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

Reference/UniProt O75746
type: UnificationXref
id: O75746
db: UniProt

Reference/UniProt Q9UI32
type: UnificationXref
id: Q9UI32
db: UniProt

TransportCatalysis5369
type: Catalysis
controller: Mitochondrial ornithine transporter 1
controlled: Transport14552
control type: ACTIVATION

Reference/PubChem-compound 6262
type: UnificationXref
id: 6262
db: PubChem-compound

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

Ornithine carbamoyltransferase, mitochondrial
type: Complex
component stoichiometry: Protein/870 Mitochondrial Matrix/Stoichiometry/3.0
cellular location: Mitochondrial Matrix
component: Ornithine carbamoyltransferase, mitochondrial

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

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

Unknown
type: ProteinReference
organism: BiologicalState55

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

Metabolsim and Physiological Effects of Argininic acid
type: Pathway
pathway order: PathwayTransport/2511380
pathway order: PathwayTransport/2511440
pathway order: PathwayEnzymeReaction/2511383
pathway order: PathwayEnzymeReaction/2511382
pathway order: PathwayEnzymeReaction/2511374
pathway order: PathwayEnzymeReaction/2511381
pathway order: PathwayEnzymeReaction/2511379
pathway order: PathwayEnzymeReaction/2511437
pathway order: PathwayEnzymeReaction/2511376
pathway order: PathwaySubPathway/118397
pathway order: PathwayEnzymeReaction/2511375
pathway order: PathwayTransport/2511806
pathway order: PathwayTransport/2511367
pathway order: PathwayTransport/2511443
pathway order: PathwayTransport/2511441
pathway order: PathwayEnzymeReaction/2511439
pathway order: PathwayTransport/2511369
pathway order: PathwayTransport/2511368
pathway component: TransportCatalysis5329
pathway component: ReactionCatalysis136
pathway component: TransportCatalysis5369
pathway component: 4.3.2.1
pathway component: ReactionCatalysis18
pathway component: Transport13859
pathway component: 3.5.3.1
pathway component: 6.3.4.5
pathway component: ReactionCatalysis12
pathway component: 2.3.1.85
pathway component: 1.4.3.3
pathway component: Neurotoxicity
pathway component: ReactionCatalysis203657
pathway component: TransportCatalysis5334
pathway component: TransportCatalysis5335
pathway component: Transport14516
pathway component: ReactionCatalysis208329
pathway component: Transport14519
pathway component: ReactionCatalysis208328
pathway component: Transport14518
pathway component: 3.5.1.2
pathway component: Reaction218866
pathway component: Transport14515
pathway component: 2.7.2.11
pathway component: ReactionCatalysis43
pathway component: Transport14552
pathway component: ReactionCatalysis208343
pathway component: ReactionCatalysis208342
pathway component: 2.1.3.3
pathway component: TransportCatalysis5333
pathway component: TransportCatalysis5330
pathway component: TransportCatalysis5331
xref: Reference/PathWhiz PW127060
xref: Reference/SMPDB SMP0125504
organism: Homo sapiens

Reference/KEGG Compound C00327
type: UnificationXref
id: C00327
db: KEGG Compound

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

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

Compound/PW C000140 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Ornithine
stoichiometric coefficient: 1.0

Transport14516
type: Transport
participant stoichiometry: Compound/PW C000723 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000723 Cytosol/Stoichiometry/1.0
right: Citrulline
spontaneous: false
left: Citrulline
name: Citrulline (→) Transport: Homo sapiens, Liver, Hepatocyte, Mitochondrial Matrix to Homo sapiens, Liver, Hepatocyte, Cytosol
conversion direction: LEFT_TO_RIGHT

Transport14519
type: Transport
participant stoichiometry: Compound/PW C001788/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001788 Cytosol/Stoichiometry/1.0
right: Argininic acid
spontaneous: false
left: Argininic acid
name: Argininic acid (→) Transport: Homo sapiens, Liver, Hepatocyte, Cytosol to Homo sapiens, Blood Vessel
conversion direction: LEFT_TO_RIGHT

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

Transport14518
type: Transport
participant stoichiometry: Compound/PW C000500/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000500 Mitochondrial Matrix/Stoichiometry/1.0
right: L-Glutamine
spontaneous: false
left: L-Glutamine
name: L-Glutamine (→) Transport: Homo sapiens, Blood Vessel to Homo sapiens, Liver, Hepatocyte, Mitochondrial Matrix
conversion direction: LEFT_TO_RIGHT

Transport14515
type: Transport
participant stoichiometry: Compound/PW C000500/Stoichiometry/1.0
right: L-Glutamine
spontaneous: false
left: L-Glutamine
name: L-Glutamine (→) Transport: Homo sapiens, Intestine to Homo sapiens, Blood Vessel
conversion direction: LEFT_TO_RIGHT

Canalicular multispecific organic anion transporter 2
type: ProteinReference
xref: Reference/UniProt O15438

Reference/UniProt O15438
type: UnificationXref
id: O15438
db: UniProt

Solute carrier family 22 member 5
type: ProteinReference
xref: Reference/UniProt O76082

Reference/UniProt O15439
type: UnificationXref
id: O15439
db: UniProt

Delta-1-pyrroline-5-carboxylate synthase
type: Protein
cellular location: Mitochondrial Inner Membrane
entity reference: Delta-1-pyrroline-5-carboxylate synthase

Reference/HMDB HMDB0004225
type: UnificationXref
id: HMDB0004225
db: HMDB

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

Ornithine aminotransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P04181
organism: BiologicalState55

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

Reference/ChemSpider 5746
type: UnificationXref
id: 5746
db: ChemSpider

Reference/ChemSpider 5745
type: UnificationXref
id: 5745
db: ChemSpider

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

Reference/ChemSpider 140984
type: UnificationXref
id: 140984
db: ChemSpider

Ornithine carbamoyltransferase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Ornithine carbamoyltransferase, mitochondrial

Compound/PW C000862 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Carbamoyl phosphate
stoichiometric coefficient: 1.0

Reference/BioCyc L-ASPARTATE
type: UnificationXref
id: L-ASPARTATE
db: BioCyc

Reference/ChemSpider 1143
type: UnificationXref
id: 1143
db: ChemSpider

Reference/ChEBI CHEBI 16474
type: UnificationXref
id: CHEBI:16474
db: ChEBI

Reference/HMDB HMDB0003148
type: UnificationXref
id: HMDB0003148
db: HMDB

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

Reference/UniProt P00812
type: UnificationXref
id: P00812
db: UniProt

Compound/PW C001788 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Argininic acid
stoichiometric coefficient: 1.0

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

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

Reference/UniProt P54886
type: UnificationXref
id: P54886
db: UniProt

Reference/BioCyc L-CITRULLINE
type: UnificationXref
id: L-CITRULLINE
db: BioCyc

L-Glutamic acid
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: L-Glutamic acid

Compound/PW C000143 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADP
stoichiometric coefficient: 1.0

Reference/PubChem-compound 16950
type: UnificationXref
id: 16950
db: PubChem-compound

Compound/PW C000146 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADPH
stoichiometric coefficient: 1.0

Reference/ChemSpider 1010
type: UnificationXref
id: 1010
db: ChemSpider

Reference/UniProt P04181
type: UnificationXref
id: P04181
db: UniProt

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

Reference/HMDB HMDB0001096
type: UnificationXref
id: HMDB0001096
db: HMDB

TransportCatalysis5329
type: Catalysis
controller: Solute carrier family 22 member 5
controlled: Transport14515
control type: ACTIVATION

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

Compound/PW C000146 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADPH
stoichiometric coefficient: 1.0

L-Glutamine
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000641
xref: Reference/ChEBI CHEBI 18050
xref: Reference/ChemSpider 5746
xref: Reference/BioCyc GLN
xref: Reference/KEGG Compound C00064
xref: Reference/PubChem-compound 5961
xref: Reference/CAS 56-85-9
chemical formula: C5H10N2O3
molecular weight: 146.06914
structure: ChemicalStructure/PW C000500

Carbamoyl phosphate
type: SmallMoleculeReference
xref: Reference/PubChem-compound 278
xref: Reference/BioCyc CARBAMOYL-P
xref: Reference/ChemSpider 272
xref: Reference/HMDB HMDB0001096
xref: Reference/CAS 590-55-6
xref: Reference/KEGG Compound C00169
xref: Reference/ChEBI CHEBI 17672
chemical formula: CH4NO5P
molecular weight: 140.98271
structure: ChemicalStructure/PW C000862

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

Neutral amino acid transporter A
type: Protein
entity reference: Neutral amino acid transporter A

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

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

Delta-1-pyrroline-5-carboxylate synthase
type: Complex
component stoichiometry: Protein/799 Mitochondrial Inner Membrane/Stoichiometry/4.0
cellular location: Mitochondrial Inner Membrane
component: Delta-1-pyrroline-5-carboxylate synthase

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

Reference/ChemSpider 1159
type: UnificationXref
id: 1159
db: ChemSpider

Reference/KEGG Compound C00009
type: UnificationXref
id: C00009
db: KEGG Compound

Reference/KEGG Compound C00006
type: UnificationXref
id: C00006
db: KEGG Compound

Compound/PW C000500/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Glutamine
stoichiometric coefficient: 1.0

ReactionCatalysis203657
type: Catalysis
controller: Arginase, mitochondrial
controlled: 3.5.3.1
control type: ACTIVATION

Reference/ChEBI CHEBI 18009
type: UnificationXref
id: CHEBI:18009
db: ChEBI

TransportCatalysis5334
type: Catalysis
controller: Calcium-binding mitochondrial carrier protein Aralar1
controlled: Transport14518
control type: ACTIVATION

TransportCatalysis5335
type: Catalysis
controller: Multidrug resistance-associated protein 4
controlled: Transport14519
control type: ACTIVATION

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 C00005
type: UnificationXref
id: C00005
db: KEGG Compound

Reference/KEGG Compound C00489
type: UnificationXref
id: C00489
db: KEGG Compound

Reference/ChEBI CHEBI 18367
type: UnificationXref
id: CHEBI:18367
db: ChEBI

Reference/ChemSpider 1032
type: UnificationXref
id: 1032
db: ChemSpider

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

Glutaric acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000661
xref: Reference/PubChem-compound 743
xref: Reference/CAS 110-94-1
xref: Reference/ChEBI CHEBI 17859
xref: Reference/KEGG Compound C00489
xref: Reference/ChemSpider 723
chemical formula: C5H8O4
molecular weight: 132.04225
structure: ChemicalStructure/PW C000518

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

Multidrug resistance-associated protein 4
type: Protein
entity reference: Multidrug resistance-associated protein 4

Mitochondrial Inner Membrane
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005743

TransportCatalysis5333
type: Catalysis
controller: Neutral amino acid transporter A
controlled: Transport14518
control type: ACTIVATION

TransportCatalysis5330
type: Catalysis
controller: Canalicular multispecific organic anion transporter 2
controlled: Transport14515
control type: ACTIVATION

TransportCatalysis5331
type: Catalysis
controller: Mitochondrial carnitine/acylcarnitine carrier protein CACL
controlled: Transport14516
control type: ACTIVATION

Citrulline
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00327
xref: Reference/CAS 372-75-8
xref: Reference/ChEBI CHEBI 16349
xref: Reference/PubChem-compound 6992098
xref: Reference/BioCyc L-CITRULLINE
xref: Reference/HMDB HMDB0000904
xref: Reference/ChemSpider 9367
chemical formula: C6H13N3O3
molecular weight: 175.09569
structure: ChemicalStructure/PW C000723

Reference/CAS 372-75-8
type: UnificationXref
id: 372-75-8
db: CAS

Reference/KEGG Compound C03406
type: UnificationXref
id: C03406
db: KEGG Compound

Reference/CAS 110-94-1
type: UnificationXref
id: 110-94-1
db: CAS

Reference/PubChem-compound 44367445
type: UnificationXref
id: 44367445
db: PubChem-compound

Transport13859
type: Transport
participant stoichiometry: Compound/PW C000500/Stoichiometry/1.0
right: L-Glutamine
spontaneous: false
left: L-Glutamine
name: L-Glutamine (→) Transport: Homo sapiens to Homo sapiens, Intestine
conversion direction: LEFT_TO_RIGHT

Reference/HMDB HMDB0000904
type: UnificationXref
id: HMDB0000904
db: HMDB

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

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

Reference/BioCyc NAD(P)
type: UnificationXref
id: NAD(P)
db: BioCyc

Compound/PW C000723 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Citrulline
stoichiometric coefficient: 1.0

1-Pyrroline-5-carboxylic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 1-Pyrroline-5-carboxylic acid

ChemicalStructure/PW C002094
type: ChemicalStructure
structure format: SMILES
structure data: NC(N)=NCCCC(=O)C(O)=O

Calcium-binding mitochondrial carrier protein Aralar1
type: Protein
entity reference: Calcium-binding mitochondrial carrier protein Aralar1

Hydrogen Ion
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00080
xref: Reference/ChEBI CHEBI 15378
xref: Reference/HMDB HMDB0059597
xref: Reference/PubChem-compound 1038
xref: Reference/ChemSpider 1010
chemical formula: H
molecular weight: 1.007825
structure: ChemicalStructure/PW C040034

Reference/PathWhiz PW127060
type: UnificationXref
id: PW127060
db: PathWhiz

Compound/PW C000396 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Arginine
stoichiometric coefficient: 1.0

BiologicalState55
type: BioSource
cell type: Hepatocyte
xref: Reference/TAXONOMY 9606
name: Homo sapiens, Liver, Hepatocyte
tissue: Liver

Phosphate
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Phosphate

L-Glutamine
type: SmallMolecule
entity reference: L-Glutamine

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

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

Reference/KEGG Compound C03771
type: UnificationXref
id: C03771
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

L-Glutamic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: L-Glutamic acid

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

Reference/ChemSpider 9367
type: UnificationXref
id: 9367
db: ChemSpider

Argininic acid
type: SmallMolecule
entity reference: Argininic acid
name: SubPathwayInput

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

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

Argininosuccinate lyase
type: ProteinReference
xref: Reference/UniProt P04424
organism: BiologicalState55

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

Compound/PW C002094 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Oxoarginine
stoichiometric coefficient: 1.0

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

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

Compound/PW C001104 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Phosphate
stoichiometric coefficient: 1.0

SubPathwayInteraction118397
type: Interaction
name: SubPathway118397Reaction
name: SubPathwayReaction
participant: Argininic acid

Reference/KEGG Compound C00049
type: UnificationXref
id: C00049
db: KEGG Compound

3.5.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000500 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Mitochondrial Matrix/Stoichiometry/1.0
right: Ammonia
right: L-Glutamic acid
spontaneous: false
EC number: 3.5.1.2
left: L-Glutamine
left: Water
name: L-Glutamine + Water → Ammonia + L-Glutamic acid
conversion direction: LEFT_TO_RIGHT

Reference/KEGG Compound C00169
type: UnificationXref
id: C00169
db: KEGG Compound

Reference/CAS 56-85-9
type: UnificationXref
id: 56-85-9
db: CAS

Delta-1-pyrroline-5-carboxylate synthase
type: ProteinReference
xref: Reference/UniProt P54886
organism: BiologicalState55

Ornithine aminotransferase, mitochondrial
type: Complex
component stoichiometry: Protein/392 Mitochondrial Matrix/Stoichiometry/4.0
cellular location: Mitochondrial Matrix
component: Ornithine aminotransferase, mitochondrial

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

Compound/PW C040034 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydrogen Ion
stoichiometric coefficient: 1.0

Glutaric acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Glutaric acid

Urea
type: SmallMoleculeReference
xref: Reference/BioCyc UREA
xref: Reference/CAS 57-13-6
xref: Reference/KEGG Compound C00086
xref: Reference/HMDB HMDB0000294
xref: Reference/ChemSpider 1143
xref: Reference/PubChem-compound 1176
xref: Reference/ChEBI CHEBI 16199
chemical formula: CH4N2O
molecular weight: 60.032364
structure: ChemicalStructure/PW C000200

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

4.3.2.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000088 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000396 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000036 Cytosol/Stoichiometry/2.0
right: Fumaric acid
right: L-Arginine
spontaneous: false
EC number: 4.3.2.1
left: Argininosuccinic acid
name: 2 Argininosuccinic acid → Fumaric acid + L-Arginine
conversion direction: LEFT_TO_RIGHT

Ammonia
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Ammonia

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

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

6.3.4.5
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000123 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000036 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000723 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000032 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000170 Cytosol/Stoichiometry/1.0
right: Argininosuccinic acid
right: Adenosine monophosphate
right: Pyrophosphate
spontaneous: false
EC number: 6.3.4.5
left: L-Aspartic acid
left: Adenosine triphosphate
left: Citrulline
name: Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate
conversion direction: LEFT_TO_RIGHT

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

Reference/ChEBI CHEBI 16199
type: UnificationXref
id: CHEBI:16199
db: ChEBI

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

ChemicalStructure/PW C000396
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CCCNC(N)=N)C(O)=O

ChemicalStructure/PW C000032
type: ChemicalStructure
structure format: SMILES

D-amino-acid oxidase
type: ProteinReference
xref: Reference/UniProt P14920
organism: BiologicalState55

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

ChemicalStructure/PW C000036
type: ChemicalStructure
structure format: SMILES

Reference/KEGG Compound C00062
type: UnificationXref
id: C00062
db: KEGG Compound

ChemicalStructure/PW C001003
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)C1CCC=N1

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/CAS 590-55-6
type: UnificationXref
id: 590-55-6
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 17053
type: UnificationXref
id: CHEBI:17053
db: ChEBI

Reference/KEGG Compound C00064
type: UnificationXref
id: C00064
db: KEGG Compound

Reference/CAS 2387-71-5
type: UnificationXref
id: 2387-71-5
db: CAS

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

Reference/ChemSpider 542
type: UnificationXref
id: 542
db: ChemSpider

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

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

Reference/KEGG Compound C00077
type: UnificationXref
id: C00077
db: KEGG Compound

Argininosuccinate synthase
type: ProteinReference
xref: Reference/UniProt P00966
organism: BiologicalState55

Reference/KEGG Compound C00080
type: UnificationXref
id: C00080
db: KEGG Compound

Water
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Water

Hydrogen Ion
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: Hydrogen Ion

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

Reference/HMDB HMDB0000052
type: UnificationXref
id: HMDB0000052
db: HMDB

Reference/HMDB HMDB0000294
type: UnificationXref
id: HMDB0000294
db: HMDB

Neurotoxicity
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway118397
pathway component: SubPathwayInteraction118397
name: SubPathway

Reference/CAS 14265-44-2
type: UnificationXref
id: 14265-44-2
db: CAS

Glutaminase liver isoform, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q9UI32
organism: BiologicalState55

Mitochondrial ornithine transporter 1
type: ProteinReference
xref: Reference/UniProt Q9Y619

Reference/KEGG Compound C00086
type: UnificationXref
id: C00086
db: KEGG Compound

Reference/KEGG Compound C04322
type: UnificationXref
id: C04322
db: KEGG Compound

Reference/ChemSpider 723
type: UnificationXref
id: 723
db: ChemSpider

Ornithine carbamoyltransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P00480
organism: BiologicalState55

L-Aspartic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 44367445
xref: Reference/ChemSpider 5745
xref: Reference/ChEBI CHEBI 17053
xref: Reference/HMDB HMDB0000191
xref: Reference/CAS 56-84-8
xref: Reference/KEGG Compound C00049
xref: Reference/BioCyc L-ASPARTATE
chemical formula: C4H7NO4
molecular weight: 133.0375
structure: ChemicalStructure/PW C000123

Reference/SMPDB SMP0125504
type: UnificationXref
id: SMP0125504
db: SMPDB

Compound/PW C001003 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 1-Pyrroline-5-carboxylic acid
stoichiometric coefficient: 1.0

Reference/PubChem-compound 743
type: UnificationXref
id: 743
db: PubChem-compound

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

2-Oxoarginine
type: SmallMolecule
cellular location: Cytosol
entity reference: 2-Oxoarginine

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

Compound/PW C001003 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: 1-Pyrroline-5-carboxylic acid
stoichiometric coefficient: 1.0

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

Reference/PubChem-compound 22833512
type: UnificationXref
id: 22833512
db: PubChem-compound

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

Reference/ChEBI CHEBI 18050
type: UnificationXref
id: CHEBI:18050
db: ChEBI

NADP
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: NADP

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

Reference/HMDB HMDB0000191
type: UnificationXref
id: HMDB0000191
db: HMDB

Protein/201 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: D-amino-acid oxidase
stoichiometric coefficient: 2.0

Compound/PW C000140 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Ornithine
stoichiometric coefficient: 1.0

Compound/PW C001788/Stoichiometry/1.0
type: Stoichiometry
physical entity: Argininic acid
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0059597
type: UnificationXref
id: HMDB0059597
db: HMDB

Argininosuccinate lyase
type: Protein
cellular location: Cytosol
entity reference: Argininosuccinate lyase

Reference/CAS 56-84-8
type: UnificationXref
id: 56-84-8
db: CAS

Argininic acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0003148
xref: Reference/CAS 157-07-3
xref: Reference/PubChem-compound 160437
xref: Reference/ChemSpider 140984
chemical formula: C6H13N3O3
molecular weight: 175.09569
structure: ChemicalStructure/PW C001788

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

Reference/UniProt P04424
type: UnificationXref
id: P04424
db: UniProt

Protein/870 Mitochondrial Matrix/Stoichiometry/3.0
type: Stoichiometry
physical entity: Ornithine carbamoyltransferase, mitochondrial
stoichiometric coefficient: 3.0

Protein/661 Cytosol/Stoichiometry/4.0
type: Stoichiometry
physical entity: Argininosuccinate lyase
stoichiometric coefficient: 4.0

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

Reference/ChemSpider 17215925
type: UnificationXref
id: 17215925
db: ChemSpider

ReactionCatalysis18
type: Catalysis
controller: Argininosuccinate lyase
controlled: 4.3.2.1
control type: ACTIVATION

ReactionCatalysis12
type: Catalysis
controller: Argininosuccinate synthase
controlled: 6.3.4.5
control type: ACTIVATION

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

1-Pyrroline-5-carboxylic acid
type: SmallMoleculeReference
xref: Reference/ChemSpider 1159
xref: Reference/HMDB HMDB0001301
xref: Reference/ChEBI CHEBI 1372
xref: Reference/CAS 2906-39-0
xref: Reference/BioCyc l-delta(1)-pyrroline 5-carboxylate
xref: Reference/KEGG Compound C04322
xref: Reference/PubChem-compound 1196
chemical formula: C5H7NO2
molecular weight: 113.047676
structure: ChemicalStructure/PW C001003

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

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

Argininosuccinic acid
type: SmallMoleculeReference
xref: Reference/KEGG Compound C03406
xref: Reference/ChemSpider 16059
xref: Reference/HMDB HMDB0000052
xref: Reference/PubChem-compound 16950
xref: Reference/CAS 2387-71-5
xref: Reference/ChEBI CHEBI 15682
chemical formula: C10H18N4O6
molecular weight: 290.12262
structure: ChemicalStructure/PW C000036

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

Reference/CAS 57-13-6
type: UnificationXref
id: 57-13-6
db: CAS

L-Arginine
type: SmallMoleculeReference
xref: Reference/BioCyc ARG
xref: Reference/ChEBI CHEBI 16467
xref: Reference/CAS 74-79-3
xref: Reference/KEGG Compound C00062
xref: Reference/ChemSpider 6082
xref: Reference/PubChem-compound 28782
xref: Reference/HMDB HMDB0000517
chemical formula: C6H14N4O2
molecular weight: 174.11168
structure: ChemicalStructure/PW C000396

Reference/UniProt Q8N8R3
type: UnificationXref
id: Q8N8R3
db: UniProt

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

D-amino-acid oxidase
type: Protein
cellular location: Cytosol
entity reference: D-amino-acid oxidase

Mitochondrial carnitine/acylcarnitine carrier protein CACL
type: ProteinReference
xref: Reference/UniProt Q8N8R3

Reference/UniProt P00480
type: UnificationXref
id: P00480
db: UniProt

Reference/PubChem-compound 6992098
type: UnificationXref
id: 6992098
db: PubChem-compound

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

ChemicalStructure/PW C001788
type: ChemicalStructure
structure format: SMILES
structure data: NC(=N)NCCC[C@H](O)C(O)=O

Compound/PW C000143 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADP
stoichiometric coefficient: 1.0

Reference/PubChem-compound 5961
type: UnificationXref
id: 5961
db: PubChem-compound

Reference/HMDB HMDB0000661
type: UnificationXref
id: HMDB0000661
db: HMDB

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

1-Pyrroline-5-carboxylic acid
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: 1-Pyrroline-5-carboxylic acid

Reference/ChEBI CHEBI 15729
type: UnificationXref
id: CHEBI:15729
db: ChEBI

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

Reference/CAS 53-59-8
type: UnificationXref
id: 53-59-8
db: CAS

Reference/PubChem-compound 1038
type: UnificationXref
id: 1038
db: PubChem-compound

Argininosuccinate synthase
type: Protein
cellular location: Cytosol
entity reference: Argininosuccinate synthase

Protein/392 Mitochondrial Matrix/Stoichiometry/4.0
type: Stoichiometry
physical entity: Ornithine aminotransferase, mitochondrial
stoichiometric coefficient: 4.0

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

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

Reference/ChemSpider 272
type: UnificationXref
id: 272
db: ChemSpider

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

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

Reference/CAS 70-26-8
type: UnificationXref
id: 70-26-8
db: CAS

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

Phosphate
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00009
xref: Reference/PubChem-compound 1061
xref: Reference/CAS 14265-44-2
xref: Reference/HMDB HMDB0001429
xref: Reference/BioCyc CPD-8587
xref: Reference/ChEBI CHEBI 18367
xref: Reference/ChemSpider 1032
chemical formula: H3O4P
molecular weight: 97.9769
structure: ChemicalStructure/PW C001104

Reference/ChemSpider 6026
type: UnificationXref
id: 6026
db: ChemSpider

ReactionCatalysis43
type: Catalysis
controller: Glutaminase liver isoform, mitochondrial
controlled: 3.5.1.2
control type: ACTIVATION

Reference/ChEBI CHEBI 58489
type: UnificationXref
id: CHEBI:58489
db: ChEBI

Protein/799 Mitochondrial Inner Membrane/Stoichiometry/4.0
type: Stoichiometry
physical entity: Delta-1-pyrroline-5-carboxylate synthase
stoichiometric coefficient: 4.0

Reference/UniProt Q9Y619
type: UnificationXref
id: Q9Y619
db: UniProt

ChemicalStructure/PW C000200
type: ChemicalStructure
structure format: SMILES
structure data: NC(N)=O

NADPH
type: SmallMoleculeReference
xref: Reference/CAS 53-57-6
xref: Reference/HMDB HMDB0000221
xref: Reference/PubChem-compound 22833512
xref: Reference/BioCyc NADPH
xref: Reference/ChemSpider 17215925
xref: Reference/KEGG Compound C00005
xref: Reference/ChEBI CHEBI 16474
chemical formula: C21H30N7O17P3
molecular weight: 745.0911
structure: ChemicalStructure/PW C000146

2.1.3.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000723 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000140 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001104 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000862 Mitochondrial Matrix/Stoichiometry/1.0
right: Citrulline
right: Phosphate
spontaneous: false
EC number: 2.1.3.3
left: Carbamoyl phosphate
left: Ornithine
name: Carbamoyl phosphate + Ornithine → Citrulline + Phosphate
conversion direction: LEFT_TO_RIGHT

NADP
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00006
xref: Reference/CAS 53-59-8
xref: Reference/BioCyc NAD(P)
xref: Reference/HMDB HMDB0000217
xref: Reference/ChEBI CHEBI 18009
xref: Reference/PubChem-compound 5886
xref: Reference/ChemSpider 5675
chemical formula: C21H28N7O17P3
molecular weight: 743.07544
structure: ChemicalStructure/PW C000143

Ornithine
type: SmallMoleculeReference
xref: Reference/CAS 70-26-8
xref: Reference/HMDB HMDB0000214
xref: Reference/PubChem-compound 6262
xref: Reference/KEGG Compound C00077
xref: Reference/BioCyc L-ORNITHINE
xref: Reference/ChemSpider 6026
xref: Reference/ChEBI CHEBI 15729
chemical formula: C5H12N2O2
molecular weight: 132.08987
structure: ChemicalStructure/PW C000140

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

NADPH
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: NADPH

Citrulline
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Citrulline

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

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

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

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

Reference/HMDB HMDB0001301
type: UnificationXref
id: HMDB0001301
db: HMDB

ChemicalStructure/PW C001104
type: ChemicalStructure
structure format: SMILES
structure data: OP(O)(O)=O

Reference/HMDB HMDB0000214
type: UnificationXref
id: HMDB0000214
db: HMDB

Reference/HMDB HMDB0000217
type: UnificationXref
id: HMDB0000217
db: HMDB

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/BioCyc l-delta(1)-pyrroline 5-carboxylate
type: UnificationXref
id: l-delta(1)-pyrroline_5-carboxylate
db: BioCyc

L-Glutamine
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: L-Glutamine

Reference/PubChem-compound 558
type: UnificationXref
id: 558
db: PubChem-compound

Reference/HMDB HMDB0001429
type: UnificationXref
id: HMDB0001429
db: HMDB

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

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

ChemicalStructure/PW C000143
type: ChemicalStructure
structure format: SMILES

Reference/HMDB HMDB0000221
type: UnificationXref
id: HMDB0000221
db: HMDB

ChemicalStructure/PW C000140
type: ChemicalStructure
structure format: SMILES
structure data: NCCC[C@H](N)C(O)=O

ChemicalStructure/PW C000146
type: ChemicalStructure
structure format: SMILES

Reference/GENE ONTOLOGY GO 0005759
type: UnificationXref
id: GO:0005759
db: GENE ONTOLOGY

Reference/BioCyc GLN
type: UnificationXref
id: GLN
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

Reference/CAS 53-57-6
type: UnificationXref
id: 53-57-6
db: CAS

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

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

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 6082
type: UnificationXref
id: 6082
db: ChemSpider

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

Reference/GENE ONTOLOGY GO 0005743
type: UnificationXref
id: GO:0005743
db: GENE ONTOLOGY

Reference/CAS 3715-10-4
type: UnificationXref
id: 3715-10-4
db: CAS

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

Ornithine
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Ornithine

Reference/UniProt O76082
type: UnificationXref
id: O76082
db: UniProt

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

ChemicalStructure/PW C000123
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CC(O)=O)C(O)=O

Reference/ChemSpider 16059
type: UnificationXref
id: 16059
db: ChemSpider

ChemicalStructure/PW C000723
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CCCNC(N)=O)C(O)=O

Reference/BioCyc CPD-8587
type: UnificationXref
id: CPD-8587
db: BioCyc

3.5.3.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000396 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000200 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000140 Cytosol/Stoichiometry/1.0
right: Urea
right: Ornithine
spontaneous: false
EC number: 3.5.3.1
left: L-Arginine
left: Water
name: L-Arginine + Water → Ornithine + Urea
conversion direction: LEFT_TO_RIGHT

2.3.1.85
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000146 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000143 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002094 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001788 Cytosol/Stoichiometry/1.0
right: Argininic acid
right: NADP
spontaneous: false
EC number: 2.3.1.85
left: 2-Oxoarginine
left: NADPH
name: 2-Oxoarginine + NADPH → Argininic acid + NADP
conversion direction: LEFT_TO_RIGHT

1.4.3.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000396 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000146 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000143 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002094 Cytosol/Stoichiometry/1.0
right: Ammonia
right: 2-Oxoarginine
right: NADPH
spontaneous: false
EC number: 1.4.3.3
left: L-Arginine
left: NADP
left: Water
name: L-Arginine + NADP + Water → 2-Oxoarginine + Ammonia + NADPH
conversion direction: LEFT_TO_RIGHT

Reference/ChEBI CHEBI 15682
type: UnificationXref
id: CHEBI:15682
db: ChEBI

Reference/UniProt P43007
type: UnificationXref
id: P43007
db: UniProt

Mitochondrial ornithine transporter 1
type: Protein
entity reference: Mitochondrial ornithine transporter 1

Reference/ChEBI CHEBI 17859
type: UnificationXref
id: CHEBI:17859
db: ChEBI

Mitochondrial Matrix
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005759

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

Reference/BioCyc CARBAMOYL-P
type: UnificationXref
id: CARBAMOYL-P
db: BioCyc

Argininosuccinate synthase
type: Complex
component stoichiometry: Protein/622 Cytosol/Stoichiometry/4.0
cellular location: Cytosol
component: Argininosuccinate synthase

Protein/622 Cytosol/Stoichiometry/4.0
type: Stoichiometry
physical entity: Argininosuccinate synthase
stoichiometric coefficient: 4.0

Reference/UniProt P14920
type: UnificationXref
id: P14920
db: UniProt

Reference/ChemSpider 5675
type: UnificationXref
id: 5675
db: ChemSpider

Compound/PW C000200 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Urea
stoichiometric coefficient: 1.0

Neutral amino acid transporter A
type: ProteinReference
xref: Reference/UniProt P43007

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

ReactionCatalysis208343
type: Catalysis
controller: Unknown
controlled: 2.3.1.85
control type: ACTIVATION

Reference/ChEBI CHEBI 1372
type: UnificationXref
id: CHEBI:1372
db: ChEBI

ReactionCatalysis208342
type: Catalysis
controller: D-amino-acid oxidase
controlled: 1.4.3.3
control type: ACTIVATION

ChemicalStructure/PW C000862
type: ChemicalStructure
structure format: SMILES
structure data: NC(=O)OP(O)(O)=O

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

Reference/BioCyc CPD-824
type: UnificationXref
id: CPD-824
db: BioCyc

Compound/PW C000723 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Citrulline
stoichiometric coefficient: 1.0

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

D-amino-acid oxidase
type: Complex
component stoichiometry: Protein/201 Cytosol/Stoichiometry/2.0
cellular location: Cytosol
component: D-amino-acid oxidase

Compound/PW C000036 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Argininosuccinic acid
stoichiometric coefficient: 1.0

Reference/PubChem-compound 160437
type: UnificationXref
id: 160437
db: PubChem-compound

Homo sapiens
type: BioSource
xref: Reference/TAXONOMY 9606

Multidrug resistance-associated protein 4
type: ProteinReference
xref: Reference/UniProt O15439

Calcium-binding mitochondrial carrier protein Aralar1
type: ProteinReference
xref: Reference/UniProt O75746

Compound/PW C000500 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Glutamine
stoichiometric coefficient: 1.0

Reference/PubChem-compound 28782
type: UnificationXref
id: 28782
db: PubChem-compound

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

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

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

ReactionCatalysis208329
type: Catalysis
controller: Ornithine aminotransferase, mitochondrial
controlled: Reaction218866
control type: ACTIVATION

ReactionCatalysis208328
type: Catalysis
controller: Delta-1-pyrroline-5-carboxylate synthase
controlled: 2.7.2.11
control type: ACTIVATION

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

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

Mitochondrial carnitine/acylcarnitine carrier protein CACL
type: Protein
entity reference: Mitochondrial carnitine/acylcarnitine carrier protein CACL

Arginase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P00812
organism: BiologicalState55

Compound/PW C000036 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: Argininosuccinic acid
stoichiometric coefficient: 2.0

Reference/CAS 157-07-3
type: UnificationXref
id: 157-07-3
db: CAS

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

Oxoglutaric acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Oxoglutaric acid

Reference/PubChem-compound 1176
type: UnificationXref
id: 1176
db: PubChem-compound

Canalicular multispecific organic anion transporter 2
type: Protein
entity reference: Canalicular multispecific organic anion transporter 2

2-Oxoarginine
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0004225
xref: Reference/ChemSpider 542
xref: Reference/PubChem-compound 558
xref: Reference/CAS 3715-10-4
xref: Reference/KEGG Compound C03771
xref: Reference/BioCyc CPD-824
xref: Reference/ChEBI CHEBI 58489
chemical formula: C6H11N3O3
molecular weight: 173.08005
structure: ChemicalStructure/PW C002094

Solute carrier family 22 member 5
type: Protein
entity reference: Solute carrier family 22 member 5

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

Glutaminase liver isoform, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Glutaminase liver isoform, mitochondrial

Reference/PubChem-compound 278
type: UnificationXref
id: 278
db: PubChem-compound

Reference/ChEBI CHEBI 15378
type: UnificationXref
id: CHEBI:15378
db: ChEBI

ChemicalStructure/PW C040034
type: ChemicalStructure
structure format: SMILES
structure data: [H+]

Reference/ChEBI CHEBI 16467
type: UnificationXref
id: CHEBI:16467
db: ChEBI

Reference/ChEBI CHEBI 16349
type: UnificationXref
id: CHEBI:16349
db: ChEBI

ChemicalStructure/PW C000414
type: ChemicalStructure
structure format: SMILES

Reference/PubChem-compound 5886
type: UnificationXref
id: 5886
db: PubChem-compound

Reference/ChEBI CHEBI 17672
type: UnificationXref
id: CHEBI:17672
db: ChEBI

Transport14552
type: Transport
participant stoichiometry: Compound/PW C000140 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000140 Cytosol/Stoichiometry/1.0
right: Ornithine
spontaneous: false
left: Ornithine
name: Ornithine (→) Transport: Homo sapiens, Liver, Hepatocyte, Cytosol to Homo sapiens, Liver, Hepatocyte, Mitochondrial Matrix
conversion direction: LEFT_TO_RIGHT

Ornithine aminotransferase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Ornithine aminotransferase, mitochondrial

Argininosuccinate lyase
type: Complex
component stoichiometry: Protein/661 Cytosol/Stoichiometry/4.0
cellular location: Cytosol
component: Argininosuccinate lyase

Carbamoyl phosphate
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Carbamoyl phosphate

Reference/UniProt P00966
type: UnificationXref
id: P00966
db: UniProt

ReactionCatalysis136
type: Catalysis
controller: Ornithine carbamoyltransferase, mitochondrial
controlled: 2.1.3.3
control type: ACTIVATION

ChemicalStructure/PW C000500
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CCC(N)=O)C(O)=O

Reference/PubChem-compound 1196
type: UnificationXref
id: 1196
db: PubChem-compound

Arginase, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: Arginase, mitochondrial

Compound/PW C000518 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glutaric acid
stoichiometric coefficient: 1.0

Reference/PubChem-compound 1061
type: UnificationXref
id: 1061
db: PubChem-compound

Reference/CAS 74-79-3
type: UnificationXref
id: 74-79-3
db: CAS

Reference/HMDB HMDB0000517
type: UnificationXref
id: HMDB0000517
db: HMDB

ChemicalStructure/PW C000518
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CCCC(O)=O

Reaction218866
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000140 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000518 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001003 Mitochondrial Matrix/Stoichiometry/1.0
right: Oxoglutaric acid
right: Ornithine
spontaneous: false
left: 1-Pyrroline-5-carboxylic acid
left: Glutaric acid
name: 1-Pyrroline-5-carboxylic acid + Glutaric acid → Ornithine + Oxoglutaric acid
conversion direction: LEFT_TO_RIGHT

2.7.2.11
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000095 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001003 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000143 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000146 Mitochondrial Inner Membrane/Stoichiometry/1.0
right: NADP
right: 1-Pyrroline-5-carboxylic acid
spontaneous: false
EC number: 2.7.2.11
left: NADPH
left: Hydrogen Ion
left: L-Glutamic acid
name: Hydrogen Ion + L-Glutamic acid + NADPH → 1-Pyrroline-5-carboxylic acid + NADP
conversion direction: LEFT_TO_RIGHT

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

Reference/HMDB HMDB0000641
type: UnificationXref
id: HMDB0000641
db: HMDB

Compound/PW C000123 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Aspartic acid
stoichiometric coefficient: 1.0

Reference/CAS 2906-39-0
type: UnificationXref
id: 2906-39-0
db: CAS

Reference/BioCyc L-ORNITHINE
type: UnificationXref
id: L-ORNITHINE
db: BioCyc