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

Protein/4317 Cytoplasm/Stoichiometry/2.0
type: Stoichiometry
physical entity: Prolyl 4-hydroxylase subunit alpha-3
stoichiometric coefficient: 2.0

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

Compound/PW C002643 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Hydroxyproline
stoichiometric coefficient: 1.0

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

Reference/ChEBI CHEBI 27391
type: UnificationXref
id: CHEBI:27391
db: ChEBI

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

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

SubPathwayInteraction118671
type: Interaction
name: SubPathway118671Reaction
name: SubPathwayReaction
participant: L-Arginine

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

SubPathwayInteraction118670
type: Interaction
name: SubPathway118670Reaction
name: SubPathwayReaction
participant: Ornithine

SubPathwayInteraction118668
type: Interaction
name: SubPathway118668Reaction
name: SubPathwayReaction
participant: Fumaric acid

SubPathwayInteraction118669
type: Interaction
name: SubPathway118669Reaction
name: SubPathwayReaction
participant: L-Aspartic acid

Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P30038
organism: Homo sapiens

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

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

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

Reference/UniProt Q5T160
type: UnificationXref
id: Q5T160
db: UniProt

Reference/UniProt P29475
type: UnificationXref
id: P29475
db: UniProt

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

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

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

Transport14622
type: Transport
participant stoichiometry: Compound/PW C000106 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001878/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000106/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001878 Cytoplasm/Stoichiometry/1.0
right: D-Proline
right: L-Proline
spontaneous: false
left: D-Proline
left: L-Proline
name: L-Proline (→), D-Proline (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Blood Vessel
conversion direction: LEFT_TO_RIGHT

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

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

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

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

TransportCatalysis5387
type: Catalysis
controller: Mitochondrial ornithine transporter 1
controlled: Transport14570
control type: ACTIVATION

TransportCatalysis5388
type: Catalysis
controller: Mitochondrial ornithine transporter 1
controlled: Transport14571
control type: ACTIVATION

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

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

Compound/PW C000083 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Guanidoacetic acid
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 16480
type: UnificationXref
id: CHEBI:16480
db: ChEBI

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

Compound/PW C001878 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: D-Proline
stoichiometric coefficient: 1.0

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

L-4-Hydroxyglutamate semialdehyde
type: SmallMolecule
cellular location: Mitochondrion
entity reference: L-4-Hydroxyglutamate semialdehyde

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

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

L-Arginyl-tRNA(Arg)
type: Rna
cellular location: Mitochondrial Matrix
entity reference: L-Arginyl-tRNA(Arg)

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

L-4-Hydroxyglutamate semialdehyde
type: SmallMoleculeReference
xref: Reference/PubChem-compound 440851
xref: Reference/KEGG Compound C05938
xref: Reference/ChemSpider 389693
xref: Reference/HMDB HMDB0006556
xref: Reference/ChEBI CHEBI 27809
chemical formula: C5H9NO4
molecular weight: 147.05316
structure: ChemicalStructure/PW C002808

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

Reference/CAS 979-92-0
type: UnificationXref
id: 979-92-0
db: CAS

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

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

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

Protein/151 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
stoichiometric coefficient: 2.0

Reference/HMDB HMDB0006556
type: UnificationXref
id: HMDB0006556
db: HMDB

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

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

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

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

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

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

Reference/KEGG Compound C05938
type: UnificationXref
id: C05938
db: KEGG Compound

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

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

Reference/UniProt Q7Z4N8
type: UnificationXref
id: Q7Z4N8
db: UniProt

Reference/KEGG Compound C05933
type: UnificationXref
id: C05933
db: KEGG Compound

Reference/KEGG Compound C00005
type: UnificationXref
id: C00005
db: KEGG Compound

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

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

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

Reference/CAS 2485-33-8
type: UnificationXref
id: 2485-33-8
db: CAS

Reference/CAS 496-92-4
type: UnificationXref
id: 496-92-4
db: CAS

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

Carbamoyl-phosphate synthase [ammonia], mitochondrial
type: ProteinReference
xref: Reference/UniProt P31327
organism: BiologicalState13

N(omega)-Hydroxyarginine
type: SmallMolecule
cellular location: Cell Membrane
entity reference: N(omega)-Hydroxyarginine

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

Reference/KEGG Compound C00019
type: UnificationXref
id: C00019
db: KEGG Compound

Reference/CAS 352-97-6
type: UnificationXref
id: 352-97-6
db: CAS

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

ReactionCatalysis2194
type: Catalysis
controller: D-amino-acid oxidase
controlled: 1.4.3.3
control type: ACTIVATION

ReactionCatalysis2193
type: Catalysis
controller: D-amino-acid oxidase
controlled: 1.4.3.3
control type: ACTIVATION

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

Protein/538 Cytoplasm/Stoichiometry/2.0
type: Stoichiometry
physical entity: Bifunctional glutamate/proline--tRNA ligase
stoichiometric coefficient: 2.0

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

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

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

Reference/CAS 10102-43-9
type: UnificationXref
id: 10102-43-9
db: CAS

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

ReactionCatalysis2196
type: Catalysis
controller: Proline dehydrogenase 1, mitochondrial
controlled: 1.5.5.2
control type: ACTIVATION

ReactionCatalysis2195
type: Catalysis
controller: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
controlled: 1.2.1.88
control type: ACTIVATION

Compound/PW C000078 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glycine
stoichiometric coefficient: 1.0

ReactionCatalysis2198
type: Catalysis
controller: Aspartate aminotransferase, cytoplasmic
controlled: Reaction2305
control type: ACTIVATION

ReactionCatalysis2197
type: Catalysis
controller: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
controlled: 1.2.1.88
control type: ACTIVATION

ReactionCatalysis2199
type: Catalysis
controller: Nitric-oxide synthase, brain
controlled: 1.14.13.39
control type: ACTIVATION

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

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

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

Reference/KEGG Compound C00021
type: UnificationXref
id: C00021
db: KEGG Compound

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

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

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

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

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

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

RNA/14 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: tRNA(Arg)
stoichiometric coefficient: 1.0

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

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

Oxygen
type: SmallMolecule
cellular location: Cell Membrane
entity reference: Oxygen

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

Reference/ChEBI CHEBI 29171
type: UnificationXref
id: CHEBI:29171
db: ChEBI

1-Pyrroline-4-hydroxy-2-carboxylate
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0002234
xref: Reference/KEGG Compound C04282
xref: Reference/PubChem-compound 440282
xref: Reference/BioCyc CPD-1128
xref: Reference/CAS 9054-77-7
xref: Reference/ChEBI CHEBI 16352
xref: Reference/ChemSpider 389257
chemical formula: C5H7NO3
molecular weight: 129.04259
structure: ChemicalStructure/PW C001512

Reference/KEGG Compound C01127
type: UnificationXref
id: C01127
db: KEGG Compound

Reference/KEGG Compound C00037
type: UnificationXref
id: C00037
db: KEGG Compound

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

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

ChemicalStructure/PW C000174
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CCC(O)=O

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

L-Prolyl-tRNA(Pro)
type: RnaReference
xref: Reference/ChEBI CHEBI 29154
organism: Homo sapiens

L-Arginyl-tRNA(Arg)
type: RnaReference
xref: Reference/ChEBI CHEBI 18366
organism: Homo sapiens

tRNA(Pro)
type: RnaReference
xref: Reference/ChEBI CHEBI 29177
organism: Homo sapiens

tRNA(Arg)
type: RnaReference
xref: Reference/ChEBI CHEBI 29171
organism: Homo sapiens

Reference/ChEBI CHEBI 29177
type: UnificationXref
id: CHEBI:29177
db: ChEBI

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

4-Hydroxyproline
type: SmallMolecule
cellular location: Cytoplasm
entity reference: 4-Hydroxyproline

Reference/ChemSpider 128566
type: UnificationXref
id: 128566
db: ChemSpider

Reference/KEGG Compound C00042
type: UnificationXref
id: C00042
db: KEGG Compound

Water
type: SmallMolecule
cellular location: Cell Membrane
entity reference: Water

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

1.2.1.88
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/2.0
participant stoichiometry: Compound/PW C000095 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001003 Mitochondrial Matrix/Stoichiometry/1.0
right: L-Glutamic acid
right: NADH
spontaneous: false
EC number: 1.2.1.88
left: Water
left: 1-Pyrroline-5-carboxylic acid
left: NAD
name: 1-Pyrroline-5-carboxylic acid + NAD + 2 Water ↔ L-Glutamic acid + NADH
conversion direction: REVERSIBLE

ChemicalStructure/PW C001034
type: ChemicalStructure
structure format: SMILES

Reaction2298
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000106 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001878 Cytosol/Stoichiometry/1.0
right: D-Proline
spontaneous: false
left: L-Proline
name: L-Proline → D-Proline
conversion direction: LEFT_TO_RIGHT

Compound/PW C000749 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-Adenosylhomocysteine
stoichiometric coefficient: 1.0

1.4.3.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001878 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001783 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002984 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Lysosome/Stoichiometry/1.0
right: 1-Pyrroline-2-carboxylic acid
right: Hydrogen peroxide
right: Ammonia
spontaneous: false
EC number: 1.4.3.3
left: D-Proline
left: Water
left: Oxygen
name: D-Proline + Oxygen + Water → 1-Pyrroline-2-carboxylic acid + Ammonia + Hydrogen peroxide
conversion direction: LEFT_TO_RIGHT

Reference/HMDB HMDB0003411
type: UnificationXref
id: HMDB0003411
db: HMDB

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

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

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

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

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

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

Reference/KEGG Compound C03564
type: UnificationXref
id: C03564
db: KEGG Compound

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

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

Bifunctional glutamate/proline--tRNA ligase
type: Complex
component stoichiometry: Protein/538 Cytoplasm/Stoichiometry/2.0
cellular location: Cytoplasm
component: Bifunctional glutamate/proline--tRNA ligase

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

Reference/ChEBI CHEBI 29154
type: UnificationXref
id: CHEBI:29154
db: ChEBI

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

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

BiologicalState13
type: BioSource
xref: Reference/TAXONOMY 9606
name: Homo sapiens, Liver
tissue: Liver

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

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

Reference/KEGG Compound C01157
type: UnificationXref
id: C01157
db: KEGG Compound

Reference/UniProt Q7Z2H8
type: UnificationXref
id: Q7Z2H8
db: UniProt

Reference/HMDB HMDB0002104
type: UnificationXref
id: HMDB0002104
db: HMDB

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

Reference/UniProt P50440
type: UnificationXref
id: P50440
db: UniProt

Reference/HMDB HMDB0000162
type: UnificationXref
id: HMDB0000162
db: HMDB

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

Reference/ChemSpider 31983
type: UnificationXref
id: 31983
db: ChemSpider

Probable arginine--tRNA ligase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q5T160
organism: Homo sapiens

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

Compound/PW C002093 Cell Membrane/Stoichiometry/2.0
type: Stoichiometry
physical entity: N(omega)-Hydroxyarginine
stoichiometric coefficient: 2.0

Reference/KEGG Compound C01165
type: UnificationXref
id: C01165
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

Proline dehydrogenase 1, mitochondrial
type: ProteinReference
xref: Reference/UniProt O43272
organism: Homo sapiens

Reference/PubChem-compound 145742
type: UnificationXref
id: 145742
db: PubChem-compound

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

Reference/CAS 2139-03-9
type: UnificationXref
id: 2139-03-9
db: CAS

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

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

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

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

Reference/CAS 9054-77-7
type: UnificationXref
id: 9054-77-7
db: CAS

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

Compound/PW C002808 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-4-Hydroxyglutamate semialdehyde
stoichiometric coefficient: 1.0

Reference/SMPDB SMP0125627
type: UnificationXref
id: SMP0125627
db: SMPDB

Reference/BioCyc CPD-1128
type: UnificationXref
id: CPD-1128
db: BioCyc

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

L-Arginine
type: SmallMolecule
cellular location: Cell Membrane
entity reference: L-Arginine

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

4-Hydroxy-L-glutamic acid
type: SmallMoleculeReference
xref: Reference/ChemSpider 4573749
xref: Reference/ChEBI CHEBI 16338
xref: Reference/HMDB HMDB0002273
xref: Reference/PubChem-compound 5460078
xref: Reference/KEGG Compound C03079
xref: Reference/CAS 2485-33-8
xref: Reference/BioCyc L-ERYTHRO-4-HYDROXY-GLUTAMATE
chemical formula: C5H9NO5
molecular weight: 163.04807
structure: ChemicalStructure/PW C001535

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

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

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

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

Compound/PW C001164 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Phosphocreatine
stoichiometric coefficient: 1.0

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

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

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

Bifunctional glutamate/proline--tRNA ligase
type: ProteinReference
xref: Reference/UniProt P07814
organism: Homo sapiens

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

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

Compound/PW C001878/Stoichiometry/1.0
type: Stoichiometry
physical entity: D-Proline
stoichiometric coefficient: 1.0

4-Hydroxyproline
type: SmallMoleculeReference
xref: Reference/ChemSpider 5605
xref: Reference/ChEBI CHEBI 18095
xref: Reference/HMDB HMDB0000725
xref: Reference/CAS 51-35-4
xref: Reference/PubChem-compound 5810
xref: Reference/KEGG Compound C01157
xref: Reference/BioCyc L-4-HYDROXY-PROLINE
chemical formula: C5H9NO3
molecular weight: 131.05824
structure: ChemicalStructure/PW C002643

ChemicalStructure/PW C001057
type: ChemicalStructure
structure format: SMILES
structure data: OC1CC(N=C1)C(O)=O

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

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/UniProt P07814
type: UnificationXref
id: P07814
db: UniProt

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

Reference/BioCyc CPD-656
type: UnificationXref
id: CPD-656
db: BioCyc

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

ChemicalStructure/PW C001535
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](C[C@@H](O)C(O)=O)C(O)=O

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

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

4-Hydroxy-L-glutamic acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 4-Hydroxy-L-glutamic acid

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

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

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

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

Reference/PubChem-compound 599
type: UnificationXref
id: 599
db: PubChem-compound

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

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

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

ChemicalStructure/PW C002643
type: ChemicalStructure
structure format: SMILES
structure data: O[C@H]1CN[C@@H](C1)C(O)=O

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

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

Creatine
type: SmallMoleculeReference
xref: Reference/ChemSpider 566
xref: Reference/PubChem-compound 586
xref: Reference/CAS 57-00-1
xref: Reference/HMDB HMDB0000064
xref: Reference/KEGG Compound C00300
xref: Reference/BioCyc CREATINE
xref: Reference/ChEBI CHEBI 16919
chemical formula: C4H9N3O2
molecular weight: 131.06947
structure: ChemicalStructure/PW C000046

Reference/PubChem-compound 5460078
type: UnificationXref
id: 5460078
db: PubChem-compound

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

Reference/ChemSpider 167744
type: UnificationXref
id: 167744
db: ChemSpider

ChemicalStructure/PW C001512
type: ChemicalStructure
structure format: SMILES
structure data: OC1CN=C(C1)C(O)=O

Compound/PW C000046 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Creatine
stoichiometric coefficient: 1.0

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

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

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

Reference/UniProt Q14353
type: UnificationXref
id: Q14353
db: UniProt

Reference/CAS 485-80-3
type: UnificationXref
id: 485-80-3
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

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

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

Compound/PW C000921 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-Adenosylmethionine
stoichiometric coefficient: 1.0

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

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

Reference/UniProt Q96C36
type: UnificationXref
id: Q96C36
db: UniProt

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

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

S-Adenosylhomocysteine
type: SmallMolecule
cellular location: Cytoplasm
entity reference: S-Adenosylhomocysteine

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

Reference/BioCyc DELTA1-PYRROLINE 2-CARBOXYLATE
type: UnificationXref
id: DELTA1-PYRROLINE_2-CARBOXYLATE
db: BioCyc

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

Pyrroline-5-carboxylate reductase 2
type: Complex
component stoichiometry: Protein/299 Cytoplasm/Stoichiometry/10.0
cellular location: Cytoplasm
component: Pyrroline-5-carboxylate reductase 2

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

Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Water
stoichiometric coefficient: 2.0

Phosphocreatine
type: SmallMoleculeReference
xref: Reference/BioCyc CREATINE-P
xref: Reference/ChemSpider 567
xref: Reference/HMDB HMDB0001511
xref: Reference/PubChem-compound 587
xref: Reference/KEGG Compound C02305
xref: Reference/CAS 67-07-2
xref: Reference/ChEBI CHEBI 17287
chemical formula: C4H10N3O5P
molecular weight: 211.03581
structure: ChemicalStructure/PW C001164

Reference/KEGG Compound C04281
type: UnificationXref
id: C04281
db: KEGG Compound

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

D-Proline
type: SmallMolecule
cellular location: Cytoplasm
entity reference: D-Proline

Reference/KEGG Compound C04282
type: UnificationXref
id: C04282
db: KEGG Compound

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

4-Hydroxyproline
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 4-Hydroxyproline

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

Glycine
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000123
xref: Reference/BioCyc GLY
xref: Reference/ChemSpider 730
xref: Reference/PubChem-compound 5257127
xref: Reference/CAS 56-40-6
xref: Reference/ChEBI CHEBI 15428
xref: Reference/KEGG Compound C00037
chemical formula: C2H5NO2
molecular weight: 75.03203
structure: ChemicalStructure/PW C000078

Ornithine
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Ornithine
name: SubPathwayOutput

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

Reference/CAS 344-25-2
type: UnificationXref
id: 344-25-2
db: CAS

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

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

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

Guanidoacetic acid
type: SmallMoleculeReference
xref: Reference/ChemSpider 743
xref: Reference/ChEBI CHEBI 16344
xref: Reference/BioCyc GUANIDOACETIC ACID
xref: Reference/CAS 352-97-6
xref: Reference/HMDB HMDB0000128
xref: Reference/KEGG Compound C00581
xref: Reference/PubChem-compound 3946848
chemical formula: C3H7N3O2
molecular weight: 117.053825
structure: ChemicalStructure/PW C000083

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

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

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

Reference/PubChem-compound 586
type: UnificationXref
id: 586
db: PubChem-compound

Reference/ChEBI CHEBI 15741
type: UnificationXref
id: CHEBI:15741
db: ChEBI

Reference/PubChem-compound 587
type: UnificationXref
id: 587
db: PubChem-compound

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

Reference/BioCyc D-4-HYDROXY-2-KETO-GLUTARATE
type: UnificationXref
id: D-4-HYDROXY-2-KETO-GLUTARATE
db: BioCyc

Reference/PubChem-compound 1110
type: UnificationXref
id: 1110
db: PubChem-compound

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

Glycine amidinotransferase, mitochondrial
type: Complex
component stoichiometry: Protein/447 Mitochondrial Matrix/Stoichiometry/2.0
cellular location: Mitochondrial Matrix
component: Glycine amidinotransferase, mitochondrial

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

Protein/871 Cell Membrane/Stoichiometry/2.0
type: Stoichiometry
physical entity: Nitric oxide synthase, brain
stoichiometric coefficient: 2.0

Compound/PW C001391 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Hydroxy-2-oxoglutaric acid
stoichiometric coefficient: 1.0

RNA/13 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Arginyl-tRNA(Arg)
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000123
type: UnificationXref
id: HMDB0000123
db: HMDB

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

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

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

Reference/HMDB HMDB0000128
type: UnificationXref
id: HMDB0000128
db: HMDB

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

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

Reference/CAS 147-85-3
type: UnificationXref
id: 147-85-3
db: CAS

4-Hydroxy-2-oxoglutaric acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 599
xref: Reference/HMDB HMDB0002070
xref: Reference/ChEBI CHEBI 30923
xref: Reference/CAS 1187-99-1
xref: Reference/KEGG Compound C01127
xref: Reference/BioCyc D-4-HYDROXY-2-KETO-GLUTARATE
xref: Reference/ChemSpider 579
chemical formula: C5H6O6
molecular weight: 162.01643
structure: ChemicalStructure/PW C001391

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

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

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

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

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

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

Compound/PW C001535 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Hydroxy-L-glutamic acid
stoichiometric coefficient: 1.0

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

2.7.3.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001034 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001164 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000046 Cytoplasm/Stoichiometry/1.0
right: Phosphocreatine
right: Adenosine diphosphate
spontaneous: false
EC number: 2.7.3.2
left: Creatine
left: Adenosine triphosphate
name: Adenosine triphosphate + Creatine → Adenosine diphosphate + Phosphocreatine
conversion direction: LEFT_TO_RIGHT

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

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

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

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

Prolyl 4-hydroxylase subunit alpha-3
type: ProteinReference
xref: Reference/UniProt Q7Z4N8
organism: Homo sapiens

Reference/UniProt P30038
type: UnificationXref
id: P30038
db: UniProt

Reference/CAS 67-07-2
type: UnificationXref
id: 67-07-2
db: CAS

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

Reference/ChemSpider 1078
type: UnificationXref
id: 1078
db: ChemSpider

Reference/ChemSpider 389057
type: UnificationXref
id: 389057
db: ChemSpider

Reference/BioCyc NITRIC-OXIDE
type: UnificationXref
id: NITRIC-OXIDE
db: BioCyc

Reference/HMDB HMDB0000939
type: UnificationXref
id: HMDB0000939
db: HMDB

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

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

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

Reference/CAS 22573-88-2
type: UnificationXref
id: 22573-88-2
db: CAS

Creatine kinase B-type
type: Protein
cellular location: Cytoplasm
entity reference: Creatine kinase B-type

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

1.2.1.88
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 C001144 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001415 Mitochondrial Matrix/Stoichiometry/1.0
right: L-Glutamic acid
right: NADH
right: Hydrogen Ion
spontaneous: false
EC number: 1.2.1.88
left: L-Glutamic γ-semialdehyde
left: Water
left: NAD
name: L-Glutamic γ-semialdehyde + NAD + Water → Hydrogen Ion + L-Glutamic acid + NADH
conversion direction: LEFT_TO_RIGHT

Compound/PW C002643 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Hydroxyproline
stoichiometric coefficient: 1.0

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

Creatine kinase B-type
type: Complex
component stoichiometry: Protein/683 Cytoplasm/Stoichiometry/2.0
cellular location: Cytoplasm
component: Creatine kinase B-type

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

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

Compound/PW C001420 Cell Membrane/Stoichiometry/4.0
type: Stoichiometry
physical entity: Water
stoichiometric coefficient: 4.0

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

Reference/ChEBI CHEBI 30923
type: UnificationXref
id: CHEBI:30923
db: ChEBI

Transport14571
type: Transport
participant stoichiometry: Compound/PW C000140 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000140 Cytoplasm/Stoichiometry/1.0
right: Ornithine
spontaneous: false
left: Ornithine
name: Ornithine (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix
conversion direction: LEFT_TO_RIGHT

Transport14570
type: Transport
participant stoichiometry: Compound/PW C000723 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000723 Cytoplasm/Stoichiometry/1.0
right: Citrulline
spontaneous: false
left: Citrulline
name: Citrulline (→) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Cytoplasm
conversion direction: LEFT_TO_RIGHT

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

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

Nitric oxide
type: SmallMolecule
cellular location: Cell Membrane
entity reference: Nitric oxide

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

Reference/HMDB HMDB0000725
type: UnificationXref
id: HMDB0000725
db: HMDB

Guanidinoacetate N-methyltransferase
type: Protein
cellular location: Cytoplasm
entity reference: Guanidinoacetate N-methyltransferase

Compound/PW C001034 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Adenosine diphosphate
stoichiometric coefficient: 1.0

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

Reference/CAS 110-15-6
type: UnificationXref
id: 110-15-6
db: CAS

Phosphoric acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Phosphoric acid

Nitric oxide synthase, brain
type: Protein
cellular location: Cell Membrane
entity reference: Nitric oxide synthase, brain

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

1.14.13.39
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001065 Cell Membrane/Stoichiometry/4.0
participant stoichiometry: Compound/PW C000396 Cell Membrane/Stoichiometry/2.0
participant stoichiometry: Compound/PW C000146 Cell Membrane/Stoichiometry/3.0
participant stoichiometry: Compound/PW C002093 Cell Membrane/Stoichiometry/2.0
participant stoichiometry: Compound/PW C000143 Cell Membrane/Stoichiometry/3.0
participant stoichiometry: Compound/PW C001420 Cell Membrane/Stoichiometry/4.0
participant stoichiometry: Compound/PW C001867 Cell Membrane/Stoichiometry/2.0
right: Nitric oxide
right: NADP
right: N(omega)-Hydroxyarginine
right: Water
spontaneous: false
EC number: 1.14.13.39
left: Oxygen
left: L-Arginine
left: NADPH
name: 2 L-Arginine + 3 NADPH + 4 Oxygen → 2 N(omega)-Hydroxyarginine + 3 NADP + 2 Nitric oxide + 4 Water
conversion direction: LEFT_TO_RIGHT

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

Reaction2305
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000123 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001535 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001391 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000148 Cytosol/Stoichiometry/1.0
right: L-Aspartic acid
right: 4-Hydroxy-2-oxoglutaric acid
spontaneous: false
left: Oxalacetic acid
left: 4-Hydroxy-L-glutamic acid
name: 4-Hydroxy-L-glutamic acid + Oxalacetic acid → 4-Hydroxy-2-oxoglutaric acid + L-Aspartic acid
conversion direction: LEFT_TO_RIGHT

1.5.5.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002808 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001535 Mitochondrion/Stoichiometry/1.0
right: L-4-Hydroxyglutamate semialdehyde
spontaneous: false
EC number: 1.5.5.2
left: 4-Hydroxy-L-glutamic acid
name: 4-Hydroxy-L-glutamic acid ↔ L-4-Hydroxyglutamate semialdehyde
conversion direction: REVERSIBLE

N(omega)-Hydroxyarginine
type: SmallMoleculeReference
xref: Reference/CAS 53054-07-2
xref: Reference/PubChem-compound 440849
xref: Reference/ChEBI CHEBI 43088
xref: Reference/ChemSpider 389691
xref: Reference/KEGG Compound C05933
xref: Reference/HMDB HMDB0004224
chemical formula: C6H14N4O3
molecular weight: 190.1066
structure: ChemicalStructure/PW C002093

1.2.1.88
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002808 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001535 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000146 Mitochondrion/Stoichiometry/1.0
right: NADPH
right: L-4-Hydroxyglutamate semialdehyde
spontaneous: false
EC number: 1.2.1.88
left: NAD
left: 4-Hydroxy-L-glutamic acid
left: Water
name: 4-Hydroxy-L-glutamic acid + NAD + Water → L-4-Hydroxyglutamate semialdehyde + NADPH
conversion direction: LEFT_TO_RIGHT

1.4.3.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001512 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001783 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002643 Lysosome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Lysosome/Stoichiometry/1.0
right: 1-Pyrroline-4-hydroxy-2-carboxylate
right: Hydrogen peroxide
right: Ammonia
spontaneous: false
EC number: 1.4.3.3
left: Water
left: 4-Hydroxyproline
left: Oxygen
name: 4-Hydroxyproline + Oxygen + Water → 1-Pyrroline-4-hydroxy-2-carboxylate + Ammonia + Hydrogen peroxide
conversion direction: LEFT_TO_RIGHT

1.2.1.88
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001057 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001535 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000146 Mitochondrion/Stoichiometry/1.0
right: 4-Hydroxy-L-glutamic acid
right: NADPH
spontaneous: false
EC number: 1.2.1.88
left: NAD
left: Water
left: Pyrroline hydroxycarboxylic acid
name: NAD + Pyrroline hydroxycarboxylic acid + Water → 4-Hydroxy-L-glutamic acid + NADPH
conversion direction: LEFT_TO_RIGHT

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

Cell Membrane
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005886

RNA/11 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Prolyl-tRNA(Pro)
stoichiometric coefficient: 1.0

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

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

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

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

ChemicalStructure/PW C000414
type: ChemicalStructure
structure format: SMILES

Reference/CAS 51-35-4
type: UnificationXref
id: 51-35-4
db: CAS

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

Reference/ChemSpider 389257
type: UnificationXref
id: 389257
db: ChemSpider

NADPH
type: SmallMolecule
cellular location: Cell Membrane
entity reference: NADPH

Reference/KEGG Compound C00763
type: UnificationXref
id: C00763
db: KEGG Compound

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

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

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

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

ChemicalStructure/PW C002808
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](C[C@@H](O)C=O)C(O)=O

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

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

Reference/KEGG Compound C00533
type: UnificationXref
id: C00533
db: KEGG Compound

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

Protein/151 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
stoichiometric coefficient: 2.0

Compound/PW C002643 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Hydroxyproline
stoichiometric coefficient: 1.0

Compound/PW C001057 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyrroline hydroxycarboxylic acid
stoichiometric coefficient: 1.0

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

Reference/ChEBI CHEBI 16680
type: UnificationXref
id: CHEBI:16680
db: ChEBI

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

Reference/KEGG Compound C00300
type: UnificationXref
id: C00300
db: KEGG Compound

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

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

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

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

Prolyl 4-hydroxylase subunit alpha-3
type: Protein
cellular location: Cytoplasm
entity reference: Prolyl 4-hydroxylase subunit alpha-3

Reference/ChemSpider 5605
type: UnificationXref
id: 5605
db: ChemSpider

Compound/PW C000174 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Succinic acid
stoichiometric coefficient: 1.0

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

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

Compound/PW C001057 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyrroline hydroxycarboxylic acid
stoichiometric coefficient: 1.0

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

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

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

Reference/ChemSpider 127983
type: UnificationXref
id: 127983
db: ChemSpider

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

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

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

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

Reference/HMDB HMDB0002273
type: UnificationXref
id: HMDB0002273
db: HMDB

Reference/HMDB HMDB0006875
type: UnificationXref
id: HMDB0006875
db: HMDB

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

Reference/HMDB HMDB0001185
type: UnificationXref
id: HMDB0001185
db: HMDB

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

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

Prolinemia Type II
type: Pathway
pathway order: PathwayTransport/2513483
pathway order: PathwayTransport/2513484
pathway order: PathwayTransport/2513482
pathway order: PathwayEnzymeReaction/2513463
pathway order: PathwayEnzymeReaction/2513464
pathway order: PathwayEnzymeReaction/2513461
pathway order: PathwayEnzymeReaction/2513462
pathway order: PathwayEnzymeReaction/2513481
pathway order: PathwayEnzymeReaction/2513460
pathway order: PathwayEnzymeReaction/2513480
pathway order: PathwayEnzymeReaction/2513469
pathway order: PathwayEnzymeReaction/2513467
pathway order: PathwayEnzymeReaction/2513468
pathway order: PathwayEnzymeReaction/2513465
pathway order: PathwayEnzymeReaction/2513466
pathway order: PathwaySubPathway/118669
pathway order: PathwaySubPathway/118668
pathway order: PathwayEnzymeReaction/2513474
pathway order: PathwayEnzymeReaction/2513453
pathway order: PathwayEnzymeReaction/2513475
pathway order: PathwayEnzymeReaction/2513472
pathway order: PathwayEnzymeReaction/2513473
pathway order: PathwayEnzymeReaction/2513470
pathway order: PathwayEnzymeReaction/2513471
pathway order: PathwayEnzymeReaction/2513458
pathway order: PathwayEnzymeReaction/2513459
pathway order: PathwayEnzymeReaction/2513456
pathway order: PathwayEnzymeReaction/2513478
pathway order: PathwayEnzymeReaction/2513457
pathway order: PathwayEnzymeReaction/2513479
pathway order: PathwayEnzymeReaction/2513454
pathway order: PathwayEnzymeReaction/2513476
pathway order: PathwaySubPathway/118671
pathway order: PathwayEnzymeReaction/2513455
pathway order: PathwayEnzymeReaction/2513477
pathway order: PathwaySubPathway/118670
pathway component: ReactionCatalysis153
pathway component: ReactionCatalysis18
pathway component: ReactionCatalysis151
pathway component: 1.4.1.3
pathway component: ReactionCatalysis152
pathway component: ReactionCatalysis150
pathway component: ReactionCatalysis12
pathway component: 1.14.13.39
pathway component: 2.7.3.2
pathway component: 1.14.11.2
pathway component: 2.1.4.1
pathway component: Reaction2305
pathway component: 2.1.1.2
pathway component: 1.5.5.2
pathway component: 1.2.1.88
pathway component: 1.4.3.3
pathway component: D-Arginine and D-Ornithine Metabolism
pathway component: ReactionCatalysis146
pathway component: ReactionCatalysis147
pathway component: Reaction649
pathway component: ReactionCatalysis145
pathway component: 6.3.4.16
pathway component: ReactionCatalysis143
pathway component: ReactionCatalysis140
pathway component: ReactionCatalysis141
pathway component: Reaction2298
pathway component: ReactionCatalysis139
pathway component: 3.5.3.1
pathway component: ReactionCatalysis137
pathway component: ReactionCatalysis138
pathway component: ReactionCatalysis38
pathway component: ReactionCatalysis136
pathway component: ReactionCatalysis133
pathway component: Reaction658
pathway component: 2.6.1.13
pathway component: 4.3.2.1
pathway component: ReactionCatalysis2194
pathway component: ReactionCatalysis2193
pathway component: 6.3.4.5

tRNA(Arg)
type: Rna
cellular location: Mitochondrial Matrix
entity reference: tRNA(Arg)

Reference/ChemSpider 389693
type: UnificationXref
id: 389693
db: ChemSpider

Pyrroline hydroxycarboxylic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Pyrroline hydroxycarboxylic acid

Reference/ChemSpider 389691
type: UnificationXref
id: 389691
db: ChemSpider

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

Reference/ChEBI CHEBI 17232
type: UnificationXref
id: CHEBI:17232
db: ChEBI

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

D-Proline
type: SmallMolecule
cellular location: Lysosome
entity reference: D-Proline

Reference/HMDB HMDB0003378
type: UnificationXref
id: HMDB0003378
db: HMDB

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

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

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

Reference/HMDB HMDB0004224
type: UnificationXref
id: HMDB0004224
db: HMDB

Glycine amidinotransferase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Glycine amidinotransferase, mitochondrial

Reference/ChEBI CHEBI 17203
type: UnificationXref
id: CHEBI:17203
db: ChEBI

S-Adenosylmethionine
type: SmallMoleculeReference
xref: Reference/PubChem-compound 24762165
xref: Reference/KEGG Compound C00019
xref: Reference/ChemSpider 31983
xref: Reference/ChEBI CHEBI 15414
xref: Reference/CAS 485-80-3
xref: Reference/BioCyc S-ADENOSYLMETHIONINE
xref: Reference/HMDB HMDB0001185
chemical formula: C15H23N6O5S
molecular weight: 399.14505
structure: ChemicalStructure/PW C000921

1-Pyrroline-2-carboxylic acid
type: SmallMolecule
cellular location: Lysosome
entity reference: 1-Pyrroline-2-carboxylic acid

Compound/PW C001415 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Glutamic γ-semialdehyde
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 16352
type: UnificationXref
id: CHEBI:16352
db: ChEBI

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

Pyrroline hydroxycarboxylic acid
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Pyrroline hydroxycarboxylic acid

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

Reference/PubChem-compound 440046
type: UnificationXref
id: 440046
db: PubChem-compound

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

Reference/PubChem-compound 440282
type: UnificationXref
id: 440282
db: PubChem-compound

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

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

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

Reference/KEGG Compound C00581
type: UnificationXref
id: C00581
db: KEGG Compound

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

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

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

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

Pyrroline-5-carboxylate reductase 2
type: ProteinReference
xref: Reference/UniProt Q96C36
organism: Homo sapiens

RNA/12 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: tRNA(Pro)
stoichiometric coefficient: 1.0

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

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

Reference/PathWhiz PW127191
type: UnificationXref
id: PW127191
db: PathWhiz

Reference/HMDB HMDB0002070
type: UnificationXref
id: HMDB0002070
db: HMDB

ChemicalStructure/PW C000921
type: ChemicalStructure
structure format: SMILES

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

S-Adenosylhomocysteine
type: SmallMoleculeReference
xref: Reference/PubChem-compound 25246222
xref: Reference/ChemSpider 388301
xref: Reference/BioCyc ADENOSYL-HOMO-CYS
xref: Reference/CAS 979-92-0
xref: Reference/KEGG Compound C00021
xref: Reference/ChEBI CHEBI 16680
xref: Reference/HMDB HMDB0000939
chemical formula: C14H20N6O5S
molecular weight: 384.12158
structure: ChemicalStructure/PW C000749

Reference/PubChem-compound 193305
type: UnificationXref
id: 193305
db: PubChem-compound

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

2.1.4.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000083 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000140 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000396 Mitochondrial Matrix/Stoichiometry/1.0
right: Guanidoacetic acid
right: Ornithine
spontaneous: false
EC number: 2.1.4.1
left: Glycine
left: L-Arginine
name: Glycine + L-Arginine ↔ Guanidoacetic acid + Ornithine
conversion direction: REVERSIBLE

2.1.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000749 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000921 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000083 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000046 Cytoplasm/Stoichiometry/1.0
right: Creatine
right: S-Adenosylhomocysteine
spontaneous: false
EC number: 2.1.1.2
left: Guanidoacetic acid
left: S-Adenosylmethionine
name: Guanidoacetic acid + S-Adenosylmethionine → Creatine + S-Adenosylhomocysteine
conversion direction: LEFT_TO_RIGHT

Reference/KEGG Compound C02305
type: UnificationXref
id: C02305
db: KEGG Compound

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

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

Reference/ChemSpider 1030
type: UnificationXref
id: 1030
db: ChemSpider

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

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

Reference/ChEBI CHEBI 18366
type: UnificationXref
id: CHEBI:18366
db: ChEBI

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

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

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

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

Compound/PW C000106/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Proline
stoichiometric coefficient: 1.0

Carbamoyl phosphate
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Carbamoyl phosphate

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

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

Bifunctional glutamate/proline--tRNA ligase
type: Protein
cellular location: Cytoplasm
entity reference: Bifunctional glutamate/proline--tRNA ligase

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

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

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

Protein/447 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Glycine amidinotransferase, mitochondrial
stoichiometric coefficient: 2.0

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

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

Prolyl 4-hydroxylase
type: Complex
component stoichiometry: Protein/4317 Cytoplasm/Stoichiometry/2.0
cellular location: Cytoplasm
component: Prolyl 4-hydroxylase subunit alpha-3

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

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

ChemicalStructure/PW C002093
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CCCNC(=N)NO)C(O)=O

Reference/ChemSpider 388301
type: UnificationXref
id: 388301
db: ChemSpider

Nitric-oxide synthase, brain
type: Complex
component stoichiometry: Protein/871 Cell Membrane/Stoichiometry/2.0
cellular location: Cell Membrane
component: Nitric oxide synthase, brain

Reference/KEGG Compound C00148
type: UnificationXref
id: C00148
db: KEGG Compound

D-Proline
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0003411
xref: Reference/CAS 344-25-2
xref: Reference/BioCyc CPD-656
xref: Reference/ChEBI CHEBI 16313
xref: Reference/PubChem-compound 8988
xref: Reference/KEGG Compound C00763
xref: Reference/ChemSpider 8640
chemical formula: C5H9NO2
molecular weight: 115.06333
structure: ChemicalStructure/PW C001878

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

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

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

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

Reference/HMDB HMDB0000254
type: UnificationXref
id: HMDB0000254
db: HMDB

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

Reference/BioCyc GUANIDOACETIC ACID
type: UnificationXref
id: GUANIDOACETIC_ACID
db: BioCyc

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

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

Adenosine diphosphate
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Adenosine diphosphate

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

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

4-Hydroxy-L-glutamic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: 4-Hydroxy-L-glutamic acid

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

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

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

Compound/PW C000083 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Guanidoacetic acid
stoichiometric coefficient: 1.0

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

1-Pyrroline-2-carboxylic acid
type: SmallMoleculeReference
xref: Reference/CAS 2139-03-9
xref: Reference/PubChem-compound 440046
xref: Reference/HMDB HMDB0006875
xref: Reference/BioCyc DELTA1-PYRROLINE 2-CARBOXYLATE
xref: Reference/KEGG Compound C03564
xref: Reference/ChemSpider 389057
xref: Reference/ChEBI CHEBI 36761
chemical formula: C5H7NO2
molecular weight: 113.047676
structure: ChemicalStructure/PW C002984

Compound/PW C000106 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Proline
stoichiometric coefficient: 1.0

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

Compound/PW C001057 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyrroline hydroxycarboxylic acid
stoichiometric coefficient: 1.0

ChemicalStructure/PW C001391
type: ChemicalStructure
structure format: SMILES
structure data: OC(CC(=O)C(O)=O)C(O)=O

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

Pyrroline hydroxycarboxylic acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Pyrroline hydroxycarboxylic acid

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

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

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

Glycine amidinotransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P50440
organism: Homo sapiens

Reference/ChEBI CHEBI 17287
type: UnificationXref
id: CHEBI:17287
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

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

ChemicalStructure/PW C000032
type: ChemicalStructure
structure format: SMILES

Creatine kinase B-type
type: ProteinReference
xref: Reference/UniProt P12277
organism: Homo sapiens

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

ChemicalStructure/PW C000036
type: ChemicalStructure
structure format: SMILES

Reference/HMDB HMDB0001369
type: UnificationXref
id: HMDB0001369
db: HMDB

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

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

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

Reference/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

Protein/683 Cytoplasm/Stoichiometry/2.0
type: Stoichiometry
physical entity: Creatine kinase B-type
stoichiometric coefficient: 2.0

TransportCatalysis5434
type: Catalysis
controller: Proton-coupled amino acid transporter 1
controlled: Transport14622
control type: ACTIVATION

Reference/ChEBI CHEBI 17053
type: UnificationXref
id: CHEBI:17053
db: ChEBI

Reference/CAS 56-40-6
type: UnificationXref
id: 56-40-6
db: CAS

D-Proline
type: SmallMolecule
entity reference: D-Proline

Nitric oxide
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0003378
xref: Reference/CAS 10102-43-9
xref: Reference/PubChem-compound 145068
xref: Reference/ChEBI CHEBI 16480
xref: Reference/ChemSpider 127983
xref: Reference/KEGG Compound C00533
xref: Reference/BioCyc NITRIC-OXIDE
chemical formula: NO
molecular weight: 29.997988
structure: ChemicalStructure/PW C001867

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

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

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

ChemicalStructure/PW C000046
type: ChemicalStructure
structure format: SMILES
structure data: CN(CC(O)=O)C(N)=N

Reference/CAS 53054-07-2
type: UnificationXref
id: 53054-07-2
db: CAS

Compound/PW C000396 Cell Membrane/Stoichiometry/2.0
type: Stoichiometry
physical entity: L-Arginine
stoichiometric coefficient: 2.0

L-Proline
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 17203
xref: Reference/ChemSpider 128566
xref: Reference/BioCyc PRO
xref: Reference/CAS 147-85-3
xref: Reference/KEGG Compound C00148
xref: Reference/HMDB HMDB0000162
xref: Reference/PubChem-compound 145742
chemical formula: C5H9NO2
molecular weight: 115.06333
structure: ChemicalStructure/PW C000106

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

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

Reference/ChemSpider 730
type: UnificationXref
id: 730
db: ChemSpider

Reference/HMDB HMDB0002234
type: UnificationXref
id: HMDB0002234
db: HMDB

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

4-Hydroxy-2-oxoglutaric acid
type: SmallMolecule
cellular location: Cytosol
entity reference: 4-Hydroxy-2-oxoglutaric acid

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 HMDB0000052
type: UnificationXref
id: HMDB0000052
db: HMDB

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

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

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

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

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

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

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

Pyrroline-5-carboxylate reductase 2
type: Protein
cellular location: Cytoplasm
entity reference: Pyrroline-5-carboxylate reductase 2

Nitric oxide synthase, brain
type: ProteinReference
xref: Reference/UniProt P29475
organism: Homo sapiens

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

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

Reference/HMDB HMDB0000064
type: UnificationXref
id: HMDB0000064
db: HMDB

Reference/BioCyc L-GLUTAMATE GAMMA-SEMIALDEHYDE
type: UnificationXref
id: L-GLUTAMATE_GAMMA-SEMIALDEHYDE
db: BioCyc

Proton-coupled amino acid transporter 1
type: Protein
entity reference: Proton-coupled amino acid transporter 1

Reference/CAS 57-00-1
type: UnificationXref
id: 57-00-1
db: CAS

Compound/PW C000106 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Proline
stoichiometric coefficient: 1.0

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

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

L-Glutamic γ-semialdehyde
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0002104
xref: Reference/PubChem-compound 193305
xref: Reference/CAS 496-92-4
xref: Reference/ChemSpider 167744
xref: Reference/KEGG Compound C01165
xref: Reference/BioCyc L-GLUTAMATE GAMMA-SEMIALDEHYDE
xref: Reference/ChEBI CHEBI 17232
chemical formula: C5H9NO3
molecular weight: 131.05824
structure: ChemicalStructure/PW C001415

Reference/PubChem-compound 25246222
type: UnificationXref
id: 25246222
db: PubChem-compound

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

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

ChemicalStructure/PW C001164
type: ChemicalStructure
structure format: SMILES
structure data: CN(CC(O)=O)C(=N)NP(O)(O)=O

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

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

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

ChemicalStructure/PW C000078
type: ChemicalStructure
structure format: SMILES
structure data: NCC(O)=O

Reference/UniProt P12277
type: UnificationXref
id: P12277
db: UniProt

Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/151 Mitochondrion/Stoichiometry/2.0
cellular location: Mitochondrion
component: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial

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

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

Guanidinoacetate N-methyltransferase
type: ProteinReference
xref: Reference/UniProt Q14353
organism: Homo sapiens

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

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

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

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

L-Proline
type: SmallMolecule
entity reference: L-Proline

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

Reference/ChemSpider 743
type: UnificationXref
id: 743
db: ChemSpider

Compound/PW C000146 Cell Membrane/Stoichiometry/3.0
type: Stoichiometry
physical entity: NADPH
stoichiometric coefficient: 3.0

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

ChemicalStructure/PW C000083
type: ChemicalStructure
structure format: SMILES
structure data: NC(=N)NCC(O)=O

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

ReactionCatalysis153
type: Catalysis
controller: Creatine kinase B-type
controlled: 2.7.3.2
control type: ACTIVATION

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

ReactionCatalysis151
type: Catalysis
controller: Glycine amidinotransferase, mitochondrial
controlled: 2.1.4.1
control type: ACTIVATION

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

ReactionCatalysis152
type: Catalysis
controller: Guanidinoacetate N-methyltransferase
controlled: 2.1.1.2
control type: ACTIVATION

ChemicalStructure/PW C001415
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CCC=O)C(O)=O

ReactionCatalysis150
type: Catalysis
controller: Probable arginine--tRNA ligase, mitochondrial
controlled: 6.1.1.19
control type: ACTIVATION

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

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

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

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

Reference/ChEBI CHEBI 43088
type: UnificationXref
id: CHEBI:43088
db: ChEBI

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

Proton-coupled amino acid transporter 1
type: ProteinReference
xref: Reference/UniProt Q7Z2H8

D-Arginine and D-Ornithine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway118671
pathway order: SubPathwayInteractionSteps/SubPathway118670
pathway component: SubPathwayInteraction118671
pathway component: SubPathwayInteraction118670
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

ReactionCatalysis146
type: Catalysis
controller: Pyrroline-5-carboxylate reductase 2
controlled: 1.5.1.2
control type: ACTIVATION

ReactionCatalysis147
type: Catalysis
controller: Proline dehydrogenase 1, mitochondrial
controlled: 1.5.5.2
control type: ACTIVATION

Reaction649
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001003 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001415 Mitochondrial Matrix/Stoichiometry/1.0
right: 1-Pyrroline-5-carboxylic acid
spontaneous: true
left: L-Glutamic γ-semialdehyde
name: L-Glutamic γ-semialdehyde ↔ 1-Pyrroline-5-carboxylic acid
conversion direction: REVERSIBLE

ReactionCatalysis145
type: Catalysis
controller: Prolyl 4-hydroxylase
controlled: 1.14.11.2
control type: ACTIVATION

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

ReactionCatalysis143
type: Catalysis
controller: Bifunctional glutamate/proline--tRNA ligase
controlled: 6.1.1.17
control type: ACTIVATION

ReactionCatalysis140
type: Catalysis
controller: Proline dehydrogenase 1, mitochondrial
controlled: 1.5.5.2
control type: ACTIVATION

ReactionCatalysis141
type: Catalysis
controller: Pyrroline-5-carboxylate reductase 2
controlled: 1.5.1.2
control type: ACTIVATION

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

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

Reference/HMDB HMDB0001511
type: UnificationXref
id: HMDB0001511
db: HMDB

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

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

ReactionCatalysis139
type: Catalysis
controller: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
controlled: 1.2.1.88
control type: ACTIVATION

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

ReactionCatalysis138
type: Catalysis
controller: Ornithine aminotransferase, mitochondrial
controlled: 2.6.1.13
control type: ACTIVATION

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

Compound/PW C001065 Cell Membrane/Stoichiometry/4.0
type: Stoichiometry
physical entity: Oxygen
stoichiometric coefficient: 4.0

Compound/PW C000106 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Proline
stoichiometric coefficient: 1.0

Compound/PW C001878 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: D-Proline
stoichiometric coefficient: 1.0

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

Reaction658
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002808 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001057 Mitochondrion/Stoichiometry/1.0
right: L-4-Hydroxyglutamate semialdehyde
spontaneous: true
left: Pyrroline hydroxycarboxylic acid
name: Pyrroline hydroxycarboxylic acid ↔ L-4-Hydroxyglutamate semialdehyde
conversion direction: REVERSIBLE

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

2.6.1.13
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000140 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001415 Mitochondrial Matrix/Stoichiometry/1.0
right: L-Glutamic γ-semialdehyde
right: L-Glutamic acid
spontaneous: false
EC number: 2.6.1.13
left: Oxoglutaric acid
left: Ornithine
name: Ornithine + Oxoglutaric acid ↔ L-Glutamic acid + L-Glutamic γ-semialdehyde
conversion direction: REVERSIBLE

1.5.5.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002643 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001057 Mitochondrial Matrix/Stoichiometry/1.0
right: Pyrroline hydroxycarboxylic acid
spontaneous: false
EC number: 1.5.5.2
left: 4-Hydroxyproline
name: 4-Hydroxyproline ↔ Pyrroline hydroxycarboxylic acid
conversion direction: REVERSIBLE

Reference/PubChem-compound 440849
type: UnificationXref
id: 440849
db: PubChem-compound

ChemicalStructure/PW C001878
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)[C@H]1CCCN1

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

Reference/ChEBI CHEBI 27809
type: UnificationXref
id: CHEBI:27809
db: ChEBI

Reference/ChEBI CHEBI 36761
type: UnificationXref
id: CHEBI:36761
db: ChEBI

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

Alanine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway118669
pathway component: SubPathwayInteraction118669
xref: Reference/PathWhiz PW000001
xref: Reference/SMPDB SMP0000055
name: SubPathway

Reference/ChEBI CHEBI 18095
type: UnificationXref
id: CHEBI:18095
db: ChEBI

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

1.5.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002643 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001057 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Cytoplasm/Stoichiometry/1.0
right: NADH
right: Pyrroline hydroxycarboxylic acid
spontaneous: false
EC number: 1.5.1.2
left: 4-Hydroxyproline
left: NAD
name: 4-Hydroxyproline + NAD → NADH + Pyrroline hydroxycarboxylic acid
conversion direction: LEFT_TO_RIGHT

6.1.1.17
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000106 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: RNA/11 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: RNA/12 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 C000032 Cytoplasm/Stoichiometry/1.0
right: Adenosine monophosphate
right: L-Prolyl-tRNA(Pro)
right: Pyrophosphate
spontaneous: false
EC number: 6.1.1.17
left: Adenosine triphosphate
left: tRNA(Pro)
left: L-Proline
name: Adenosine triphosphate + L-Proline + tRNA(Pro) → Adenosine monophosphate + L-Prolyl-tRNA(Pro) + Pyrophosphate
conversion direction: LEFT_TO_RIGHT

1.5.5.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000106 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001003 Mitochondrial Matrix/Stoichiometry/1.0
right: L-Proline
spontaneous: false
EC number: 1.5.5.2
left: 1-Pyrroline-5-carboxylic acid
name: 1-Pyrroline-5-carboxylic acid ↔ L-Proline
conversion direction: REVERSIBLE

1.5.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000106 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001003 Cytoplasm/Stoichiometry/1.0
right: 1-Pyrroline-5-carboxylic acid
right: NADH
spontaneous: false
EC number: 1.5.1.2
left: L-Proline
left: NAD
name: L-Proline + NAD → 1-Pyrroline-5-carboxylic acid + NADH
conversion direction: LEFT_TO_RIGHT

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

Reference/BioCyc L-ERYTHRO-4-HYDROXY-GLUTAMATE
type: UnificationXref
id: L-ERYTHRO-4-HYDROXY-GLUTAMATE
db: BioCyc

Compound/PW C001867 Cell Membrane/Stoichiometry/2.0
type: Stoichiometry
physical entity: Nitric oxide
stoichiometric coefficient: 2.0

Citric Acid Cycle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway118668
pathway component: SubPathwayInteraction118668
xref: Reference/PathWhiz PW000005
xref: Reference/SMPDB SMP0000057
name: SubPathway

Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial

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

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

Reference/BioCyc ADENOSYL-HOMO-CYS
type: UnificationXref
id: ADENOSYL-HOMO-CYS
db: BioCyc

ReactionCatalysis41
type: Catalysis
controller: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
controlled: 1.2.1.88
control type: ACTIVATION

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

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

ChemicalStructure/PW C002984
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)C1=NCCC1

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

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

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

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

6.1.1.19
type: BiochemicalReaction
participant stoichiometry: RNA/13 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: RNA/14 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000032 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000170 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000396 Mitochondrial Matrix/Stoichiometry/1.0
right: Adenosine monophosphate
right: Pyrophosphate
right: L-Arginyl-tRNA(Arg)
spontaneous: false
EC number: 6.1.1.19
left: L-Arginine
left: tRNA(Arg)
left: Adenosine triphosphate
name: Adenosine triphosphate + L-Arginine + tRNA(Arg) → Adenosine monophosphate + L-Arginyl-tRNA(Arg) + Pyrophosphate
conversion direction: LEFT_TO_RIGHT

Reference/ChEBI CHEBI 16919
type: UnificationXref
id: CHEBI:16919
db: ChEBI

Reference/UniProt O43272
type: UnificationXref
id: O43272
db: UniProt

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

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

Reference/CAS 1187-99-1
type: UnificationXref
id: 1187-99-1
db: CAS

Reference/PubChem-compound 5810
type: UnificationXref
id: 5810
db: PubChem-compound

Reference/ChemSpider 579
type: UnificationXref
id: 579
db: ChemSpider

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

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

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

Reference/GENE ONTOLOGY GO 0005886
type: UnificationXref
id: GO:0005886
db: GENE ONTOLOGY

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

Reference/ChEBI CHEBI 15414
type: UnificationXref
id: CHEBI:15414
db: ChEBI

NADP
type: SmallMolecule
cellular location: Cell Membrane
entity reference: NADP

Reference/KEGG Compound C03079
type: UnificationXref
id: C03079
db: KEGG Compound

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

Reference/ChemSpider 567
type: UnificationXref
id: 567
db: ChemSpider

Reference/PubChem-compound 440851
type: UnificationXref
id: 440851
db: PubChem-compound

ChemicalStructure/PW C000143
type: ChemicalStructure
structure format: SMILES

Reference/ChemSpider 566
type: UnificationXref
id: 566
db: ChemSpider

Compound/PW C001512 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: 1-Pyrroline-4-hydroxy-2-carboxylate
stoichiometric coefficient: 1.0

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

Reference/PubChem-compound 5257127
type: UnificationXref
id: 5257127
db: PubChem-compound

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

Pyrroline hydroxycarboxylic acid
type: SmallMoleculeReference
xref: Reference/KEGG Compound C04281
xref: Reference/HMDB HMDB0001369
xref: Reference/BioCyc PYRROLINE-HYDROXY-CARBOXYLATE
xref: Reference/CAS 22573-88-2
xref: Reference/ChEBI CHEBI 27391
xref: Reference/ChemSpider 1030
xref: Reference/PubChem-compound 1059
chemical formula: C5H7NO3
molecular weight: 129.04259
structure: ChemicalStructure/PW C001057

ChemicalStructure/PW C000146
type: ChemicalStructure
structure format: SMILES

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

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

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

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

ChemicalStructure/PW C000106
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)[C@@H]1CCCN1

Reference/BioCyc S-ADENOSYLMETHIONINE
type: UnificationXref
id: S-ADENOSYLMETHIONINE
db: BioCyc

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

Hydrogen Ion
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Hydrogen Ion

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

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

Succinic acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000254
xref: Reference/CAS 110-15-6
xref: Reference/ChEBI CHEBI 15741
xref: Reference/KEGG Compound C00042
xref: Reference/ChemSpider 1078
xref: Reference/PubChem-compound 1110
xref: Reference/BioCyc SUC
chemical formula: C4H6O4
molecular weight: 118.02661
structure: ChemicalStructure/PW C000174

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

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

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

Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/151 Mitochondrial Matrix/Stoichiometry/2.0
cellular location: Mitochondrial Matrix
component: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial

Compound/PW C002984 Lysosome/Stoichiometry/1.0
type: Stoichiometry
physical entity: 1-Pyrroline-2-carboxylic acid
stoichiometric coefficient: 1.0

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

Protein/299 Cytoplasm/Stoichiometry/10.0
type: Stoichiometry
physical entity: Pyrroline-5-carboxylate reductase 2
stoichiometric coefficient: 10.0

Compound/PW C000143 Cell Membrane/Stoichiometry/3.0
type: Stoichiometry
physical entity: NADP
stoichiometric coefficient: 3.0

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

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

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

4-Hydroxyproline
type: SmallMolecule
cellular location: Lysosome
entity reference: 4-Hydroxyproline

Proline dehydrogenase 1, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Proline dehydrogenase 1, mitochondrial

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

1-Pyrroline-4-hydroxy-2-carboxylate
type: SmallMolecule
cellular location: Lysosome
entity reference: 1-Pyrroline-4-hydroxy-2-carboxylate

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

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

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

Probable arginine--tRNA ligase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Probable arginine--tRNA ligase, mitochondrial

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

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

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/PubChem-compound 8988
type: UnificationXref
id: 8988
db: PubChem-compound

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

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

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

Reference/BioCyc PYRROLINE-HYDROXY-CARBOXYLATE
type: UnificationXref
id: PYRROLINE-HYDROXY-CARBOXYLATE
db: BioCyc

Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial

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

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

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

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

Reference/ChEBI CHEBI 15428
type: UnificationXref
id: CHEBI:15428
db: ChEBI

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

Reference/ChEBI CHEBI 16338
type: UnificationXref
id: CHEBI:16338
db: ChEBI

Reference/PubChem-compound 145068
type: UnificationXref
id: 145068
db: PubChem-compound

Reference/PubChem-compound 1059
type: UnificationXref
id: 1059
db: PubChem-compound

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

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

1.14.11.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000106 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000174 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002643 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Cytoplasm/Stoichiometry/1.0
right: 4-Hydroxyproline
right: Carbon dioxide
right: Succinic acid
spontaneous: false
EC number: 1.14.11.2
left: Oxygen
left: Oxoglutaric acid
left: L-Proline
name: L-Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Carbon dioxide + Succinic acid
conversion direction: LEFT_TO_RIGHT

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

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

Reference/ChEBI CHEBI 16344
type: UnificationXref
id: CHEBI:16344
db: ChEBI

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

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

L-Prolyl-tRNA(Pro)
type: Rna
cellular location: Cytoplasm
entity reference: L-Prolyl-tRNA(Pro)

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

ChemicalStructure/PW C001867
type: ChemicalStructure
structure format: SMILES
structure data: [N]=O

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

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/BioCyc L-4-HYDROXY-PROLINE
type: UnificationXref
id: L-4-HYDROXY-PROLINE
db: BioCyc

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

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

Compound/PW C001878 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: D-Proline
stoichiometric coefficient: 1.0

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

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

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

Reference/ChEBI CHEBI 16313
type: UnificationXref
id: CHEBI:16313
db: ChEBI

D-Proline
type: SmallMolecule
cellular location: Cytosol
entity reference: D-Proline

ChemicalStructure/PW C000749
type: ChemicalStructure
structure format: SMILES

Reference/ChemSpider 4573749
type: UnificationXref
id: 4573749
db: ChemSpider

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

Compound/PW C001535 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Hydroxy-L-glutamic acid
stoichiometric coefficient: 1.0

Reference/PubChem-compound 3946848
type: UnificationXref
id: 3946848
db: PubChem-compound

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

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

Reference/ChemSpider 8640
type: UnificationXref
id: 8640
db: ChemSpider

Reference/PubChem-compound 24762165
type: UnificationXref
id: 24762165
db: PubChem-compound

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

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

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

S-Adenosylmethionine
type: SmallMolecule
cellular location: Cytoplasm
entity reference: S-Adenosylmethionine

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