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

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

Acetyl-CoA
type: SmallMoleculeReference
xref: Reference/BioCyc ACETYL-COA
xref: Reference/PubChem-compound 444493
xref: Reference/HMDB HMDB0001206
xref: Reference/ChemSpider 392413
xref: Reference/CAS 72-89-9
xref: Reference/ChEBI CHEBI 15351
xref: Reference/KEGG Compound C00024
chemical formula: C23H38N7O17P3S
molecular weight: 809.1258
structure: ChemicalStructure/PW C000940

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

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

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

Compound/PW C000040 Extracellular Space/Stoichiometry/1.0
type: Stoichiometry
physical entity: β-Alanine
stoichiometric coefficient: 1.0

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

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

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

Beta-ureidopropionase
type: Protein
cellular location: Cytoplasm
entity reference: Beta-ureidopropionase

Reference/ChEBI CHEBI 18323
type: UnificationXref
id: CHEBI:18323
db: ChEBI

Reference/ChemSpider 388363
type: UnificationXref
id: 388363
db: ChemSpider

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

Hydrogen peroxide
type: SmallMolecule
cellular location: Cell Membrane
entity reference: Hydrogen peroxide

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

Reference/ChEBI CHEBI 17568
type: UnificationXref
id: CHEBI:17568
db: ChEBI

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

Reference/ChemSpider 100482
type: UnificationXref
id: 100482
db: ChemSpider

Compound/PW C000364 Extracellular Space/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Methylhistidine
stoichiometric coefficient: 1.0

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

Compound/PW C000018 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Ureidopropionic acid
stoichiometric coefficient: 1.0

Reference/ChemSpider 1141
type: UnificationXref
id: 1141
db: ChemSpider

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

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

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

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

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

Reference/ChemSpider 74
type: UnificationXref
id: 74
db: ChemSpider

Reference/PubChem-compound 64969
type: UnificationXref
id: 64969
db: PubChem-compound

Reference/PubChem-compound 6274
type: UnificationXref
id: 6274
db: PubChem-compound

Reference/KEGG Compound C00106
type: UnificationXref
id: C00106
db: KEGG Compound

Reference/PathWhiz PW000032
type: UnificationXref
id: PW000032
db: PathWhiz

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

Reference/KEGG Compound C02642
type: UnificationXref
id: C02642
db: KEGG Compound

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

Reference/ChemSpider 58494
type: UnificationXref
id: 58494
db: ChemSpider

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

Reference/PathWhiz PW121955
type: UnificationXref
id: PW121955
db: PathWhiz

Reference/KEGG Compound C00222
type: UnificationXref
id: C00222
db: KEGG Compound

ChemicalStructure/PW C001099
type: ChemicalStructure
structure format: SMILES

Rattus norvegicus
type: BioSource
xref: Reference/TAXONOMY 10116

Compound/PW C000053 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dihydrouracil
stoichiometric coefficient: 1.0

3-Aminopropionaldehyde
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 18090
xref: Reference/HMDB HMDB0001106
xref: Reference/BioCyc 3-AMINO-PROPANAL
xref: Reference/CAS 352-92-1
xref: Reference/PubChem-compound 75
xref: Reference/ChemSpider 74
xref: Reference/KEGG Compound C05665
chemical formula: C3H7NO
molecular weight: 73.052765
structure: ChemicalStructure/PW C000866

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

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

Reference/BioCyc 3-AMINO-PROPANAL
type: UnificationXref
id: 3-AMINO-PROPANAL
db: BioCyc

Reference/UniProt P16453
type: UnificationXref
id: P16453
db: UniProt

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

4-aminobutyrate aminotransferase, mitochondrial
type: Complex
component stoichiometry: Protein/14638 Mitochondrial Matrix/Stoichiometry/2.0
cellular location: Mitochondrial Matrix
component: 4-aminobutyrate aminotransferase, mitochondrial

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

Reference/KEGG Compound C00006
type: UnificationXref
id: C00006
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

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

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

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

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

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

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

3-Aminopropionaldehyde
type: SmallMolecule
cellular location: Cell Membrane
entity reference: 3-Aminopropionaldehyde

Coenzyme A
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Coenzyme A

Malonic semialdehyde
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Malonic semialdehyde
name: SubPathwayInput

β-Alanine
type: SmallMolecule
cellular location: Extracellular Space
entity reference: β-Alanine
name: SubPathwayOutput

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: C21H28N7O14P2
molecular weight: 664.11694
structure: ChemicalStructure/PW C000721

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

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

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

Reference/KEGG Compound C00010
type: UnificationXref
id: C00010
db: KEGG Compound

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

Reference/KEGG Compound C00135
type: UnificationXref
id: C00135
db: KEGG Compound

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

Protein/15346 Cytoplasm/Stoichiometry/4.0
type: Stoichiometry
physical entity: Dihydropyrimidinase
stoichiometric coefficient: 4.0

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

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

Compound/PW C000866 Cell Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Aminopropionaldehyde
stoichiometric coefficient: 1.0

Histidine decarboxylase
type: Protein
cellular location: Cytoplasm
entity reference: Histidine decarboxylase

Protein/15336 Extracellular Space/Stoichiometry/2.0
type: Stoichiometry
physical entity: Beta-Ala-His dipeptidase
stoichiometric coefficient: 2.0

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

4-aminobutyrate aminotransferase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: 4-aminobutyrate aminotransferase, mitochondrial

Reference/KEGG Compound C00386
type: UnificationXref
id: C00386
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/KEGG Compound C00024
type: UnificationXref
id: C00024
db: KEGG Compound

Dihydropyrimidinase
type: Protein
cellular location: Cytoplasm
entity reference: Dihydropyrimidinase

Anserine
type: SmallMolecule
cellular location: Extracellular Space
entity reference: Anserine

Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
type: Complex
component stoichiometry: Protein/15355 Mitochondrial Matrix/Stoichiometry/4.0
cellular location: Mitochondrial Matrix
component: Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial

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

Protein/15360 Mitochondrial Matrix/Stoichiometry/4.0
type: Stoichiometry
physical entity: Aldehyde dehydrogenase, mitochondrial
stoichiometric coefficient: 4.0

Reference/UniProt Q5R1T5
type: UnificationXref
id: Q5R1T5
db: UniProt

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

Reference/CAS 462-88-4
type: UnificationXref
id: 462-88-4
db: CAS

Dihydropyrimidinase
type: Complex
component stoichiometry: Protein/15346 Cytoplasm/Stoichiometry/4.0
cellular location: Cytoplasm
component: Dihydropyrimidinase

Reference/CAS 79-83-4
type: UnificationXref
id: 79-83-4
db: CAS

Reference/CAS 504-07-4
type: UnificationXref
id: 504-07-4
db: CAS

Reference/ChemSpider 415
type: UnificationXref
id: 415
db: ChemSpider

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

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C000053
type: ChemicalStructure
structure format: SMILES
structure data: O=C1CCNC(=O)N1

Reference/HMDB HMDB0001106
type: UnificationXref
id: HMDB0001106
db: HMDB

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

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

Reference/PubChem-compound 649
type: UnificationXref
id: 649
db: PubChem-compound

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

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

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

GABA-Transaminase Deficiency
type: Pathway
pathway order: PathwaySubPathway/108473
pathway order: PathwaySubPathway/108474
pathway order: PathwayTransport/2113911
pathway order: PathwaySubPathway/108470
pathway order: PathwaySubPathway/108471
pathway order: PathwaySubPathway/108472
pathway order: PathwayEnzymeReaction/2113901
pathway order: PathwayEnzymeReaction/2113910
pathway order: PathwayEnzymeReaction/2113904
pathway order: PathwayEnzymeReaction/2113905
pathway order: PathwayEnzymeReaction/2113902
pathway order: PathwayEnzymeReaction/2113903
pathway order: PathwayEnzymeReaction/2113908
pathway order: PathwayEnzymeReaction/2113909
pathway order: PathwayEnzymeReaction/2113906
pathway order: PathwayEnzymeReaction/2113907
pathway component: Transport7994
pathway component: ReactionCatalysis137317
pathway component: ReactionCatalysis137318
pathway component: ReactionCatalysis137319
pathway component: 4.1.1.15
pathway component: Reaction147008
pathway component: ReactionCatalysis137172
pathway component: ReactionCatalysis137173
pathway component: Propanoate Metabolism
pathway component: ReactionCatalysis137323
pathway component: ReactionCatalysis137324
pathway component: ReactionCatalysis137320
pathway component: ReactionCatalysis137321
pathway component: ReactionCatalysis137322
pathway component: Aspartate Metabolism
pathway component: Intestinal Microflora
pathway component: 1.4.3.21
pathway component: 1.2.1.3
pathway component: Reaction147116
pathway component: 1.3.1.2
pathway component: 3.5.2.2
pathway component: 3.5.1.6
pathway component: 3.4.13.20
pathway component: Pantothenate and CoA Biosynthesis
pathway component: Pyrimidine Metabolism
xref: Reference/SMPDB SMP0120698
xref: Reference/PathWhiz PW121955
organism: Rattus norvegicus

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

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

Reference/HMDB HMDB0000026
type: UnificationXref
id: HMDB0000026
db: HMDB

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

SubPathwayInteraction108470
type: Interaction
name: SubPathwayReaction
name: SubPathway108470Reaction
participant: Pantothenic acid
participant: β-Alanine

SubPathwayInteraction108471
type: Interaction
name: SubPathway108471Reaction
name: SubPathwayReaction
participant: Pantothenic acid

Reference/KEGG Compound C01262
type: UnificationXref
id: C01262
db: KEGG Compound

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

SubPathwayInteraction108472
type: Interaction
name: SubPathwayReaction
name: SubPathway108472Reaction
participant: L-Aspartic acid

SubPathwayInteraction108473
type: Interaction
name: SubPathway108473Reaction
name: SubPathwayReaction
participant: Uracil

SubPathwayInteraction108474
type: Interaction
name: SubPathway108474Reaction
name: SubPathwayReaction
participant: Malonic semialdehyde

Carnosine
type: SmallMolecule
cellular location: Extracellular Space
entity reference: Carnosine

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

Reference/PubChem-compound 428
type: UnificationXref
id: 428
db: PubChem-compound

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

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

Protein/15355 Mitochondrial Matrix/Stoichiometry/4.0
type: Stoichiometry
physical entity: Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
stoichiometric coefficient: 4.0

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

Glutamate decarboxylase 1
type: Complex
component stoichiometry: Protein/14637 Cytoplasm/Stoichiometry/2.0
cellular location: Cytoplasm
component: Histidine decarboxylase

Reference/KEGG Compound C01152
type: UnificationXref
id: C01152
db: KEGG Compound

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

Reference/CAS 926-61-4
type: UnificationXref
id: 926-61-4
db: CAS

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/UniProt P50554
type: UnificationXref
id: P50554
db: UniProt

Compound/PW C001099 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Coenzyme A
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000033
type: UnificationXref
id: HMDB0000033
db: HMDB

Beta-Ala-His dipeptidase
type: Complex
component stoichiometry: Protein/15336 Extracellular Space/Stoichiometry/2.0
cellular location: Extracellular Space
component: Beta-Ala-His dipeptidase

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

Reference/ChEBI CHEBI 18261
type: UnificationXref
id: CHEBI:18261
db: ChEBI

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

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

Compound/PW C000023 Extracellular Space/Stoichiometry/1.0
type: Stoichiometry
physical entity: Carnosine
stoichiometric coefficient: 1.0

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

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

ChemicalStructure/PW C000040
type: ChemicalStructure
structure format: SMILES
structure data: NCCC(O)=O

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

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

Reference/HMDB HMDB0000056
type: UnificationXref
id: HMDB0000056
db: HMDB

Reference/HMDB HMDB0000177
type: UnificationXref
id: HMDB0000177
db: HMDB

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

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

L-Histidine
type: SmallMolecule
cellular location: Extracellular Space
entity reference: L-Histidine

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

Compound/PW C000126 Extracellular Space/Stoichiometry/1.0
type: Stoichiometry
physical entity: Anserine
stoichiometric coefficient: 1.0

ChemicalStructure/PW C006753
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CC=O

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

Reference/CAS 352-92-1
type: UnificationXref
id: 352-92-1
db: CAS

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

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

3-Methylhistidine
type: SmallMolecule
cellular location: Extracellular Space
entity reference: 3-Methylhistidine

L-Histidine
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15971
xref: Reference/HMDB HMDB0000177
xref: Reference/PubChem-compound 6274
xref: Reference/ChemSpider 6038
xref: Reference/CAS 71-00-1
xref: Reference/BioCyc HIS
xref: Reference/KEGG Compound C00135
chemical formula: C6H9N3O2
molecular weight: 155.06947
structure: ChemicalStructure/PW C000116

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

Reference/ChemSpider 845
type: UnificationXref
id: 845
db: ChemSpider

Reference/ChemSpider 963
type: UnificationXref
id: 963
db: ChemSpider

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

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

1,3-Diaminopropane
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000002
xref: Reference/KEGG Compound C00986
xref: Reference/BioCyc CPD-313
xref: Reference/PubChem-compound 428
xref: Reference/CAS 109-76-2
xref: Reference/ChEBI CHEBI 15725
xref: Reference/ChemSpider 415
chemical formula: C3H10N2
molecular weight: 74.0844
structure: ChemicalStructure/PW C000002

3-Methylhistidine
type: SmallMoleculeReference
xref: Reference/CAS 368-16-1
xref: Reference/HMDB HMDB0000479
xref: Reference/KEGG Compound C01152
xref: Reference/ChemSpider 58494
xref: Reference/PubChem-compound 64969
xref: Reference/ChEBI CHEBI 27596
chemical formula: C7H11N3O2
molecular weight: 169.08513
structure: ChemicalStructure/PW C000364

Malonic semialdehyde
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Malonic semialdehyde

Reference/PubChem-compound 444493
type: UnificationXref
id: 444493
db: PubChem-compound

Reference/PubChem-compound 988
type: UnificationXref
id: 988
db: PubChem-compound

Uracil
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 17568
xref: Reference/KEGG Compound C00106
xref: Reference/HMDB HMDB0000300
xref: Reference/CAS 66-22-8
xref: Reference/BioCyc URACIL
xref: Reference/PubChem-compound 1174
xref: Reference/ChemSpider 1141
chemical formula: C4H4N2O2
molecular weight: 112.027275
structure: ChemicalStructure/PW C000204

Reference/PubChem-compound 868
type: UnificationXref
id: 868
db: PubChem-compound

Reference/KEGG Compound C00099
type: UnificationXref
id: C00099
db: KEGG Compound

Reference/KEGG Compound C05665
type: UnificationXref
id: C05665
db: KEGG Compound

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

Compound/PW C000116 Extracellular Space/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Histidine
stoichiometric coefficient: 1.0

Reference/BioCyc ACETYL-COA
type: UnificationXref
id: ACETYL-COA
db: BioCyc

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

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

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

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

Reference/HMDB HMDB0000194
type: UnificationXref
id: HMDB0000194
db: HMDB

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

Reference/HMDB HMDB0000076
type: UnificationXref
id: HMDB0000076
db: HMDB

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

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

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

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

Reference/ChemSpider 629
type: UnificationXref
id: 629
db: ChemSpider

Acetyl-CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Acetyl-CoA

Reference/ChEBI CHEBI 15901
type: UnificationXref
id: CHEBI:15901
db: ChEBI

Beta-Ala-His dipeptidase
type: ProteinReference
xref: Reference/UniProt Q66HG3
organism: Rattus norvegicus

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

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

Reference/CAS 66-22-8
type: UnificationXref
id: 66-22-8
db: CAS

Protein/15366 Cell Membrane/Stoichiometry/2.0
type: Stoichiometry
physical entity: Membrane primary amine oxidase
stoichiometric coefficient: 2.0

ChemicalStructure/PW C000204
type: ChemicalStructure
structure format: SMILES
structure data: O=C1NC=CC(=O)N1

Reference/UniProt Q02253
type: UnificationXref
id: Q02253
db: UniProt

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

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

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

Reference/ChEBI CHEBI 15971
type: UnificationXref
id: CHEBI:15971
db: ChEBI

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

Dihydropyrimidinase
type: ProteinReference
xref: Reference/UniProt Q642F0
organism: Rattus norvegicus

Reference/UniProt O89000
type: UnificationXref
id: O89000
db: UniProt

Reference/PubChem-compound 239
type: UnificationXref
id: 239
db: PubChem-compound

Beta-ureidopropionase
type: ProteinReference
xref: Reference/UniProt Q03248
organism: Rattus norvegicus

Reference/PubChem-compound 111
type: UnificationXref
id: 111
db: PubChem-compound

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

Compound/PW C000040 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: β-Alanine
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000300
type: UnificationXref
id: HMDB0000300
db: HMDB

Reference/PubChem-compound 6816
type: UnificationXref
id: 6816
db: PubChem-compound

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

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

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

Reference/ChEBI CHEBI 15725
type: UnificationXref
id: CHEBI:15725
db: ChEBI

Reference/ChEBI CHEBI 15727
type: UnificationXref
id: CHEBI:15727
db: ChEBI

β-Alanine
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000056
xref: Reference/ChemSpider 234
xref: Reference/PubChem-compound 239
xref: Reference/ChEBI CHEBI 16958
xref: Reference/KEGG Compound C00099
xref: Reference/CAS 107-95-9
xref: Reference/BioCyc B-ALANINE
chemical formula: C3H7NO2
molecular weight: 89.047676
structure: ChemicalStructure/PW C000040

Pyrimidine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108473
pathway component: SubPathwayInteraction108473
xref: Reference/SMPDB SMP0000046
xref: Reference/PathWhiz PW000160
name: SubPathway

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

Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
type: ProteinReference
xref: Reference/UniProt Q02253
organism: Rattus norvegicus

Anserine
type: SmallMoleculeReference
xref: Reference/CAS 584-85-0
xref: Reference/PubChem-compound 112072
xref: Reference/KEGG Compound C01262
xref: Reference/ChemSpider 100482
xref: Reference/HMDB HMDB0000194
xref: Reference/ChEBI CHEBI 18323
chemical formula: C10H16N4O3
molecular weight: 240.12224
structure: ChemicalStructure/PW C000126

Reference/ChemSpider 6038
type: UnificationXref
id: 6038
db: ChemSpider

Dihydropyrimidine dehydrogenase [NADP(+)]
type: ProteinReference
xref: Reference/UniProt O89000
organism: Rattus norvegicus

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

ReactionCatalysis137317
type: Catalysis
controller: Beta-Ala-His dipeptidase
controlled: 3.4.13.20
control type: ACTIVATION

ReactionCatalysis137318
type: Catalysis
controller: Beta-Ala-His dipeptidase
controlled: 3.4.13.20
control type: ACTIVATION

Reference/UniProt Q03248
type: UnificationXref
id: Q03248
db: UniProt

ReactionCatalysis137319
type: Catalysis
controller: Beta-ureidopropionase
controlled: 3.5.1.6
control type: ACTIVATION

Reference/ChEBI CHEBI 18090
type: UnificationXref
id: CHEBI:18090
db: ChEBI

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

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

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

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

ReactionCatalysis137323
type: Catalysis
controller: Aldehyde dehydrogenase, mitochondrial
controlled: 1.2.1.3
control type: ACTIVATION

ReactionCatalysis137324
type: Catalysis
controller: Membrane primary amine oxidase
controlled: 1.4.3.21
control type: ACTIVATION

Ureidopropionic acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000026
xref: Reference/CAS 462-88-4
xref: Reference/KEGG Compound C02642
xref: Reference/ChEBI CHEBI 18261
xref: Reference/PubChem-compound 111
xref: Reference/ChemSpider 109
xref: Reference/BioCyc 3-UREIDO-PROPIONATE
chemical formula: C4H8N2O3
molecular weight: 132.0535
structure: ChemicalStructure/PW C000018

ReactionCatalysis137320
type: Catalysis
controller: Dihydropyrimidinase
controlled: 3.5.2.2
control type: ACTIVATION

Membrane primary amine oxidase
type: ProteinReference
xref: Reference/UniProt Q5R1T5
organism: Rattus norvegicus

ReactionCatalysis137321
type: Catalysis
controller: Dihydropyrimidine dehydrogenase [NADP(+)]
controlled: 1.3.1.2
control type: ACTIVATION

ReactionCatalysis137322
type: Catalysis
controller: Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
controlled: Reaction147116
control type: ACTIVATION

Reference/CAS 109-76-2
type: UnificationXref
id: 109-76-2
db: CAS

Pantothenic acid
type: SmallMoleculeReference
xref: Reference/ChemSpider 963
xref: Reference/HMDB HMDB0000210
xref: Reference/KEGG Compound C00864
xref: Reference/BioCyc PANTOTHENATE
xref: Reference/PubChem-compound 988
xref: Reference/CAS 79-83-4
xref: Reference/ChEBI CHEBI 7916
chemical formula: C9H17NO5
molecular weight: 219.11067
structure: ChemicalStructure/PW C000136

Reference/CAS 305-84-0
type: UnificationXref
id: 305-84-0
db: CAS

Reference/BioCyc DI-H-URACIL
type: UnificationXref
id: DI-H-URACIL
db: BioCyc

Aldehyde dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q91ZD7
organism: Rattus norvegicus

Dihydropyrimidine dehydrogenase [NADP(+)]
type: Protein
cellular location: Cytoplasm
entity reference: Dihydropyrimidine dehydrogenase [NADP(+)]

Aspartate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108472
pathway component: SubPathwayInteraction108472
xref: Reference/PathWhiz PW000002
xref: Reference/SMPDB SMP0000067
name: SubPathway

Intestinal Microflora
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108470
pathway component: SubPathwayInteraction108470
name: SubPathway

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

Carnosine
type: SmallMoleculeReference
xref: Reference/PubChem-compound 439224
xref: Reference/BioCyc CARNOSINE
xref: Reference/KEGG Compound C00386
xref: Reference/CAS 305-84-0
xref: Reference/ChEBI CHEBI 15727
xref: Reference/HMDB HMDB0000033
xref: Reference/ChemSpider 388363
chemical formula: C9H14N4O3
molecular weight: 226.1066
structure: ChemicalStructure/PW C000023

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: C21H29N7O17P3
molecular weight: 744.08325
structure: ChemicalStructure/PW C000143

Reference/PubChem-compound 75
type: UnificationXref
id: 75
db: PubChem-compound

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

Compound/PW C000002 Cell Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: 1,3-Diaminopropane
stoichiometric coefficient: 1.0

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

Transport7994
type: Transport
participant stoichiometry: Compound/PW C000040 Extracellular Space/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000040 Cytoplasm/Stoichiometry/1.0
right: β-Alanine
spontaneous: false
left: β-Alanine
name: β-Alanine (→) Transport: Rattus norvegicus, Extracellular Space to Rattus norvegicus, Cell, Cytoplasm
conversion direction: LEFT_TO_RIGHT

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

ChemicalStructure/PW C000126
type: ChemicalStructure
structure format: SMILES
structure data: CN1C=NC=C1C[C@H](NC(=O)CCN)C(O)=O

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

Reference/CAS 71-00-1
type: UnificationXref
id: 71-00-1
db: CAS

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

1,3-Diaminopropane
type: SmallMolecule
cellular location: Cell Membrane
entity reference: 1,3-Diaminopropane

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

Reference/HMDB HMDB0000210
type: UnificationXref
id: HMDB0000210
db: HMDB

Reference/HMDB HMDB0001423
type: UnificationXref
id: HMDB0001423
db: HMDB

ChemicalStructure/PW C000136
type: ChemicalStructure
structure format: SMILES
structure data: CC(C)(CO)[C@@H](O)C(=O)NCCC(O)=O

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

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

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

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

Beta-Ala-His dipeptidase
type: Protein
cellular location: Extracellular Space
entity reference: Beta-Ala-His dipeptidase

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

ChemicalStructure/PW C000018
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CCNC(O)=N

Reference/BioCyc 3-UREIDO-PROPIONATE
type: UnificationXref
id: 3-UREIDO-PROPIONATE
db: BioCyc

Reference/HMDB HMDB0011111
type: UnificationXref
id: HMDB0011111
db: HMDB

ChemicalStructure/PW C000143
type: ChemicalStructure
structure format: SMILES

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

ChemicalStructure/PW C000146
type: ChemicalStructure
structure format: SMILES

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

Reference/SMPDB SMP0120698
type: UnificationXref
id: SMP0120698
db: SMPDB

ChemicalStructure/PW C000023
type: ChemicalStructure
structure format: SMILES
structure data: NCCC(=O)N[C@@H](CC1=CN=CN1)C(O)=O

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

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

Reference/CAS 107-95-9
type: UnificationXref
id: 107-95-9
db: CAS

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

Reference/BioCyc CPD-313
type: UnificationXref
id: CPD-313
db: BioCyc

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

Compound/PW C000204 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Uracil
stoichiometric coefficient: 1.0

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

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

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

Reference/ChemSpider 234
type: UnificationXref
id: 234
db: ChemSpider

4.1.1.15
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000040 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000123 Cytoplasm/Stoichiometry/1.0
right: Carbon dioxide
right: β-Alanine
spontaneous: false
EC number: 4.1.1.15
left: L-Aspartic acid
name: L-Aspartic acid → Carbon dioxide + β-Alanine
conversion direction: LEFT_TO_RIGHT

Reference/HMDB HMDB0000479
type: UnificationXref
id: HMDB0000479
db: HMDB

Reaction147008
type: BiochemicalReaction
participant stoichiometry: Compound/PW C006753 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 C000040 Mitochondrial Matrix/Stoichiometry/1.0
right: Malonic semialdehyde
right: L-Glutamic acid
spontaneous: false
left: β-Alanine
left: Oxoglutaric acid
name: Oxoglutaric acid + β-Alanine → L-Glutamic acid + Malonic semialdehyde
conversion direction: LEFT_TO_RIGHT

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

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

Reference/HMDB HMDB0001206
type: UnificationXref
id: HMDB0001206
db: HMDB

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

Propanoate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108474
pathway component: SubPathwayInteraction108474
xref: Reference/PathWhiz PW000149
xref: Reference/SMPDB SMP0000016
name: SubPathway

Dihydrouracil
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00429
xref: Reference/ChEBI CHEBI 15901
xref: Reference/ChemSpider 629
xref: Reference/PubChem-compound 649
xref: Reference/CAS 504-07-4
xref: Reference/BioCyc DI-H-URACIL
xref: Reference/HMDB HMDB0000076
chemical formula: C4H6N2O2
molecular weight: 114.04293
structure: ChemicalStructure/PW C000053

Membrane primary amine oxidase
type: Complex
component stoichiometry: Protein/15366 Cell Membrane/Stoichiometry/2.0
cellular location: Cell Membrane
component: Membrane primary amine oxidase

ChemicalStructure/PW C000116
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CC1=CN=CN1)C(O)=O

Reference/ChemSpider 109
type: UnificationXref
id: 109
db: ChemSpider

Reference/HMDB HMDB0000002
type: UnificationXref
id: HMDB0000002
db: HMDB

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

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

1.4.3.21
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000035 Cell Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000866 Cell Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000002 Cell Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Cell Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cell Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001783 Cell Membrane/Stoichiometry/1.0
right: 3-Aminopropionaldehyde
right: Hydrogen peroxide
right: Ammonia
spontaneous: false
EC number: 1.4.3.21
left: Oxygen
left: Water
left: 1,3-Diaminopropane
name: 1,3-Diaminopropane + Oxygen + Water → 3-Aminopropionaldehyde + Ammonia + Hydrogen peroxide
conversion direction: LEFT_TO_RIGHT

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

Compound/PW C000940 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Acetyl-CoA
stoichiometric coefficient: 1.0

1.2.1.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000866 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 C000040 Mitochondrial Matrix/Stoichiometry/1.0
right: β-Alanine
right: NADH
right: Hydrogen Ion
spontaneous: false
EC number: 1.2.1.3
left: Water
left: 3-Aminopropionaldehyde
left: NAD
name: 3-Aminopropionaldehyde + NAD + Water → Hydrogen Ion + NADH + β-Alanine
conversion direction: LEFT_TO_RIGHT

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

Reaction147116
type: BiochemicalReaction
participant stoichiometry: Compound/PW C006753 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000940 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
right: Acetyl-CoA
right: Carbon dioxide
right: NADH
spontaneous: false
left: Coenzyme A
left: Malonic semialdehyde
left: NAD
name: Coenzyme A + Malonic semialdehyde + NAD → Acetyl-CoA + Carbon dioxide + NADH
conversion direction: LEFT_TO_RIGHT

ChemicalStructure/PW C000002
type: ChemicalStructure
structure format: SMILES
structure data: NCCCN

ChemicalStructure/PW C000364
type: ChemicalStructure
structure format: SMILES
structure data: CN1C=NC=C1C[C@H](N)C(O)=O

1.3.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000146 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000053 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000143 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000204 Cytoplasm/Stoichiometry/1.0
right: Uracil
right: NADPH
spontaneous: false
EC number: 1.3.1.2
left: Dihydrouracil
left: NADP
name: Dihydrouracil + NADP ↔ NADPH + Uracil
conversion direction: REVERSIBLE

Reference/ChEBI CHEBI 16958
type: UnificationXref
id: CHEBI:16958
db: ChEBI

3.5.2.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000053 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000018 Cytoplasm/Stoichiometry/1.0
right: Ureidopropionic acid
spontaneous: false
EC number: 3.5.2.2
left: Water
left: Dihydrouracil
name: Dihydrouracil + Water ↔ Ureidopropionic acid
conversion direction: REVERSIBLE

3.5.1.6
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000035 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000040 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000018 Cytoplasm/Stoichiometry/1.0
right: Carbon dioxide
right: β-Alanine
right: Ammonia
spontaneous: false
EC number: 3.5.1.6
left: Ureidopropionic acid
left: Water
name: Ureidopropionic acid + Water ↔ Ammonia + Carbon dioxide + β-Alanine
conversion direction: REVERSIBLE

Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial

3.4.13.20
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Extracellular Space/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000023 Extracellular Space/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000116 Extracellular Space/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000040 Extracellular Space/Stoichiometry/1.0
right: L-Histidine
right: β-Alanine
spontaneous: false
EC number: 3.4.13.20
left: Water
left: Carnosine
name: Carnosine + Water → L-Histidine + β-Alanine
conversion direction: LEFT_TO_RIGHT

3.4.13.20
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Extracellular Space/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000040 Extracellular Space/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000126 Extracellular Space/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000364 Extracellular Space/Stoichiometry/1.0
right: 3-Methylhistidine
right: β-Alanine
spontaneous: false
EC number: 3.4.13.20
left: Anserine
left: Water
name: Anserine + Water → 3-Methylhistidine + β-Alanine
conversion direction: LEFT_TO_RIGHT

Pantothenate and CoA Biosynthesis
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108471
pathway component: SubPathwayInteraction108471
xref: Reference/PathWhiz PW000032
xref: Reference/SMPDB SMP0000027
name: SubPathway

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

Reference/TAXONOMY 10116
type: UnificationXref
id: 10116
db: TAXONOMY

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

Reference/ChEBI CHEBI 1146900
type: UnificationXref
id: CHEBI:1146900
db: ChEBI

ReactionCatalysis137172
type: Catalysis
controller: 4-aminobutyrate aminotransferase, mitochondrial
controlled: Reaction147008
control type: ACTIVATION

ReactionCatalysis137173
type: Catalysis
controller: Glutamate decarboxylase 1
controlled: 4.1.1.15
control type: ACTIVATION

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

Reference/BioCyc CO-A
type: UnificationXref
id: CO-A
db: BioCyc

Reference/SMPDB SMP0000016
type: UnificationXref
id: SMP0000016
db: SMPDB

Reference/BioCyc B-ALANINE
type: UnificationXref
id: B-ALANINE
db: BioCyc

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

3-Aminopropionaldehyde
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 3-Aminopropionaldehyde

Reference/CAS 72-89-9
type: UnificationXref
id: 72-89-9
db: CAS

Dihydropyrimidine dehydrogenase [NADP(+)]
type: Complex
component stoichiometry: Protein/15352 Cytoplasm/Stoichiometry/2.0
cellular location: Cytoplasm
component: Dihydropyrimidine dehydrogenase [NADP(+)]

Membrane primary amine oxidase
type: Protein
cellular location: Cell Membrane
entity reference: Membrane primary amine oxidase

Coenzyme A
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 1146900
xref: Reference/HMDB HMDB0001423
xref: Reference/PubChem-compound 6816
xref: Reference/BioCyc CO-A
xref: Reference/ChemSpider 6557
xref: Reference/CAS 85-61-0
xref: Reference/KEGG Compound C00010
chemical formula: C21H36N7O16P3S
molecular weight: 767.11523
structure: ChemicalStructure/PW C001099

Compound/PW C000040 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: β-Alanine
stoichiometric coefficient: 1.0

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

Protein/14637 Cytoplasm/Stoichiometry/2.0
type: Stoichiometry
physical entity: Histidine decarboxylase
stoichiometric coefficient: 2.0

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

Reference/ChEBI CHEBI 7916
type: UnificationXref
id: CHEBI:7916
db: ChEBI

Compound/PW C000866 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Aminopropionaldehyde
stoichiometric coefficient: 1.0

Reference/CAS 584-85-0
type: UnificationXref
id: 584-85-0
db: CAS

Reference/ChemSpider 6557
type: UnificationXref
id: 6557
db: ChemSpider

Reference/SMPDB SMP0000027
type: UnificationXref
id: SMP0000027
db: SMPDB

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

ChemicalStructure/PW C000940
type: ChemicalStructure
structure format: SMILES

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

Reference/ChEBI CHEBI 17960
type: UnificationXref
id: CHEBI:17960
db: ChEBI

Malonic semialdehyde
type: SmallMoleculeReference
xref: Reference/CAS 926-61-4
xref: Reference/PubChem-compound 868
xref: Reference/ChEBI CHEBI 17960
xref: Reference/ChemSpider 845
xref: Reference/HMDB HMDB0011111
xref: Reference/KEGG Compound C00222
chemical formula: C3H4O3
molecular weight: 88.016045
structure: ChemicalStructure/PW C006753

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

Water
type: SmallMolecule
cellular location: Extracellular Space
entity reference: Water

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

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

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

Reference/KEGG Compound C00986
type: UnificationXref
id: C00986
db: KEGG Compound

Compound/PW C006753 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Malonic semialdehyde
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00864
type: UnificationXref
id: C00864
db: KEGG Compound

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

Reference/UniProt Q642F0
type: UnificationXref
id: Q642F0
db: UniProt

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

Reference/PathWhiz PW000149
type: UnificationXref
id: PW000149
db: PathWhiz

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

Reference/ChemSpider 392413
type: UnificationXref
id: 392413
db: ChemSpider

Reference/PubChem-compound 1174
type: UnificationXref
id: 1174
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

Protein/15352 Cytoplasm/Stoichiometry/2.0
type: Stoichiometry
physical entity: Dihydropyrimidine dehydrogenase [NADP(+)]
stoichiometric coefficient: 2.0

Reference/ChEBI CHEBI 27596
type: UnificationXref
id: CHEBI:27596
db: ChEBI

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

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

Reference/CAS 368-16-1
type: UnificationXref
id: 368-16-1
db: CAS

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

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

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

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

Reference/PubChem-compound 439224
type: UnificationXref
id: 439224
db: PubChem-compound

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

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

Pantothenic acid
type: SmallMolecule
cellular location: Extracellular Space
entity reference: Pantothenic acid
name: SubPathwayInput

ChemicalStructure/PW C000866
type: ChemicalStructure
structure format: SMILES
structure data: NCCC=O

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

Reference/PubChem-compound 112072
type: UnificationXref
id: 112072
db: PubChem-compound

Aldehyde dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/15360 Mitochondrial Matrix/Stoichiometry/4.0
cellular location: Mitochondrial Matrix
component: Aldehyde dehydrogenase, mitochondrial

Reference/UniProt Q66HG3
type: UnificationXref
id: Q66HG3
db: UniProt

Reference/KEGG Compound C00429
type: UnificationXref
id: C00429
db: KEGG Compound

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

Reference/ChEBI CHEBI 15351
type: UnificationXref
id: CHEBI:15351
db: ChEBI

Reference/UniProt Q91ZD7
type: UnificationXref
id: Q91ZD7
db: UniProt

Protein/14638 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: 4-aminobutyrate aminotransferase, mitochondrial
stoichiometric coefficient: 2.0

Reference/CAS 85-61-0
type: UnificationXref
id: 85-61-0
db: CAS

4-aminobutyrate aminotransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P50554
organism: Rattus norvegicus

Histidine decarboxylase
type: ProteinReference
xref: Reference/UniProt P16453
organism: Rattus norvegicus