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

Reference/PubChem-compound 6022
type: UnificationXref
id: 6022
db: PubChem-compound

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

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

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

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

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

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

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

Compound/PW C001034 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Adenosine diphosphate
stoichiometric coefficient: 2.0

L-Aspartic acid
type: SmallMolecule
cellular location: Cytoplasm
entity reference: L-Aspartic acid
name: SubPathwayOutput

Reference/PubChem-compound 439153
type: UnificationXref
id: 439153
db: PubChem-compound

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

Arginase-1
type: Complex
component stoichiometry: Protein/292 Cytoplasm/Stoichiometry/3.0
cellular location: Cytoplasm
component: Arginase-1

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

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

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

Reference/BioCyc OXALACETIC ACID
type: UnificationXref
id: OXALACETIC_ACID
db: BioCyc

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

Reference/PathWhiz PW000052
type: UnificationXref
id: PW000052
db: PathWhiz

Aspartate aminotransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P00505
organism: Homo sapiens

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

Reference/PathWhiz PW000046
type: UnificationXref
id: PW000046
db: PathWhiz

Reference/PathWhiz PW000043
type: UnificationXref
id: PW000043
db: PathWhiz

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

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

Reference/PathWhiz PW000162
type: UnificationXref
id: PW000162
db: PathWhiz

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/PathWhiz PW000160
type: UnificationXref
id: PW000160
db: PathWhiz

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

Transport139
type: Transport
participant stoichiometry: Compound/PW C000500 Extracellular Space/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000500 Cytoplasm/Stoichiometry/1.0
right: L-Glutamine
spontaneous: false
left: L-Glutamine
name: L-Glutamine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm
conversion direction: LEFT_TO_RIGHT

Compound/PW C001441 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Phosphoric acid
stoichiometric coefficient: 1.0

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

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

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

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

Carbon dioxide
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Carbon dioxide

Transport141
type: Transport
participant stoichiometry: Compound/PW C000105 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000105 Extracellular Space/Stoichiometry/1.0
right: L-Alanine
spontaneous: false
left: L-Alanine
name: L-Alanine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm
conversion direction: LEFT_TO_RIGHT

Reference/ChemSpider 5735
type: UnificationXref
id: 5735
db: ChemSpider

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

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

Reference/PathWhiz PW000158
type: UnificationXref
id: PW000158
db: PathWhiz

Reference/ChemSpider 945
type: UnificationXref
id: 945
db: ChemSpider

Reference/PathWhiz PW000152
type: UnificationXref
id: PW000152
db: PathWhiz

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

Reference/PathWhiz PW000030
type: UnificationXref
id: PW000030
db: PathWhiz

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

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

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

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

Arginase-1
type: ProteinReference
xref: Reference/UniProt P05089
organism: Homo sapiens

Compound/PW C000088 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Fumaric acid
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

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

L-Alanine
type: SmallMolecule
cellular location: Extracellular Space
entity reference: L-Alanine
name: SubPathwayInput

Alanine aminotransferase 1
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Alanine aminotransferase 1

Reference/CAS 7664-38-2
type: UnificationXref
id: 7664-38-2
db: CAS

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

Protein/658 Mitochondrial Matrix/Stoichiometry/6.0
type: Stoichiometry
physical entity: Glutamate dehydrogenase 1, mitochondrial
stoichiometric coefficient: 6.0

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

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

Reference/KEGG Compound C00008
type: UnificationXref
id: C00008
db: KEGG Compound

Reference/BioCyc L-ALPHA-ALANINE
type: UnificationXref
id: L-ALPHA-ALANINE
db: BioCyc

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

Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADH
stoichiometric coefficient: 1.0

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

Reference/CAS 56-41-7
type: UnificationXref
id: 56-41-7
db: CAS

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

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

Reference/KEGG Compound C00004
type: UnificationXref
id: C00004
db: KEGG Compound

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

Reference/KEGG Compound C00003
type: UnificationXref
id: C00003
db: KEGG Compound

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

Reference/ChemSpider 1031
type: UnificationXref
id: 1031
db: ChemSpider

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

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

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

Carbamoyl-phosphate synthase [ammonia], mitochondrial
type: ProteinReference
xref: Reference/UniProt P31327
organism: Homo sapiens

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

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

NAD
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15846
xref: Reference/HMDB HMDB0000902
xref: Reference/CAS 53-84-9
xref: Reference/KEGG Compound C00003
xref: Reference/PubChem-compound 5893
xref: Reference/BioCyc NAD
xref: Reference/ChemSpider 5682
chemical formula: C21H27N7O14P2
molecular weight: 663.10913
structure: ChemicalStructure/PW C000721

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

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

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

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

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

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

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

Reference/HMDB HMDB0000902
type: UnificationXref
id: HMDB0000902
db: HMDB

Compound/PW C000414 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Adenosine triphosphate
stoichiometric coefficient: 2.0

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

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

Extracellular Space
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005615

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

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

Neutral amino acid transporter B(0)
type: ProteinReference
xref: Reference/UniProt Q15758

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

Reference/KEGG Compound C00022
type: UnificationXref
id: C00022
db: KEGG Compound

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

Reference/CAS 53-84-9
type: UnificationXref
id: 53-84-9
db: CAS

Reference/KEGG Compound C00020
type: UnificationXref
id: C00020
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

Reference/ChEBI CHEBI 26078
type: UnificationXref
id: CHEBI:26078
db: ChEBI

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

SubPathwayInteraction675
type: Interaction
name: SubPathway675Reaction
name: SubPathwayReaction
participant: L-Glutamine

SubPathwayInteraction676
type: Interaction
name: SubPathway676Reaction
name: SubPathwayReaction
participant: L-Glutamine

SubPathwayInteraction674
type: Interaction
name: SubPathwayReaction
name: SubPathway674Reaction
participant: L-Glutamine

SubPathwayInteraction679
type: Interaction
name: SubPathway679Reaction
name: SubPathwayReaction
participant: L-Alanine

SubPathwayInteraction677
type: Interaction
name: SubPathwayReaction
name: SubPathway677Reaction
participant: L-Alanine

1.4.1.3
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 C000721 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Mitochondrial Matrix/Stoichiometry/1.0
right: Ammonia
right: Oxoglutaric acid
right: NADH
spontaneous: false
EC number: 1.4.1.3
left: Water
left: L-Glutamic acid
left: NAD
name: L-Glutamic acid + NAD + Water → Ammonia + NADH + Oxoglutaric acid
conversion direction: LEFT_TO_RIGHT

SubPathwayInteraction678
type: Interaction
name: SubPathwayReaction
name: SubPathway678Reaction
participant: L-Alanine

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

Reference/KEGG Compound C00036
type: UnificationXref
id: C00036
db: KEGG Compound

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

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

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

Argininosuccinate lyase
type: ProteinReference
xref: Reference/UniProt P04424
organism: Homo sapiens

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

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

Reference/KEGG Compound C00041
type: UnificationXref
id: C00041
db: KEGG Compound

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

Reference/HMDB HMDB0001341
type: UnificationXref
id: HMDB0001341
db: HMDB

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

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

L-Glutamine
type: SmallMolecule
cellular location: Cytoplasm
entity reference: L-Glutamine

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

3.5.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000500 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Mitochondrion/Stoichiometry/1.0
right: L-Glutamic acid
right: Ammonia
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

ChemicalStructure/PW C001034
type: ChemicalStructure
structure format: SMILES

Alanine aminotransferase 1
type: ProteinReference
xref: Reference/UniProt P24298
organism: Homo sapiens

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

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

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

Compound/PW C000105 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Alanine
stoichiometric coefficient: 1.0

SubPathwayInteraction698
type: Interaction
name: SubPathway698Reaction
name: SubPathwayReaction
participant: Oxoglutaric acid

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

4.3.2.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000036 Cytoplasm/Stoichiometry/2.0
participant stoichiometry: Compound/PW C000396 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000088 Cytoplasm/Stoichiometry/1.0
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

SubPathwayInteraction699
type: Interaction
name: SubPathway699Reaction
name: SubPathwayReaction
participant: Oxoglutaric acid

Ammonia
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Ammonia
name: SubPathwayOutput
name: SubPathwayInput

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

ChemicalStructure/PW C000148
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CC(=O)C(O)=O

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

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

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

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

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

Reference/HMDB HMDB0001487
type: UnificationXref
id: HMDB0001487
db: HMDB

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

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

Compound/PW C000105 Extracellular Space/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Alanine
stoichiometric coefficient: 1.0

ChemicalStructure/PW C000032
type: ChemicalStructure
structure format: SMILES

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

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

ChemicalStructure/PW C000036
type: ChemicalStructure
structure format: SMILES

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

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

Glutamate dehydrogenase 1, mitochondrial
type: Complex
component stoichiometry: Protein/658 Mitochondrial Matrix/Stoichiometry/6.0
cellular location: Mitochondrial Matrix
component: Glutamate dehydrogenase 1, mitochondrial

L-Aspartic acid
type: SmallMolecule
cellular location: Mitochondrial Intermembrane Space
entity reference: L-Aspartic acid

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/UniProt P24298
type: UnificationXref
id: P24298
db: UniProt

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

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

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

2.6.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000095 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000164 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000105 Mitochondrial Matrix/Stoichiometry/1.0
right: Pyruvic acid
right: L-Glutamic acid
spontaneous: false
EC number: 2.6.1.2
left: L-Alanine
left: Oxoglutaric acid
name: L-Alanine + Oxoglutaric acid ↔ L-Glutamic acid + Pyruvic acid
conversion direction: REVERSIBLE

ChemicalStructure/PW C000164
type: ChemicalStructure
structure format: SMILES
structure data: CC(=O)C(O)=O

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

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

Reference/HMDB HMDB0000161
type: UnificationXref
id: HMDB0000161
db: HMDB

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

L-Alanine
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00041
xref: Reference/ChemSpider 5735
xref: Reference/ChEBI CHEBI 16977
xref: Reference/BioCyc L-ALPHA-ALANINE
xref: Reference/HMDB HMDB0000161
xref: Reference/CAS 56-41-7
xref: Reference/PubChem-compound 5950
chemical formula: C3H7NO2
molecular weight: 89.047676
structure: ChemicalStructure/PW C000105

Reference/ChemSpider 5800
type: UnificationXref
id: 5800
db: ChemSpider

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

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

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/ChemSpider 979
type: UnificationXref
id: 979
db: ChemSpider

Argininosuccinate synthase
type: ProteinReference
xref: Reference/UniProt P00966
organism: Homo sapiens

Carbamoyl-phosphate synthase [ammonia], mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Carbamoyl-phosphate synthase [ammonia], mitochondrial

Compound/PW C000105 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Alanine
stoichiometric coefficient: 1.0

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

Phosphoric acid
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00009
xref: Reference/ChEBI CHEBI 26078
xref: Reference/CAS 7664-38-2
xref: Reference/PubChem-compound 1004
xref: Reference/ChemSpider 979
xref: Reference/HMDB HMDB0002142
chemical formula: H3O4P
molecular weight: 97.9769
structure: ChemicalStructure/PW C001441

Glutamate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway699
pathway order: SubPathwayInteractionSteps/SubPathway676
pathway order: SubPathwayInteractionSteps/SubPathway708
pathway component: SubPathwayInteraction676
pathway component: SubPathwayInteraction699
pathway component: SubPathwayInteraction708
xref: Reference/SMPDB SMP0000072
xref: Reference/PathWhiz PW000003
name: SubPathway

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 HMDB0000294
type: UnificationXref
id: HMDB0000294
db: HMDB

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

Adenosine diphosphate
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Adenosine diphosphate

L-Glutamine
type: SmallMolecule
cellular location: Mitochondrion
entity reference: L-Glutamine

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

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

Reference/CAS 58-64-0
type: UnificationXref
id: 58-64-0
db: CAS

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

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

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

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

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

L-Glutamine
type: SmallMolecule
cellular location: Extracellular Space
entity reference: L-Glutamine
name: SubPathwayOutput
name: SubPathwayInput

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

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

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

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

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

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

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

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

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

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

Compound/PW C000723 Cytoplasm/Stoichiometry/0.0
type: Stoichiometry
physical entity: Citrulline
stoichiometric coefficient: 0.0

Glutamate dehydrogenase 1, mitochondrial
type: ProteinReference
xref: Reference/UniProt P00367
organism: Homo sapiens

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

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

Glutamate dehydrogenase 1, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Glutamate dehydrogenase 1, mitochondrial

Reference/PubChem-compound 970
type: UnificationXref
id: 970
db: PubChem-compound

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

Reference/HMDB HMDB0002142
type: UnificationXref
id: HMDB0002142
db: HMDB

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

SubPathwayInteraction712
type: Interaction
name: SubPathway712Reaction
name: SubPathwayReaction
participant: Ammonia

SubPathwayInteraction713
type: Interaction
name: SubPathwayReaction
name: SubPathway713Reaction
participant: Ammonia

Oxalacetic acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000223
xref: Reference/CAS 328-42-7
xref: Reference/ChEBI CHEBI 30744
xref: Reference/BioCyc OXALACETIC ACID
xref: Reference/ChemSpider 945
xref: Reference/PubChem-compound 970
xref: Reference/KEGG Compound C00036
chemical formula: C4H4O5
molecular weight: 132.00587
structure: ChemicalStructure/PW C000148

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

SubPathwayInteraction710
type: Interaction
name: SubPathway710Reaction
name: SubPathwayReaction
participant: Oxalacetic acid

SubPathwayInteraction711
type: Interaction
name: SubPathwayReaction
name: SubPathway711Reaction
participant: Ammonia

SubPathwayInteraction716
type: Interaction
name: SubPathwayReaction
name: SubPathway716Reaction
participant: L-Arginine

SubPathwayInteraction717
type: Interaction
name: SubPathwayReaction
name: SubPathway717Reaction
participant: Fumaric acid

Adenosine triphosphate
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Adenosine triphosphate

SubPathwayInteraction714
type: Interaction
name: SubPathway714Reaction
name: SubPathwayReaction
participant: Ornithine

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

SubPathwayInteraction715
type: Interaction
name: SubPathway715Reaction
name: SubPathwayReaction
participant: L-Arginine

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

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

SubPathwayInteraction718
type: Interaction
name: SubPathway718Reaction
name: SubPathwayReaction
participant: L-Aspartic acid

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

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-Arginine and D-Ornithine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway716
pathway order: SubPathwayInteractionSteps/SubPathway714
pathway component: SubPathwayInteraction716
pathway component: SubPathwayInteraction714
xref: Reference/SMPDB SMP0000036
xref: Reference/PathWhiz PW000019
name: SubPathway

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

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

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

SubPathwayInteraction701
type: Interaction
name: SubPathway701Reaction
name: SubPathwayReaction
participant: Oxoglutaric acid

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

SubPathwayInteraction702
type: Interaction
name: SubPathwayReaction
name: SubPathway702Reaction
participant: Oxoglutaric acid

SubPathwayInteraction700
type: Interaction
name: SubPathway700Reaction
name: SubPathwayReaction
participant: Oxoglutaric acid

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

SubPathwayInteraction705
type: Interaction
name: SubPathwayReaction
name: SubPathway705Reaction
participant: L-Glutamic acid

SubPathwayInteraction706
type: Interaction
name: SubPathway706Reaction
name: SubPathwayReaction
participant: L-Glutamic acid

Reference/PubChem-compound 1004
type: UnificationXref
id: 1004
db: PubChem-compound

SubPathwayInteraction703
type: Interaction
name: SubPathway703Reaction
name: SubPathwayReaction
participant: L-Glutamic acid

SubPathwayInteraction704
type: Interaction
name: SubPathwayReaction
name: SubPathway704Reaction
participant: L-Glutamic acid

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

SubPathwayInteraction709
type: Interaction
name: SubPathwayReaction
name: SubPathway709Reaction
participant: L-Glutamic acid

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

TransportCatalysis7
type: Catalysis
controller: Calcium-binding mitochondrial carrier protein Aralar1
controlled: Transport31
control type: ACTIVATION

SubPathwayInteraction708
type: Interaction
name: SubPathwayReaction
name: SubPathway708Reaction
participant: L-Glutamic acid

Histidine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway703
pathway component: SubPathwayInteraction703
xref: Reference/PathWhiz PW000043
xref: Reference/SMPDB SMP0000044
name: SubPathway

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

Compound/PW C000148 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Oxalacetic acid
stoichiometric coefficient: 1.0

Reference/CAS 328-42-7
type: UnificationXref
id: 328-42-7
db: CAS

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

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

Reference/ChEBI CHEBI 15846
type: UnificationXref
id: CHEBI:15846
db: ChEBI

Reference/UniProt P00367
type: UnificationXref
id: P00367
db: UniProt

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

Pyruvic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 1060
xref: Reference/HMDB HMDB0000243
xref: Reference/CAS 127-17-3
xref: Reference/BioCyc PYRUVATE
xref: Reference/KEGG Compound C00022
xref: Reference/ChEBI CHEBI 32816
xref: Reference/ChemSpider 1031
chemical formula: C3H4O3
molecular weight: 88.016045
structure: ChemicalStructure/PW C000164

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

ReactionCatalysis137
type: Catalysis
controller: Arginase-1
controlled: 3.5.3.1
control type: ACTIVATION

Pyrimidine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway675
pathway component: SubPathwayInteraction675
xref: Reference/SMPDB SMP0000046
xref: Reference/PathWhiz PW000160
name: SubPathway

ReactionCatalysis38
type: Catalysis
controller: Glutamate dehydrogenase 1, mitochondrial
controlled: 1.4.1.3
control type: ACTIVATION

Neutral amino acid transporter B(0)
type: Protein
entity reference: Neutral amino acid transporter B(0)

Alanine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway700
pathway order: SubPathwayInteractionSteps/SubPathway677
pathway order: SubPathwayInteractionSteps/SubPathway705
pathway component: SubPathwayInteraction677
pathway component: SubPathwayInteraction700
pathway component: SubPathwayInteraction705
xref: Reference/PathWhiz PW000001
xref: Reference/SMPDB SMP0000055
name: SubPathway

Purine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway712
pathway component: SubPathwayInteraction712
xref: Reference/SMPDB SMP0000050
xref: Reference/PathWhiz PW000052
name: SubPathway

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

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

Ornithine
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Ornithine
name: SubPathwayInput

Urea Cycle
type: Pathway
pathway order: PathwaySubPathway/674
pathway order: PathwaySubPathway/675
pathway order: PathwayEnzymeReaction/2781
pathway order: PathwaySubPathway/715
pathway order: PathwaySubPathway/716
pathway order: PathwaySubPathway/713
pathway order: PathwaySubPathway/714
pathway order: PathwaySubPathway/678
pathway order: PathwaySubPathway/711
pathway order: PathwaySubPathway/679
pathway order: PathwaySubPathway/712
pathway order: PathwaySubPathway/676
pathway order: PathwaySubPathway/698
pathway order: PathwaySubPathway/677
pathway order: PathwaySubPathway/699
pathway order: PathwaySubPathway/710
pathway order: PathwayEnzymeReaction/2778
pathway order: PathwayEnzymeReaction/2834
pathway order: PathwayTransport/2843
pathway order: PathwayEnzymeReaction/2851
pathway order: PathwaySubPathway/708
pathway order: PathwayEnzymeReaction/2830
pathway order: PathwaySubPathway/709
pathway order: PathwayTransport/1879
pathway order: PathwayEnzymeReaction/2776
pathway order: PathwayEnzymeReaction/2853
pathway order: PathwaySubPathway/706
pathway order: PathwaySubPathway/704
pathway order: PathwaySubPathway/705
pathway order: PathwaySubPathway/702
pathway order: PathwaySubPathway/703
pathway order: PathwaySubPathway/700
pathway order: PathwaySubPathway/701
pathway order: PathwayTransport/2891
pathway order: PathwayTransport/1881
pathway order: PathwayEnzymeReaction/2747
pathway order: PathwayEnzymeReaction/2847
pathway order: PathwaySubPathway/717
pathway order: PathwaySubPathway/718
pathway component: ReactionCatalysis38
pathway component: ReactionCatalysis136
pathway component: ReactionCatalysis133
pathway component: Reaction78
pathway component: 4.3.2.1
pathway component: ReactionCatalysis18
pathway component: 1.4.1.3
pathway component: ReactionCatalysis372
pathway component: TransportCatalysis12
pathway component: 6.3.4.5
pathway component: ReactionCatalysis12
pathway component: Alanine Metabolism
pathway component: Glutamate Metabolism
pathway component: Purine Metabolism
pathway component: Transport139
pathway component: D-Arginine and D-Ornithine Metabolism
pathway component: Citric Acid Cycle
pathway component: Transport39
pathway component: 6.3.4.16
pathway component: Transport141
pathway component: ReactionCatalysis2
pathway component: Transport31
pathway component: 3.5.1.2
pathway component: ReactionCatalysis43
pathway component: TransportCatalysis7
pathway component: 2.6.1.2
pathway component: Aspartate Metabolism
pathway component: Histidine Metabolism
pathway component: Arginine and Proline Metabolism
pathway component: 2.1.3.3
pathway component: Ammonia Recycling
pathway component: Malate-Aspartate Shuttle
pathway component: Selenoamino Acid Metabolism
pathway component: Gluconeogenesis
pathway component: Glucose-Alanine Cycle
pathway component: TransportCatalysis103
pathway component: TransportCatalysis101
pathway component: 3.5.3.1
pathway component: ReactionCatalysis137
pathway component: Pyrimidine Metabolism

Reference/ChEBI CHEBI 16908
type: UnificationXref
id: CHEBI:16908
db: ChEBI

Citric Acid Cycle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway717
pathway component: SubPathwayInteraction717
xref: Reference/PathWhiz PW000005
xref: Reference/SMPDB SMP0000057
name: SubPathway

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

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

Aspartate aminotransferase, mitochondrial
type: Complex
component stoichiometry: Protein/278 Mitochondrial Matrix/Stoichiometry/2.0
cellular location: Mitochondrial Matrix
component: Aspartate aminotransferase, mitochondrial

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

Aspartate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway718
pathway component: SubPathwayInteraction718
xref: Reference/PathWhiz PW000002
xref: Reference/SMPDB SMP0000067
name: SubPathway

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

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

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

Mitochondrion
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005739

TransportCatalysis103
type: Catalysis
controller: Neutral amino acid transporter A
controlled: Transport141
control type: ACTIVATION

TransportCatalysis101
type: Catalysis
controller: Neutral amino acid transporter B(0)
controlled: Transport139
control type: ACTIVATION

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

Reaction78
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000148 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000123 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Mitochondrial Matrix/Stoichiometry/1.0
right: Oxalacetic acid
right: L-Glutamic acid
spontaneous: false
left: L-Aspartic acid
left: Oxoglutaric acid
name: L-Aspartic acid + Oxoglutaric acid ↔ L-Glutamic acid + Oxalacetic acid
conversion direction: REVERSIBLE

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

Reference/ChEBI CHEBI 16977
type: UnificationXref
id: CHEBI:16977
db: ChEBI

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

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

Reference/CAS 58-68-4
type: UnificationXref
id: 58-68-4
db: CAS

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

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

Reference/HMDB HMDB0000214
type: UnificationXref
id: HMDB0000214
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/CAS 124-38-9
type: UnificationXref
id: 124-38-9
db: CAS

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

Reference/SMPDB SMP0000046
type: UnificationXref
id: SMP0000046
db: SMPDB

Reference/SMPDB SMP0000044
type: UnificationXref
id: SMP0000044
db: SMPDB

ReactionCatalysis2
type: Catalysis
controller: Alanine aminotransferase 1
controlled: 2.6.1.2
control type: ACTIVATION

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

Arginase-1
type: Protein
cellular location: Cytoplasm
entity reference: Arginase-1

Reference/SMPDB SMP0000050
type: UnificationXref
id: SMP0000050
db: SMPDB

Reference/HMDB HMDB0000223
type: UnificationXref
id: HMDB0000223
db: HMDB

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

Reference/GENE ONTOLOGY GO 0005758
type: UnificationXref
id: GO:0005758
db: GENE ONTOLOGY

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

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

Ammonia Recycling
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway711
pathway order: SubPathwayInteractionSteps/SubPathway674
pathway order: SubPathwayInteractionSteps/SubPathway706
pathway component: SubPathwayInteraction674
pathway component: SubPathwayInteraction711
pathway component: SubPathwayInteraction706
xref: Reference/SMPDB SMP0000009
xref: Reference/PathWhiz PW000009
name: SubPathway

Reference/SMPDB SMP0000059
type: UnificationXref
id: SMP0000059
db: SMPDB

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

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

Malate-Aspartate Shuttle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway698
pathway component: SubPathwayInteraction698
xref: Reference/SMPDB SMP0000129
xref: Reference/PathWhiz PW000030
name: SubPathway

Gluconeogenesis
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway702
pathway component: SubPathwayInteraction702
xref: Reference/SMPDB SMP0000128
xref: Reference/PathWhiz PW000152
name: SubPathway

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

Glucose-Alanine Cycle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway678
pathway order: SubPathwayInteractionSteps/SubPathway710
pathway order: SubPathwayInteractionSteps/SubPathway709
pathway order: SubPathwayInteractionSteps/SubPathway713
pathway order: SubPathwayInteractionSteps/SubPathway701
pathway component: SubPathwayInteraction701
pathway component: SubPathwayInteraction713
pathway component: SubPathwayInteraction710
pathway component: SubPathwayInteraction678
pathway component: SubPathwayInteraction709
xref: Reference/PathWhiz PW000046
xref: Reference/SMPDB SMP0000127
name: SubPathway

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

Reference/SMPDB SMP0000055
type: UnificationXref
id: SMP0000055
db: SMPDB

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

ChemicalStructure/PW C000105
type: ChemicalStructure
structure format: SMILES
structure data: C[C@H](N)C(O)=O

Protein/278 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Aspartate aminotransferase, mitochondrial
stoichiometric coefficient: 2.0

Reference/PubChem-compound 5950
type: UnificationXref
id: 5950
db: PubChem-compound

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

ChemicalStructure/PW C001441
type: ChemicalStructure
structure format: SMILES
structure data: OP(O)(O)=O

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

NADH
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001487
xref: Reference/CAS 58-68-4
xref: Reference/PubChem-compound 439153
xref: Reference/BioCyc NADH
xref: Reference/ChemSpider 388299
xref: Reference/KEGG Compound C00004
xref: Reference/ChEBI CHEBI 16908
chemical formula: C21H29N7O14P2
molecular weight: 665.12476
structure: ChemicalStructure/PW C001144

Reference/ChemSpider 6082
type: UnificationXref
id: 6082
db: ChemSpider

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

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

Reference/SMPDB SMP0000067
type: UnificationXref
id: SMP0000067
db: SMPDB

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

Alanine aminotransferase 1
type: Complex
component stoichiometry: Protein/795 Mitochondrial Matrix/Stoichiometry/2.0
cellular location: Mitochondrial Matrix
component: Alanine aminotransferase 1

Protein/795 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Alanine aminotransferase 1
stoichiometric coefficient: 2.0

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

Reference/SMPDB SMP0000072
type: UnificationXref
id: SMP0000072
db: SMPDB

Reference/HMDB HMDB0000243
type: UnificationXref
id: HMDB0000243
db: HMDB

Reference/GENE ONTOLOGY GO 0005739
type: UnificationXref
id: GO:0005739
db: GENE ONTOLOGY

Arginine and Proline Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway715
pathway component: SubPathwayInteraction715
xref: Reference/PathWhiz PW000010
xref: Reference/SMPDB SMP0000020
name: SubPathway

Reference/GENE ONTOLOGY GO 0005615
type: UnificationXref
id: GO:0005615
db: GENE ONTOLOGY

Adenosine diphosphate
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00008
xref: Reference/PubChem-compound 6022
xref: Reference/ChemSpider 5800
xref: Reference/CAS 58-64-0
xref: Reference/BioCyc ADP
xref: Reference/ChEBI CHEBI 16761
xref: Reference/HMDB HMDB0001341
chemical formula: C10H15N5O10P2
molecular weight: 427.02942
structure: ChemicalStructure/PW C001034

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

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

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

Selenoamino Acid Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway679
pathway component: SubPathwayInteraction679
xref: Reference/SMPDB SMP0000029
xref: Reference/PathWhiz PW000007
name: SubPathway

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

Porphyrin Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway704
pathway component: SubPathwayInteraction704
xref: Reference/PathWhiz PW000158
xref: Reference/SMPDB SMP0000024
name: SubPathway

Compound/PW C000723 Mitochondrial Matrix/Stoichiometry/0.0
type: Stoichiometry
physical entity: Citrulline
stoichiometric coefficient: 0.0

Reference/UniProt P05089
type: UnificationXref
id: P05089
db: UniProt

Reference/SMPDB SMP0000009
type: UnificationXref
id: SMP0000009
db: SMPDB

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

Reference/SMPDB SMP0000128
type: UnificationXref
id: SMP0000128
db: SMPDB

Reference/SMPDB SMP0000129
type: UnificationXref
id: SMP0000129
db: SMPDB

Reference/SMPDB SMP0000127
type: UnificationXref
id: SMP0000127
db: SMPDB

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

TransportCatalysis12
type: Catalysis
controller: Mitochondrial ornithine transporter 1
controlled: Transport39
control type: ACTIVATION

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

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

Reference/ChemSpider 5682
type: UnificationXref
id: 5682
db: ChemSpider

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

Oxalacetic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Oxalacetic acid
name: SubPathwayInput

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

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

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

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

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

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

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

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

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

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

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

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

Reference/SMPDB SMP0000029
type: UnificationXref
id: SMP0000029
db: SMPDB

Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: NAD
stoichiometric coefficient: 1.0

Homo sapiens
type: BioSource
xref: Reference/TAXONOMY 9606

Reference/ChemSpider 388299
type: UnificationXref
id: 388299
db: ChemSpider

Reference/CAS 127-17-3
type: UnificationXref
id: 127-17-3
db: CAS

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

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

Reference/SMPDB SMP0000024
type: UnificationXref
id: SMP0000024
db: SMPDB

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

Reference/SMPDB SMP0000020
type: UnificationXref
id: SMP0000020
db: SMPDB

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

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

Transport39
type: Transport
participant stoichiometry: Compound/PW C000723 Mitochondrial Matrix/Stoichiometry/0.0
participant stoichiometry: Compound/PW C000140 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000723 Cytoplasm/Stoichiometry/0.0
participant stoichiometry: Compound/PW C000140 Cytoplasm/Stoichiometry/1.0
right: Citrulline
right: Ornithine
spontaneous: false
left: Citrulline
left: Ornithine
name: Ornithine (→), Citrulline (←) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix
conversion direction: LEFT_TO_RIGHT

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

Transport31
type: Transport
participant stoichiometry: Compound/PW C000095 Mitochondrial Intermembrane Space/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000123 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000123 Mitochondrial Intermembrane Space/Stoichiometry/1.0
right: L-Aspartic acid
right: L-Glutamic acid
spontaneous: false
left: L-Aspartic acid
left: L-Glutamic acid
name: L-Aspartic acid (→), L-Glutamic acid (←) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Mitochondrial Intermembrane Space
conversion direction: LEFT_TO_RIGHT

Reference/ChEBI CHEBI 16761
type: UnificationXref
id: CHEBI:16761
db: ChEBI

Reference/SMPDB SMP0000036
type: UnificationXref
id: SMP0000036
db: SMPDB

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

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

Oxoglutaric acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Oxoglutaric acid
name: SubPathwayOutput
name: SubPathwayInput

Reference/PubChem-compound 5893
type: UnificationXref
id: 5893
db: PubChem-compound

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

L-Alanine
type: SmallMolecule
cellular location: Cytoplasm
entity reference: L-Alanine

Reference/PathWhiz PW000019
type: UnificationXref
id: PW000019
db: PathWhiz

Mitochondrial Intermembrane Space
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005758

Reference/ChEBI CHEBI 32816
type: UnificationXref
id: CHEBI:32816
db: ChEBI

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

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

Reference/PathWhiz PW000010
type: UnificationXref
id: PW000010
db: PathWhiz

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

6.3.4.16
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001034 Mitochondrial Matrix/Stoichiometry/2.0
participant stoichiometry: Compound/PW C001316 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Mitochondrial Matrix/Stoichiometry/2.0
participant stoichiometry: Compound/PW C000862 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001441 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Mitochondrial Matrix/Stoichiometry/1.0
right: Phosphoric acid
right: Carbamoyl phosphate
right: Adenosine diphosphate
spontaneous: false
EC number: 6.3.4.16
left: Water
left: Carbon dioxide
left: Ammonia
left: Adenosine triphosphate
name: 2 Adenosine triphosphate + Ammonia + Carbon dioxide + Water → 2 Adenosine diphosphate + Carbamoyl phosphate + Phosphoric acid
conversion direction: LEFT_TO_RIGHT

ChemicalStructure/PW C000414
type: ChemicalStructure
structure format: SMILES

Reference/UniProt P31327
type: UnificationXref
id: P31327
db: UniProt

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

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

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

Reference/ChEBI CHEBI 30744
type: UnificationXref
id: CHEBI:30744
db: ChEBI

Reference/PathWhiz PW000009
type: UnificationXref
id: PW000009
db: PathWhiz

Reference/PathWhiz PW000007
type: UnificationXref
id: PW000007
db: PathWhiz

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

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

Reference/PathWhiz PW000002
type: UnificationXref
id: PW000002
db: PathWhiz

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

Reference/PathWhiz PW000001
type: UnificationXref
id: PW000001
db: PathWhiz

ReactionCatalysis133
type: Catalysis
controller: Carbamoyl-phosphate synthase [ammonia], mitochondrial
controlled: 6.3.4.16
control type: ACTIVATION

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

ReactionCatalysis372
type: Catalysis
controller: Aspartate aminotransferase, mitochondrial
controlled: Reaction78
control type: ACTIVATION

Reference/PathWhiz PW000003
type: UnificationXref
id: PW000003
db: PathWhiz

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

Reference/UniProt Q15758
type: UnificationXref
id: Q15758
db: UniProt

Reference/PubChem-compound 1060
type: UnificationXref
id: 1060
db: PubChem-compound

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

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

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

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

Compound/PW C000164 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvic acid
stoichiometric coefficient: 1.0

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

Reference/UniProt P00505
type: UnificationXref
id: P00505
db: UniProt

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

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

Protein/292 Cytoplasm/Stoichiometry/3.0
type: Stoichiometry
physical entity: Arginase-1
stoichiometric coefficient: 3.0