ChEBI CHEBI 15377
xref: Reference/KEGG Compound C00001
xref: Reference/ChemSpider 937
xref: Reference/HMDB HMDB0002111
xref: Reference/CAS 7732-18-5
chemical formula: H2O
molecular weight: 18.010565
structure: ChemicalStructure/PW C001420

Hydrogen peroxide
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0003125
xref: Reference/PubChem-compound 784
xref: Reference/ChemSpider 763
xref: Reference/CAS 7722-84-1
xref: Reference/BioCyc HYDROGEN-PEROXIDE
xref: Reference/ChEBI CHEBI 16240
xref: Reference/KEGG Compound C00027
chemical formula: H2O2
molecular weight: 34.005478
structure: ChemicalStructure/PW C001783

4a-Hydroxytetrahydrobiopterin
type: SmallMoleculeReference
xref: Reference/CAS 70110-58-6
xref: Reference/PubChem-compound 129803
xref: Reference/KEGG Compound C15522
xref: Reference/ChemSpider 114908
xref: Reference/HMDB HMDB0002281
xref: Reference/ChEBI CHEBI 15374
chemical formula: C9H15N5O4
molecular weight: 257.1124
structure: ChemicalStructure/PW C001541

Oxygen
type: SmallMolecule
cellular location: Lysosome
entity reference: Oxygen

Ammonia
type: SmallMolecule
cellular location: Lysosome
entity reference: Ammonia

Compound/PW C000558 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Hydroxyphenylpyruvic acid
stoichiometric coefficient: 1.0

Compound/PW C000131 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: Phenylpyruvic acid
stoichiometric coefficient: 1.0

Reference/PubChem-compound 977
type: UnificationXref
id: 977
db: PubChem-compound

Reference/ChEBI CHEBI 29182
type: UnificationXref
id: CHEBI:29182
db: ChEBI

Compound/PW C001316 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Carbon dioxide
stoichiometric coefficient: 1.0

L-Phenylalanine
type: SmallMolecule
entity reference: L-Phenylalanine

L-Tyrosine
type: SmallMolecule
entity reference: L-Tyrosine

Reference/UniProt P17735
type: UnificationXref
id: P17735
db: UniProt

Compound/PW C001420 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: Water
stoichiometric coefficient: 1.0

ChemicalStructure/PW C000088
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)\C=C\C(O)=O

Reference/SMPDB SMP0125605
type: UnificationXref
id: SMP0125605
db: SMPDB

Reference/ChEBI CHEBI 29184
type: UnificationXref
id: CHEBI:29184
db: ChEBI

Reference/PubChem-compound 979
type: UnificationXref
id: 979
db: PubChem-compound

ChemicalStructure/PW C000084
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CC1=C(O)C=CC(O)=C1

Reference/ChemSpider 30572
type: UnificationXref
id: 30572
db: ChemSpider

Reference/UniProt Q96RQ9
type: UnificationXref
id: Q96RQ9
db: UniProt

Reference/UniProt P08195
type: UnificationXref
id: P08195
db: UniProt

Reference/BioCyc P-HYDROXY-PHENYLPYRUVATE
type: UnificationXref
id: P-HYDROXY-PHENYLPYRUVATE
db: BioCyc

Adenosine monophosphate
type: SmallMolecule
cellular location: Cytosol
entity reference: Adenosine monophosphate

Reference/UniProt P16930
type: UnificationXref
id: P16930
db: UniProt

Reference/PubChem-compound 21883788
type: UnificationXref
id: 21883788
db: PubChem-compound

ChemicalStructure/PW C001420
type: ChemicalStructure
structure format: SMILES
structure data: O

ChemicalStructure/PW C001783
type: ChemicalStructure
structure format: SMILES
structure data: OO

ChemicalStructure/PW C001541
type: ChemicalStructure
structure format: SMILES

Ammonia
type: SmallMoleculeReference
xref: Reference/BioCyc AMMONIA
xref: Reference/CAS 7664-41-7
xref: Reference/HMDB HMDB0000051
xref: Reference/PubChem-compound 222
xref: Reference/KEGG Compound C00014
xref: Reference/ChEBI CHEBI 16134
xref: Reference/ChemSpider 217
chemical formula: H3N
molecular weight: 17.026548
structure: ChemicalStructure/PW C000035

Adenosine monophosphate
type: SmallMoleculeReference
xref: Reference/CAS 61-19-8
xref: Reference/ChemSpider 5858
xref: Reference/HMDB HMDB0000045
xref: Reference/BioCyc AMP
xref: Reference/ChEBI CHEBI 16027
xref: Reference/PubChem-compound 6083
xref: Reference/KEGG Compound C00020
chemical formula: C10H14N5O7P
molecular weight: 347.06308
structure: ChemicalStructure/PW C000032

L-amino-acid oxidase
type: ProteinReference
xref: Reference/UniProt Q96RQ9
organism: BiologicalState26

Compound/PW C001065 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Oxygen
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000538
type: UnificationXref
id: HMDB0000538
db: HMDB

ChemicalStructure/PW C000103
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CC1=CC=C(O)C=C1)C(O)=O

Reference/PubChem-compound 51
type: UnificationXref
id: 51
db: PubChem-compound

Maleylacetoacetic acid
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 47904
xref: Reference/ChemSpider 4444078
xref: Reference/CAS 5698-52-2
xref: Reference/HMDB HMDB0002052
xref: Reference/PubChem-compound 5280393
xref: Reference/KEGG Compound C01036
xref: Reference/BioCyc 4-MALEYL-ACETOACETATE
chemical formula: C8H8O6
molecular weight: 200.03209
structure: ChemicalStructure/PW C001376

Phenylalanine--tRNA ligase alpha subunit
type: Protein
cellular location: Cytosol
entity reference: Phenylalanine--tRNA ligase alpha subunit

Acetoacetic acid
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15344
xref: Reference/HMDB HMDB0000060
xref: Reference/BioCyc 3-KETOBUTYRATE
xref: Reference/KEGG Compound C00164
xref: Reference/PubChem-compound 96
xref: Reference/CAS 541-50-4
xref: Reference/ChemSpider 94
chemical formula: C4H6O3
molecular weight: 102.03169
structure: ChemicalStructure/PW C000042

Reference/CELL TYPE ONTOLOGY CL 0000182
type: UnificationXref
id: CL:0000182
db: CELL TYPE ONTOLOGY

Lysosome
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005764

Reference/ChEBI CHEBI 59560
type: UnificationXref
id: CHEBI:59560
db: ChEBI

Protein/271 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: Tyrosine aminotransferase
stoichiometric coefficient: 2.0

tRNA(Tyr)
type: Rna
cellular location: Cytoplasm
entity reference: tRNA(Tyr)

Reference/GENE ONTOLOGY GO 0005829
type: UnificationXref
id: GO:0005829
db: GENE ONTOLOGY

4-Fumarylacetoacetic acid
type: SmallMolecule
cellular location: Cytoplasm
entity reference: 4-Fumarylacetoacetic acid

Reference/ChemSpider 274
type: UnificationXref
id: 274
db: ChemSpider

Oxoglutaric acid
type: SmallMoleculeReference
xref: Reference/CAS 328-50-7
xref: Reference/PubChem-compound 51
xref: Reference/BioCyc 2-KETOGLUTARATE
xref: Reference/HMDB HMDB0000208
xref: Reference/ChEBI CHEBI 30915
xref: Reference/KEGG Compound C00026
xref: Reference/ChemSpider 50
chemical formula: C5H6O5
molecular weight: 146.02153
structure: ChemicalStructure/PW C000134

Phenylpyruvic acid
type: SmallMoleculeReference
xref: Reference/CAS 156-06-9
xref: Reference/ChEBI CHEBI 30851
xref: Reference/ChemSpider 972
xref: Reference/BioCyc PHENYL-PYRUVATE
xref: Reference/PubChem-compound 997
xref: Reference/HMDB HMDB0000205
xref: Reference/KEGG Compound C00166
chemical formula: C9H8O3
molecular weight: 164.04735
structure: ChemicalStructure/PW C000131

Citric Acid Cycle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway118575
pathway component: SubPathwayInteraction118575
xref: Reference/PathWhiz PW000005
xref: Reference/SMPDB SMP0000057
name: SubPathway

Compound/PW C000088 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Fumaric acid
stoichiometric coefficient: 1.0

Pyrophosphate
type: SmallMolecule
cellular location: Cytosol
entity reference: Pyrophosphate

ChemicalStructure/PW C000558
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)C(=O)CC1=CC=C(O)C=C1

Maleylacetoacetate isomerase
type: Protein
cellular location: Cytoplasm
entity reference: Maleylacetoacetate isomerase

Phenylalanine--tRNA ligase
type: Complex
component stoichiometry: Protein/268 Cytosol/Stoichiometry/2.0
component stoichiometry: Protein/496 Cytosol/Stoichiometry/2.0
cellular location: Cytosol
component: Phenylalanine--tRNA ligase alpha subunit
component: Phenylalanine--tRNA ligase beta subunit

Reference/HMDB HMDB0000205
type: UnificationXref
id: HMDB0000205
db: HMDB

Reference/ChemSpider 4444078
type: UnificationXref
id: 4444078
db: ChemSpider

Reaction79
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000104 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000131 Cytosol/Stoichiometry/1.0
right: Phenylpyruvic acid
right: L-Glutamic acid
spontaneous: false
left: L-Phenylalanine
left: Oxoglutaric acid
name: L-Phenylalanine + Oxoglutaric acid ↔ L-Glutamic acid + Phenylpyruvic acid
conversion direction: REVERSIBLE

Fumaric acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Fumaric acid

Compound/PW C001376 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Maleylacetoacetic acid
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000208
type: UnificationXref
id: HMDB0000208
db: HMDB

Carbon dioxide
type: SmallMolecule
cellular location: Cytosol
entity reference: Carbon dioxide

Reference/BioCyc CPD-6641
type: UnificationXref
id: CPD-6641
db: BioCyc

Reference/ChemSpider 217
type: UnificationXref
id: 217
db: ChemSpider

Reference/UniProt P00439
type: UnificationXref
id: P00439
db: UniProt

ChemicalStructure/PW C000131
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)C(=O)CC1=CC=CC=C1

Acetoacetic acid
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Acetoacetic acid
name: SubPathwayInput

Reaction80
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000103 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000558 Cytosol/Stoichiometry/1.0
right: 4-Hydroxyphenylpyruvic acid
right: L-Glutamic acid
spontaneous: false
left: Oxoglutaric acid
left: L-Tyrosine
name: L-Tyrosine + Oxoglutaric acid ↔ 4-Hydroxyphenylpyruvic acid + L-Glutamic acid
conversion direction: REVERSIBLE

ChemicalStructure/PW C000134
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CCC(=O)C(O)=O

Reference/ChEBI CHEBI 30915
type: UnificationXref
id: CHEBI:30915
db: ChEBI

Reference/CAS 124-38-9
type: UnificationXref
id: 124-38-9
db: CAS

Reference/GENE ONTOLOGY GO 0005764
type: UnificationXref
id: GO:0005764
db: GENE ONTOLOGY

Fumarylacetoacetase
type: Complex
component stoichiometry: Protein/677 Cytosol/Stoichiometry/2.0
cellular location: Cytosol
component: Fumarylacetoacetase

Liver
type: TissueVocabulary
xref: Reference/BRENDA TISSUE ONTOLOGY BTO 0000759

Reference/HMDB HMDB0059658
type: UnificationXref
id: HMDB0059658
db: HMDB

Compound/PW C000103 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Tyrosine
stoichiometric coefficient: 1.0

Reference/BioCyc 4-FUMARYL-ACETOACETATE
type: UnificationXref
id: 4-FUMARYL-ACETOACETATE
db: BioCyc

Reference/BioCyc PHENYL-PYRUVATE
type: UnificationXref
id: PHENYL-PYRUVATE
db: BioCyc

Reference/BioCyc GLT
type: UnificationXref
id: GLT
db: BioCyc

Reference/CAS 7664-41-7
type: UnificationXref
id: 7664-41-7
db: CAS

Fumaric acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000134
xref: Reference/ChemSpider 10197150
xref: Reference/CAS 110-17-8
xref: Reference/PubChem-compound 21883788
xref: Reference/KEGG Compound C00122
xref: Reference/BioCyc FUM
xref: Reference/ChEBI CHEBI 18012
chemical formula: C4H4O4
molecular weight: 116.010956
structure: ChemicalStructure/PW C000088

Tyrosine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway118574
pathway component: SubPathwayInteraction118574
xref: Reference/PathWhiz PW000142
xref: Reference/SMPDB SMP0000006
name: SubPathway

Reference/SMPDB SMP0000057
type: UnificationXref
id: SMP0000057
db: SMPDB

Reference/ChEBI CHEBI 30907
type: UnificationXref
id: CHEBI:30907
db: ChEBI

Homogentisic acid
type: SmallMoleculeReference
xref: Reference/CAS 451-13-8
xref: Reference/ChEBI CHEBI 44747
xref: Reference/ChemSpider 759
xref: Reference/HMDB HMDB0000130
xref: Reference/KEGG Compound C00544
xref: Reference/PubChem-compound 780
chemical formula: C8H8O4
molecular weight: 168.04225
structure: ChemicalStructure/PW C000084

Oxoglutaric acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Oxoglutaric acid

Maleylacetoacetic acid
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Maleylacetoacetic acid

ChemicalStructure/PW C001316
type: ChemicalStructure
structure format: SMILES
structure data: O=C=O

ChemicalStructure/PW C000104
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CC1=CC=CC=C1)C(O)=O

4-hydroxyphenylpyruvate dioxygenase
type: Complex
component stoichiometry: Protein/786 Cytosol/Stoichiometry/2.0
cellular location: Cytosol
component: 4-hydroxyphenylpyruvate dioxygenase

Reference/PubChem-compound 222
type: UnificationXref
id: 222
db: PubChem-compound

Catecholamine Biosynthesis
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway118573
pathway component: SubPathwayInteraction118573
xref: Reference/PathWhiz PW000017
xref: Reference/SMPDB SMP0000012
name: SubPathway

Pyrophosphate
type: SmallMoleculeReference
xref: Reference/PubChem-compound 644102
xref: Reference/BioCyc PPI
xref: Reference/ChemSpider 559142
xref: Reference/ChEBI CHEBI 18361
xref: Reference/HMDB HMDB0000250
xref: Reference/CAS 14000-31-8
xref: Reference/KEGG Compound C00013
chemical formula: H4O7P2
molecular weight: 177.94322
structure: ChemicalStructure/PW C000170

Reference/ChemSpider 40270
type: UnificationXref
id: 40270
db: ChemSpider

Reference/PubChem-compound 5957
type: UnificationXref
id: 5957
db: PubChem-compound

Hepatocyte
type: CellVocabulary
xref: Reference/CELL TYPE ONTOLOGY CL 0000182

6.1.1.1
type: BiochemicalReaction
participant stoichiometry: RNA/22 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: RNA/21 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000170 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000103 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000032 Cytoplasm/Stoichiometry/1.0
right: Adenosine monophosphate
right: L-Tyrosyl-tRNA(Tyr)
right: Pyrophosphate
spontaneous: false
EC number: 6.1.1.1
left: Adenosine triphosphate
left: tRNA(Tyr)
left: L-Tyrosine
name: Adenosine triphosphate + L-Tyrosine + tRNA(Tyr) → Adenosine monophosphate + L-Tyrosyl-tRNA(Tyr) + Pyrophosphate
conversion direction: LEFT_TO_RIGHT

Reference/ChEBI CHEBI 15999
type: UnificationXref
id: CHEBI:15999
db: ChEBI

6.1.1.20
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000414 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000104 Cytosol/Stoichiometry/1.0
participant stoichiometry: RNA/19 Cytosol/Stoichiometry/1.0
participant stoichiometry: RNA/20 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000032 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000170 Cytosol/Stoichiometry/1.0
right: Adenosine monophosphate
right: Pyrophosphate
right: L-Phenylalanyl-tRNA(Phe)
spontaneous: false
EC number: 6.1.1.20
left: L-Phenylalanine
left: Adenosine triphosphate
left: tRNA(Phe)
name: Adenosine triphosphate + L-Phenylalanine + tRNA(Phe) → Adenosine monophosphate + L-Phenylalanyl-tRNA(Phe) + Pyrophosphate
conversion direction: LEFT_TO_RIGHT

4F2 cell-surface antigen heavy chain
type: ProteinReference
xref: Reference/UniProt P08195

Y+L amino acid transporter 1
type: ProteinReference
xref: Reference/UniProt Q9UM01

Phenylpyruvic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Phenylpyruvic acid

Adenosine triphosphate
type: SmallMolecule
cellular location: Cytosol
entity reference: Adenosine triphosphate

Protein/786 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: 4-hydroxyphenylpyruvate dioxygenase
stoichiometric coefficient: 2.0

Reference/GENE ONTOLOGY GO 0005737
type: UnificationXref
id: GO:0005737
db: GENE ONTOLOGY

RNA/19 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: tRNA(Phe)
stoichiometric coefficient: 1.0

Compound/PW C000104 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Phenylalanine
stoichiometric coefficient: 1.0

Tyrosine--tRNA ligase, cytoplasmic
type: Complex
component stoichiometry: Protein/573 Cytoplasm/Stoichiometry/2.0
cellular location: Cytoplasm
component: Tyrosine--tRNA ligase, cytoplasmic

Reference/SMPDB SMP0000006
type: UnificationXref
id: SMP0000006
db: SMPDB

Reference/BioCyc 3-KETOBUTYRATE
type: UnificationXref
id: 3-KETOBUTYRATE
db: BioCyc

Reference/UniProt Q9NSD9
type: UnificationXref
id: Q9NSD9
db: UniProt

Reference/PubChem-compound 44257
type: UnificationXref
id: 44257
db: PubChem-compound

Reference/CAS 14000-31-8
type: UnificationXref
id: 14000-31-8
db: CAS

Protein/277 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: Aspartate aminotransferase, cytoplasmic
stoichiometric coefficient: 2.0

Phenylalanine--tRNA ligase beta subunit
type: Protein
cellular location: Cytosol
entity reference: Phenylalanine--tRNA ligase beta subunit

Transport14593
type: Transport
participant stoichiometry: Compound/PW C000104/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000104 Cytosol/Stoichiometry/1.0
right: L-Phenylalanine
spontaneous: false
left: L-Phenylalanine
name: L-Phenylalanine (→) Transport: Homo sapiens, Liver, Hepatocyte, Cytosol to Homo sapiens, Blood Vessel
conversion direction: LEFT_TO_RIGHT

ChemicalStructure/PW C000981
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CC(=O)CC(=O)\C=C\C(O)=O

Cytoplasm
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005737

Reference/SMPDB SMP0000012
type: UnificationXref
id: SMP0000012
db: SMPDB

Compound/PW C000032 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Adenosine monophosphate
stoichiometric coefficient: 1.0

Compound/PW C001541 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4a-Hydroxytetrahydrobiopterin
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 15422
type: UnificationXref
id: CHEBI:15422
db: ChEBI

Homo sapiens
type: BioSource
xref: Reference/TAXONOMY 9606

Reference/HMDB HMDB0000707
type: UnificationXref
id: HMDB0000707
db: HMDB

Oxygen
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001377
xref: Reference/ChemSpider 952
xref: Reference/KEGG Compound C00007
xref: Reference/ChEBI CHEBI 15379
xref: Reference/PubChem-compound 977
xref: Reference/BioCyc CPD-6641
xref: Reference/CAS 7782-44-7
chemical formula: O2
molecular weight: 31.98983
structure: ChemicalStructure/PW C001065

ChemicalStructure/PW C040095
type: ChemicalStructure
structure format: SMILES

ReactionCatalysis447
type: Catalysis
controller: Fumarylacetoacetase
controlled: 3.7.1.2
control type: ACTIVATION

ReactionCatalysis445
type: Catalysis
controller: Homogentisate 1,2-dioxygenase
controlled: 1.13.11.5
control type: ACTIVATION

L-Glutamic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 33032
xref: Reference/ChEBI CHEBI 16015
xref: Reference/ChemSpider 30572
xref: Reference/HMDB HMDB0000148
xref: Reference/CAS 56-86-0
xref: Reference/BioCyc GLT
xref: Reference/KEGG Compound C00025
chemical formula: C5H9NO4
molecular weight: 147.05316
structure: ChemicalStructure/PW C000095

ReactionCatalysis446
type: Catalysis
controller: Maleylacetoacetate isomerase
controlled: Reaction386
control type: ACTIVATION

Water
type: SmallMolecule
cellular location: Cytosol
entity reference: Water

Reference/PubChem-compound 33032
type: UnificationXref
id: 33032
db: PubChem-compound

ReactionCatalysis443
type: Catalysis
controller: Tyrosine--tRNA ligase, cytoplasmic
controlled: 6.1.1.1
control type: ACTIVATION

Reference/CAS 60-18-4
type: UnificationXref
id: 60-18-4
db: CAS

ReactionCatalysis444
type: Catalysis
controller: 4-hydroxyphenylpyruvate dioxygenase
controlled: 1.13.11.27
control type: ACTIVATION

Water
type: SmallMolecule
cellular location: Lysosome
entity reference: Water

ReactionCatalysis441
type: Catalysis
controller: L-amino-acid oxidase
controlled: 1.4.3.2
control type: ACTIVATION

ReactionCatalysis442
type: Catalysis
controller: Phenylalanine--tRNA ligase
controlled: 6.1.1.20
control type: ACTIVATION

ReactionCatalysis440
type: Catalysis
controller: Tyrosine aminotransferase
controlled: Reaction80
control type: ACTIVATION

Compound/PW C000095 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Glutamic acid
stoichiometric coefficient: 1.0

Reference/PubChem-compound 129803
type: UnificationXref
id: 129803
db: PubChem-compound

Reference/ChEBI CHEBI 16526
type: UnificationXref
id: CHEBI:16526
db: ChEBI

Reference/CAS 7782-44-7
type: UnificationXref
id: 7782-44-7
db: CAS

Compound/PW C000981 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Fumarylacetoacetic acid
stoichiometric coefficient: 1.0

ReactionCatalysis438
type: Catalysis
controller: Phenylalanine-4-hydroxylase
controlled: 1.14.16.1
control type: ACTIVATION

Compound/PW C001420 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Water
stoichiometric coefficient: 1.0

ReactionCatalysis436
type: Catalysis
controller: Tyrosine aminotransferase
controlled: Reaction79
control type: ACTIVATION

Reaction386
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001376 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000981 Cytoplasm/Stoichiometry/1.0
right: 4-Fumarylacetoacetic acid
spontaneous: false
left: Maleylacetoacetic acid
name: Maleylacetoacetic acid → 4-Fumarylacetoacetic acid
conversion direction: LEFT_TO_RIGHT

ReactionCatalysis437
type: Catalysis
controller: L-amino-acid oxidase
controlled: 1.4.3.2
control type: ACTIVATION

3.7.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000088 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000981 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000042 Cytosol/Stoichiometry/1.0
right: Acetoacetic acid
right: Fumaric acid
spontaneous: false
EC number: 3.7.1.2
left: 4-Fumarylacetoacetic acid
left: Water
name: 4-Fumarylacetoacetic acid + Water → Acetoacetic acid + Fumaric acid
conversion direction: LEFT_TO_RIGHT

ReactionCatalysis435
type: Catalysis
controller: Aspartate aminotransferase, cytoplasmic
controlled: Reaction79
control type: ACTIVATION

Reference/TAXONOMY 9606
type: UnificationXref
id: 9606
db: TAXONOMY

Reference/PathWhiz PW000142
type: UnificationXref
id: PW000142
db: PathWhiz

Reference/CAS 70110-58-6
type: UnificationXref
id: 70110-58-6
db: CAS

Reference/UniProt P54577
type: UnificationXref
id: P54577
db: UniProt

Reference/BioCyc 2-KETOGLUTARATE
type: UnificationXref
id: 2-KETOGLUTARATE
db: BioCyc

Compound/PW C000042 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Acetoacetic acid
stoichiometric coefficient: 1.0

ReactionCatalysis208490
type: Catalysis
controller: Aspartate aminotransferase, cytoplasmic
controlled: Reaction80
control type: ACTIVATION

1.4.3.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000131 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001783 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000104 Lysosome/Stoichiometry/1.0
right: Phenylpyruvic acid
right: Hydrogen peroxide
right: Ammonia
spontaneous: false
EC number: 1.4.3.2
left: Water
left: Oxygen
left: L-Phenylalanine
name: L-Phenylalanine + Oxygen + Water → Ammonia + Hydrogen peroxide + Phenylpyruvic acid
conversion direction: LEFT_TO_RIGHT

1.4.3.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001065 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000558 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001783 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000103 Lysosome/Stoichiometry/1.0
right: 4-Hydroxyphenylpyruvic acid
right: Hydrogen peroxide
right: Ammonia
spontaneous: false
EC number: 1.4.3.2
left: Water
left: Oxygen
left: L-Tyrosine
name: L-Tyrosine + Oxygen + Water → 4-Hydroxyphenylpyruvic acid + Ammonia + Hydrogen peroxide
conversion direction: LEFT_TO_RIGHT

1.13.11.5
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000084 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001376 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Cytosol/Stoichiometry/1.0
right: Maleylacetoacetic acid
spontaneous: false
EC number: 1.13.11.5
left: Oxygen
left: Homogentisic acid
name: Homogentisic acid + Oxygen → Maleylacetoacetic acid
conversion direction: LEFT_TO_RIGHT

Compound/PW C000032 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Adenosine monophosphate
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 15377
type: UnificationXref
id: CHEBI:15377
db: ChEBI

Reference/ChEBI CHEBI 15379
type: UnificationXref
id: CHEBI:15379
db: ChEBI

Reference/PathWhiz PW000017
type: UnificationXref
id: PW000017
db: PathWhiz

ChemicalStructure/PW C000414
type: ChemicalStructure
structure format: SMILES

Compound/PW C000414 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Adenosine triphosphate
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 15374
type: UnificationXref
id: CHEBI:15374
db: ChEBI

Oxygen
type: SmallMolecule
cellular location: Cytosol
entity reference: Oxygen

Reference/PubChem-compound 280
type: UnificationXref
id: 280
db: PubChem-compound

1.13.11.27
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000084 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000558 Cytosol/Stoichiometry/1.0
right: Carbon dioxide
right: Homogentisic acid
spontaneous: false
EC number: 1.13.11.27
left: 4-Hydroxyphenylpyruvic acid
left: Oxygen
name: 4-Hydroxyphenylpyruvic acid + Oxygen → Carbon dioxide + Homogentisic acid
conversion direction: LEFT_TO_RIGHT

Compound/PW C001376 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Maleylacetoacetic acid
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 15344
type: UnificationXref
id: CHEBI:15344
db: ChEBI

Reference/PathWhiz PW000005
type: UnificationXref
id: PW000005
db: PathWhiz

Reference/ChEBI CHEBI 30851
type: UnificationXref
id: CHEBI:30851
db: ChEBI

L-amino-acid oxidase
type: Protein
cellular location: Lysosome
entity reference: L-amino-acid oxidase

1.14.16.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C040095 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000103 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000104 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001541 Cytosol/Stoichiometry/1.0
right: 4a-Hydroxytetrahydrobiopterin
right: L-Tyrosine
spontaneous: false
EC number: 1.14.16.1
left: Oxygen
left: L-Phenylalanine
left: L-erythro-tetrahydrobiopterin
name: L-Phenylalanine + L-erythro-tetrahydrobiopterin + Oxygen → 4a-Hydroxytetrahydrobiopterin + L-Tyrosine
conversion direction: LEFT_TO_RIGHT

Reference/CAS 541-50-4
type: UnificationXref
id: 541-50-4
db: CAS

Reference/ChEBI CHEBI 17895
type: UnificationXref
id: CHEBI:17895
db: ChEBI

Reference/CAS 5698-52-2
type: UnificationXref
id: 5698-52-2
db: CAS

Reference/HMDB HMDB0001967
type: UnificationXref
id: HMDB0001967
db: HMDB

Reference/KEGG Compound C00544
type: UnificationXref
id: C00544
db: KEGG Compound

Compound/PW C000558 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Hydroxyphenylpyruvic acid
stoichiometric coefficient: 1.0

RNA/21 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: tRNA(Tyr)
stoichiometric coefficient: 1.0

Reference/UniProt P17174
type: UnificationXref
id: P17174
db: UniProt

Compound/PW C000131 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Phenylpyruvic acid
stoichiometric coefficient: 1.0

Homogentisate 1,2-dioxygenase
type: Protein
cellular location: Cytosol
entity reference: Homogentisate 1,2-dioxygenase

Reference/BioCyc 4-MALEYL-ACETOACETATE
type: UnificationXref
id: 4-MALEYL-ACETOACETATE
db: BioCyc