Reference/ChemSpider 151611
type: UnificationXref
id: 151611
db: ChemSpider

Reference/CAS 60113-82-8
type: UnificationXref
id: 60113-82-8
db: CAS

5-Hydroxyvalproic acid
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: 5-Hydroxyvalproic acid

Valproic acid
type: SmallMolecule
cellular location: Endoplasmic Reticulum Lumen
entity reference: Valproic acid

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

Reference/ChemSpider 10467181
type: UnificationXref
id: 10467181
db: ChemSpider

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

Reference/HMDB HMDB0061239
type: UnificationXref
id: HMDB0061239
db: HMDB

Trifunctional enzyme subunit alpha, mitochondrial
type: ProteinReference
xref: Reference/UniProt P40939
organism: BiologicalState10

3-oxo-Valproic acid CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 3-oxo-Valproic acid CoA
name: SubPathwayActivator

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

NADPH
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: NADPH

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

Compound/PW C040714 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-ene-Valproic acid CoA
stoichiometric coefficient: 1.0

2-ene-Valproic acid CoA
type: SmallMolecule
cellular location: Peroxisome
entity reference: 2-ene-Valproic acid CoA

Reaction2330
type: BiochemicalReaction
participant stoichiometry: Compound/PW C008175 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040716 Mitochondrion/Stoichiometry/1.0
right: Pentanoyl-CoA
spontaneous: false
left: 3-Hydroxyvalproic acid CoA
name: 3-Hydroxyvalproic acid CoA → Pentanoyl-CoA
conversion direction: LEFT_TO_RIGHT

Compound/PW C000146 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADPH
stoichiometric coefficient: 1.0

Compound/PW C040715 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-ene-Valproic acid CoA
stoichiometric coefficient: 1.0

Reference/UniProt P45954
type: UnificationXref
id: P45954
db: UniProt

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

Compound/PW C008639 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-ene-Valproic acid
stoichiometric coefficient: 1.0

3-ene-Valproic acid CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 3-ene-Valproic acid CoA

2-ene-Valproic acid CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 2-ene-Valproic acid CoA

Reference/HMDB HMDB0061342
type: UnificationXref
id: HMDB0061342
db: HMDB

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

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

Reference/CAS 60113-81-7
type: UnificationXref
id: 60113-81-7
db: CAS

Reference/ChEBI CHEBI 17200
type: UnificationXref
id: CHEBI:17200
db: ChEBI

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

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

Hydrogen Ion
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: Hydrogen Ion

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

Reference/ChemSpider 94669
type: UnificationXref
id: 94669
db: ChemSpider

Compound/PW C008640/Stoichiometry/1.0
type: Stoichiometry
physical entity: 5-Hydroxyvalproic acid
stoichiometric coefficient: 1.0

3-Hydroxyvalproic acid CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 3-Hydroxyvalproic acid CoA

Isovaleryl-CoA dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P26440
organism: BiologicalState10

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

Reference/UniProt P11712
type: UnificationXref
id: P11712
db: UniProt

Reference/BioCyc UDP-GLUCURONATE
type: UnificationXref
id: UDP-GLUCURONATE
db: BioCyc

Reference/PubChem-compound 6031
type: UnificationXref
id: 6031
db: PubChem-compound

Reference/KEGG Compound C00100
type: UnificationXref
id: C00100
db: KEGG Compound

Reference/ChemSpider 388465
type: UnificationXref
id: 388465
db: ChemSpider

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

ChemicalStructure/PW C001099
type: ChemicalStructure
structure format: SMILES

Reference/PubChem-compound 439164
type: UnificationXref
id: 439164
db: PubChem-compound

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

Compound/PW C040717 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-ene-Valproic acid CoA
stoichiometric coefficient: 1.0

Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial

ChemicalStructure/PW C040543
type: ChemicalStructure
structure format: SMILES
structure data: CCCC(C(O)=O)C(=O)CC

Reference/ChemSpider 79488
type: UnificationXref
id: 79488
db: ChemSpider

Uridine diphosphate glucuronic acid
type: SmallMoleculeReference
xref: Reference/BioCyc UDP-GLUCURONATE
xref: Reference/CAS 2616-64-0
xref: Reference/PubChem-compound 17473
xref: Reference/HMDB HMDB0000935
xref: Reference/ChEBI CHEBI 17200
xref: Reference/ChemSpider 16522
xref: Reference/KEGG Compound C00167
chemical formula: C15H22N2O18P2
molecular weight: 580.0343
structure: ChemicalStructure/PW C000746

Propionyl-CoA
type: SmallMoleculeReference
xref: Reference/CAS 317-66-8
xref: Reference/PubChem-compound 439164
xref: Reference/KEGG Compound C00100
xref: Reference/ChEBI CHEBI 15539
xref: Reference/BioCyc PROPIONYL-COA
xref: Reference/ChemSpider 388310
xref: Reference/HMDB HMDB0001275
chemical formula: C24H40N7O17P3S
molecular weight: 823.1414
structure: ChemicalStructure/PW C000988

ChemicalStructure/PW C040545
type: ChemicalStructure
structure format: SMILES
structure data: CCCC(\C=C/C)C(O)=O

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

ChemicalStructure/PW C040548
type: ChemicalStructure
structure format: SMILES
structure data: CCCC(CCC(O)=O)C(O)=O

UDP-glucuronosyltransferase 1-3
type: ProteinReference
xref: Reference/UniProt P35503
organism: BiologicalState10

Reference/ChemSpider 388310
type: UnificationXref
id: 388310
db: ChemSpider

Compound/PW C008643/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-ene-Valproic acid
stoichiometric coefficient: 1.0

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

Propionyl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Propionyl-CoA

Valproic acid CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Valproic acid CoA

Compound/PW C001144 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADH
stoichiometric coefficient: 1.0

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

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

Reference/PubChem-compound 134459
type: UnificationXref
id: 134459
db: PubChem-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/BioCyc Beta-D-Glucuronides
type: UnificationXref
id: Beta-D-Glucuronides
db: BioCyc

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

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

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

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

Reference/ChEBI CHEBI 288752
type: UnificationXref
id: CHEBI:288752
db: ChEBI

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

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

3-hydroxyacyl-CoA dehydrogenase type-2
type: Complex
component stoichiometry: Protein/371 Mitochondrial Matrix/Stoichiometry/4.0
cellular location: Mitochondrial Matrix
component: 3-hydroxyacyl-CoA dehydrogenase type-2

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

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

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

Compound/PW C008642/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Hydroxyvalproic acid
stoichiometric coefficient: 1.0

ChemicalStructure/PW C040565
type: ChemicalStructure
structure format: SMILES
structure data: CCCC(CC(C)=O)C(O)=O

Reference/ChemSpider 3009
type: UnificationXref
id: 3009
db: ChemSpider

Compound/PW C001249/Stoichiometry/1.0
type: Stoichiometry
physical entity: Valproic acid
stoichiometric coefficient: 1.0

ChemicalStructure/PW C040569
type: ChemicalStructure
structure format: SMILES
structure data: CCC\C(=C/C=C)C(O)=O

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

Valproic acid glucuronide
type: SmallMoleculeReference
xref: Reference/ChemSpider 79488
xref: Reference/CAS 60113-83-9
xref: Reference/HMDB HMDB0000901
xref: Reference/BioCyc Beta-D-Glucuronides
xref: Reference/PubChem-compound 88111
chemical formula: C14H24O8
molecular weight: 320.14713
structure: ChemicalStructure/PW C000720

Reference/HMDB HMDB0061381
type: UnificationXref
id: HMDB0061381
db: HMDB

Compound/PW C040713 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Valproic acid CoA
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0061382
type: UnificationXref
id: HMDB0061382
db: HMDB

Reference/HMDB HMDB0000901
type: UnificationXref
id: HMDB0000901
db: HMDB

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

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

Reference/KEGG Compound C00015
type: UnificationXref
id: C00015
db: KEGG Compound

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

ChemicalStructure/PW C008639
type: ChemicalStructure
structure format: SMILES
structure data: CCCC(CC=C)C(O)=O

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

Protein/371 Mitochondrial Matrix/Stoichiometry/4.0
type: Stoichiometry
physical entity: 3-hydroxyacyl-CoA dehydrogenase type-2
stoichiometric coefficient: 4.0

Reference/BioCyc 5-HYDROXYPENTANOYL-COA
type: UnificationXref
id: 5-HYDROXYPENTANOYL-COA
db: BioCyc

Reference/PubChem-compound 134467
type: UnificationXref
id: 134467
db: PubChem-compound

ChemicalStructure/PW C040575
type: ChemicalStructure
structure format: SMILES
structure data: CCCC(CC(O)=O)C(O)=O

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

Reference/SMPDB SMP0000635
type: UnificationXref
id: SMP0000635
db: SMPDB

4-Hydroxyvalproic acid
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: 4-Hydroxyvalproic acid

BiologicalState54
type: BioSource
cell type: Hepatocyte
xref: Reference/TAXONOMY 9606
name: Homo sapiens, Liver, Hepatocyte
tissue: Liver

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

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

UDP-glucuronosyltransferase 1-3
type: Protein
cellular location: Endoplasmic Reticulum Lumen
entity reference: UDP-glucuronosyltransferase 1-3

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

Protein/46 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Trifunctional enzyme subunit beta, mitochondrial
stoichiometric coefficient: 4.0

Isovaleryl-CoA dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Isovaleryl-CoA dehydrogenase, mitochondrial

Reference/CAS 317-66-8
type: UnificationXref
id: 317-66-8
db: CAS

Peroxisomal bifunctional enzyme
type: ProteinReference
xref: Reference/UniProt Q08426
organism: Homo sapiens

Cytochrome P450 2A6
type: Protein
cellular location: Endoplasmic Reticulum Membrane
entity reference: Cytochrome P450 2A6

3-hydroxyacyl-CoA dehydrogenase type-2
type: ProteinReference
xref: Reference/UniProt Q99714
organism: BiologicalState10

ChemicalStructure/PW C008643
type: ChemicalStructure
structure format: SMILES
structure data: CCC\C(=C/CC)C(O)=O

3-Hydroxyvalproic acid
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: 3-Hydroxyvalproic acid

ChemicalStructure/PW C008641
type: ChemicalStructure
structure format: SMILES
structure data: CCCC(C(O)CC)C(O)=O

ChemicalStructure/PW C008642
type: ChemicalStructure
structure format: SMILES
structure data: CCCC(CC(C)O)C(O)=O

ChemicalStructure/PW C008640
type: ChemicalStructure
structure format: SMILES
structure data: CCCC(CCCO)C(O)=O

4-ene-Valproic acid
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: 4-ene-Valproic acid

Reference/PubChem-compound 24892812
type: UnificationXref
id: 24892812
db: PubChem-compound

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

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

Peroxisome
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005777

Uridine 5'-diphosphate
type: SmallMolecule
cellular location: Endoplasmic Reticulum Lumen
entity reference: Uridine 5'-diphosphate

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

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

Compound/PW C040718 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-oxo-Valproic acid CoA
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0060682
type: UnificationXref
id: HMDB0060682
db: HMDB

Reference/HMDB HMDB0060684
type: UnificationXref
id: HMDB0060684
db: HMDB

Reference/HMDB HMDB0060683
type: UnificationXref
id: HMDB0060683
db: HMDB

Reference/PubChem-compound 6441809
type: UnificationXref
id: 6441809
db: PubChem-compound

Compound/PW C040543/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Oxovalproic acid
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00167
type: UnificationXref
id: C00167
db: KEGG Compound

Reference/PubChem-compound 3031829
type: UnificationXref
id: 3031829
db: PubChem-compound

4-ene-Valproic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 4-ene-Valproic acid

2-ene-Valproic acid CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 2-ene-Valproic acid CoA

Uridine 5'-diphosphate
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 17659
xref: Reference/PubChem-compound 6031
xref: Reference/CAS 58-98-0
xref: Reference/HMDB HMDB0000295
xref: Reference/KEGG Compound C00015
xref: Reference/ChemSpider 5809
xref: Reference/BioCyc UDP
chemical formula: C9H14N2O12P2
molecular weight: 404.0022
structure: ChemicalStructure/PW C000201

Pentanoyl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Pentanoyl-CoA

Compound/PW C040569/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Propyl-2,4-pentadienoic acid
stoichiometric coefficient: 1.0

Reference/ChemSpider 119147
type: UnificationXref
id: 119147
db: ChemSpider

Compound/PW C001249 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Valproic acid
stoichiometric coefficient: 1.0

Reference/ChemSpider 4945939
type: UnificationXref
id: 4945939
db: ChemSpider

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

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

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

Water
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: Water

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

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

ChemicalStructure/PW C000032
type: ChemicalStructure
structure format: SMILES

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

3-Hydroxyvalproic acid CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 3-Hydroxyvalproic acid CoA

Reference/ChemSpider 16522
type: UnificationXref
id: 16522
db: ChemSpider

Reference/HMDB HMDB0060762
type: UnificationXref
id: HMDB0060762
db: HMDB

Reference/KEGG Compound C16650
type: UnificationXref
id: C16650
db: KEGG Compound

Reference/KEGG Compound C16651
type: UnificationXref
id: C16651
db: KEGG Compound

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

BiologicalState10
type: BioSource
xref: Reference/TAXONOMY 9606
name: Homo sapiens, Liver
tissue: Liver

ChemicalStructure/PW C001249
type: ChemicalStructure
structure format: SMILES
structure data: CCCC(CCC)C(O)=O

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

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

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

Reference/KEGG Compound C16649
type: UnificationXref
id: C16649
db: KEGG Compound

ReactionCatalysis1759
type: Catalysis
controller: Cytochrome P450 2A6
controlled: 1.14.13.-
control type: ACTIVATION

Acyl-coenzyme A synthetase ACSM1, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Acyl-coenzyme A synthetase ACSM1, mitochondrial

Reference/KEGG Compound C16648
type: UnificationXref
id: C16648
db: KEGG Compound

Reference/UniProt P20813
type: UnificationXref
id: P20813
db: UniProt

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

Endoplasmic Reticulum Lumen
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005788

Reference/CAS 58-98-0
type: UnificationXref
id: 58-98-0
db: CAS

Compound/PW C040716 Peroxisome/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxyvalproic acid CoA
stoichiometric coefficient: 1.0

Compound/PW C040034 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydrogen Ion
stoichiometric coefficient: 1.0

Compound/PW C040548/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Propylglutaric acid
stoichiometric coefficient: 1.0

Compound/PW C040575/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Propylsuccinic acid
stoichiometric coefficient: 1.0

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

Reference/CAS 58888-84-9
type: UnificationXref
id: 58888-84-9
db: CAS

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

Compound/PW C040716 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxyvalproic acid CoA
stoichiometric coefficient: 1.0

Reference/KEGG Compound C16656
type: UnificationXref
id: C16656
db: KEGG Compound

Reference/KEGG Compound C16657
type: UnificationXref
id: C16657
db: KEGG Compound

Reference/KEGG Compound C16658
type: UnificationXref
id: C16658
db: KEGG Compound

Reference/KEGG Compound C16652
type: UnificationXref
id: C16652
db: KEGG Compound

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

Reference/HMDB HMDB0000295
type: UnificationXref
id: HMDB0000295
db: HMDB

Reference/KEGG Compound C16653
type: UnificationXref
id: C16653
db: KEGG Compound

Reference/KEGG Compound C16654
type: UnificationXref
id: C16654
db: KEGG Compound

Reference/ChemSpider 5809
type: UnificationXref
id: 5809
db: ChemSpider

Reference/KEGG Compound C16655
type: UnificationXref
id: C16655
db: KEGG Compound

ChemicalStructure/PW C040716
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C040715
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C040714
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C040713
type: ChemicalStructure
structure format: SMILES

4-ene-Valproic acid CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 4-ene-Valproic acid CoA

ChemicalStructure/PW C040719
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C040718
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C040717
type: ChemicalStructure
structure format: SMILES

2,3-diene-Valproic acid-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 2,3-diene-Valproic acid-CoA

Reference/CAS 32806-62-5
type: UnificationXref
id: 32806-62-5
db: CAS

Compound/PW C040715 Peroxisome/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-ene-Valproic acid CoA
stoichiometric coefficient: 1.0

Reference/PubChem-compound 134970
type: UnificationXref
id: 134970
db: PubChem-compound

Reference/HMDB HMDB0001275
type: UnificationXref
id: HMDB0001275
db: HMDB

1.14.13.-
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000721 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001249 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C008639 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
right: NADH
right: 4-ene-Valproic acid
spontaneous: false
EC number: 1.14.13.-
left: NAD
left: Valproic acid
name: NAD + Valproic acid → 4-ene-Valproic acid + NADH
conversion direction: LEFT_TO_RIGHT

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

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

ChemicalStructure/PW C040721
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C040720
type: ChemicalStructure
structure format: SMILES

Compound/PW C040719 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2,3-diene-Valproic acid-CoA
stoichiometric coefficient: 1.0

Reference/UniProt Q08426
type: UnificationXref
id: Q08426
db: UniProt

Reference/UniProt P26440
type: UnificationXref
id: P26440
db: UniProt

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

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

Compound/PW C040715 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-ene-Valproic acid CoA
stoichiometric coefficient: 1.0

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

Protein/1496 Mitochondrial Matrix/Stoichiometry/4.0
type: Stoichiometry
physical entity: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
stoichiometric coefficient: 4.0

Compound/PW C040720 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2,4-diene-Valproic acid-CoA
stoichiometric coefficient: 1.0

Pentanoyl-CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0013037
xref: Reference/KEGG Compound C00888
xref: Reference/ChEBI CHEBI 15536
xref: Reference/BioCyc 5-HYDROXYPENTANOYL-COA
xref: Reference/PubChem-compound 439337
xref: Reference/ChemSpider 388465
chemical formula: C26H44N7O17P3S
molecular weight: 851.1727
structure: ChemicalStructure/PW C008175

Reference/UniProt P55084
type: UnificationXref
id: P55084
db: UniProt

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

Reference/HMDB HMDB0060685
type: UnificationXref
id: HMDB0060685
db: HMDB

Reference/ChemSpider 4941657
type: UnificationXref
id: 4941657
db: ChemSpider

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

Reference/HMDB HMDB0013037
type: UnificationXref
id: HMDB0013037
db: HMDB

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

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

ReactionCatalysis1707
type: Catalysis
controller: Cytochrome P450 2A6
controlled: 1.14.13.-
control type: ACTIVATION

Peroxisomal bifunctional enzyme
type: Protein
cellular location: Peroxisome
entity reference: Peroxisomal bifunctional enzyme

ReactionCatalysis1711
type: Catalysis
controller: Cytochrome P450 2A6
controlled: 1.14.13.-
control type: ACTIVATION

ReactionCatalysis1713
type: Catalysis
controller: Cytochrome P450 2C9
controlled: 1.14.13.-
control type: ACTIVATION

ReactionCatalysis1712
type: Catalysis
controller: Cytochrome P450 2B6
controlled: 1.14.13.-
control type: ACTIVATION

Uridine diphosphate glucuronic acid
type: SmallMolecule
cellular location: Endoplasmic Reticulum Lumen
entity reference: Uridine diphosphate glucuronic acid

Acyl-coenzyme A synthetase ACSM1, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q08AH1
organism: BiologicalState54

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

Reference/ChEBI CHEBI 410867
type: UnificationXref
id: CHEBI:410867
db: ChEBI

2,4-diene-Valproic acid-CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0061381
chemical formula: C29H46N7O17P3S
molecular weight: 889.18835
structure: ChemicalStructure/PW C040720

Valproyl-dephospho-CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0061382
chemical formula: C29H49N7O14P2S
molecular weight: 813.25336
structure: ChemicalStructure/PW C040721

Trifunctional enzyme subunit beta, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Trifunctional enzyme subunit beta, mitochondrial

Reference/PubChem-compound 88111
type: UnificationXref
id: 88111
db: PubChem-compound

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

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

Compound/PW C000988 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Propionyl-CoA
stoichiometric coefficient: 1.0

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

ChemicalStructure/PW C008175
type: ChemicalStructure
structure format: SMILES

Reference/UniProt P40939
type: UnificationXref
id: P40939
db: UniProt

Reference/PathWhiz PW000611
type: UnificationXref
id: PW000611
db: PathWhiz

Reaction1923
type: BiochemicalReaction
participant stoichiometry: Compound/PW C040565/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040575/Stoichiometry/1.0
right: 2-Propylsuccinic acid
spontaneous: false
left: 2-n-Propyl-4-oxopentanoic acid
name: 2-n-Propyl-4-oxopentanoic acid → 2-Propylsuccinic acid
conversion direction: LEFT_TO_RIGHT

Reference/CAS 2616-64-0
type: UnificationXref
id: 2616-64-0
db: CAS

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

Cytochrome P450 2B6
type: ProteinReference
xref: Reference/UniProt P20813
organism: BiologicalState10

Valproic acid
type: SmallMoleculeReference
xref: Reference/CAS 99-66-1
xref: Reference/ChemSpider 3009
xref: Reference/HMDB HMDB0001877
xref: Reference/KEGG Compound C07185
xref: Reference/ChEBI CHEBI 39867
xref: Reference/PubChem-compound 3121
chemical formula: C8H16O2
molecular weight: 144.11504
structure: ChemicalStructure/PW C001249

Compound/PW C040713 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Valproic acid CoA
stoichiometric coefficient: 1.0

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

Reference/HMDB HMDB0001877
type: UnificationXref
id: HMDB0001877
db: HMDB

Reaction1922
type: BiochemicalReaction
participant stoichiometry: Compound/PW C040565/Stoichiometry/1.0
participant stoichiometry: Compound/PW C008642/Stoichiometry/1.0
right: 2-n-Propyl-4-oxopentanoic acid
spontaneous: false
left: 4-Hydroxyvalproic acid
name: 4-Hydroxyvalproic acid → 2-n-Propyl-4-oxopentanoic acid
conversion direction: LEFT_TO_RIGHT

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

Reference/HMDB HMDB0060714
type: UnificationXref
id: HMDB0060714
db: HMDB

Reaction1920
type: BiochemicalReaction
participant stoichiometry: Compound/PW C008639/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040569/Stoichiometry/1.0
right: 2-Propyl-2,4-pentadienoic acid
spontaneous: false
left: 4-ene-Valproic acid
name: 4-ene-Valproic acid → 2-Propyl-2,4-pentadienoic acid
conversion direction: LEFT_TO_RIGHT

Compound/PW C008639 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-ene-Valproic acid
stoichiometric coefficient: 1.0

Reference/CAS 99-66-1
type: UnificationXref
id: 99-66-1
db: CAS

Reference/PubChem-compound 104896
type: UnificationXref
id: 104896
db: PubChem-compound

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

Cytochrome P450 2A6
type: ProteinReference
xref: Reference/UniProt P11509
organism: BiologicalState10

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

Reference/CAS 1575-72-0
type: UnificationXref
id: 1575-72-0
db: CAS

Compound/PW C001249 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Valproic acid
stoichiometric coefficient: 1.0

Compound/PW C001249 Endoplasmic Reticulum Lumen/Stoichiometry/1.0
type: Stoichiometry
physical entity: Valproic acid
stoichiometric coefficient: 1.0

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

Valproic acid
type: SmallMolecule
entity reference: Valproic acid

Reference/UniProt Q99714
type: UnificationXref
id: Q99714
db: UniProt

Compound/PW C040716 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxyvalproic acid CoA
stoichiometric coefficient: 1.0

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

Compound/PW C008640 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: 5-Hydroxyvalproic acid
stoichiometric coefficient: 1.0

Cytochrome P450 2C9
type: ProteinReference
xref: Reference/UniProt P11712
organism: BiologicalState10

ReactionCatalysis2203
type: Catalysis
controller: UDP-glucuronosyltransferase 1-3
controlled: 2.4.1.17
control type: ACTIVATION

ChemicalStructure/PW C000201
type: ChemicalStructure
structure format: SMILES

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

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

Valproic acid glucuronide
type: SmallMolecule
cellular location: Endoplasmic Reticulum Lumen
entity reference: Valproic acid glucuronide

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

Reference/ChemSpider 2962072
type: UnificationXref
id: 2962072
db: ChemSpider

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

SubPathwayControl860
type: Interaction
name: ActivatingSubPathwayControl
name: SubPathway860Control
participant: 2,4-diene-Valproic acid-CoA
participant: Hepatotoxicity
participant: 3-oxo-Valproic acid CoA

Compound/PW C000720 Endoplasmic Reticulum Lumen/Stoichiometry/1.0
type: Stoichiometry
physical entity: Valproic acid glucuronide
stoichiometric coefficient: 1.0

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

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

ReactionCatalysis1764
type: Catalysis
controller: Cytochrome P450 2C9
controlled: 1.14.13.-
control type: ACTIVATION

ReactionCatalysis1763
type: Catalysis
controller: Cytochrome P450 2B6
controlled: 1.14.13.-
control type: ACTIVATION

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

Reference/HMDB HMDB0060749
type: UnificationXref
id: HMDB0060749
db: HMDB

Compound/PW C040721 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Valproyl-dephospho-CoA
stoichiometric coefficient: 1.0

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

Reference/HMDB HMDB0060744
type: UnificationXref
id: HMDB0060744
db: HMDB

ReactionCatalysis1760
type: Catalysis
controller: Cytochrome P450 2C9
controlled: 1.14.13.-
control type: ACTIVATION

Reference/HMDB HMDB0060740
type: UnificationXref
id: HMDB0060740
db: HMDB

ReactionCatalysis1762
type: Catalysis
controller: Cytochrome P450 2A6
controlled: 1.14.13.-
control type: ACTIVATION

ReactionCatalysis1761
type: Catalysis
controller: Cytochrome P450 2B6
controlled: 1.14.13.-
control type: ACTIVATION

NADH
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: NADH

Oxygen
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: Oxygen

Reference/HMDB HMDB0013897
type: UnificationXref
id: HMDB0013897
db: HMDB

3-Hydroxyvalproic acid CoA
type: SmallMolecule
cellular location: Peroxisome
entity reference: 3-Hydroxyvalproic acid CoA

Reference/HMDB HMDB0013898
type: UnificationXref
id: HMDB0013898
db: HMDB

Reference/HMDB HMDB0013899
type: UnificationXref
id: HMDB0013899
db: HMDB

Reference/ChEBI CHEBI 15536
type: UnificationXref
id: CHEBI:15536
db: ChEBI

Compound/PW C008639/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-ene-Valproic acid
stoichiometric coefficient: 1.0

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

ChemicalStructure/PW C000143
type: ChemicalStructure
structure format: SMILES

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

Reference/ChemSpider 30791778
type: UnificationXref
id: 30791778
db: ChemSpider

Reference/CAS 60218-41-9
type: UnificationXref
id: 60218-41-9
db: CAS

ChemicalStructure/PW C000146
type: ChemicalStructure
structure format: SMILES

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

Reference/PubChem-compound 17473
type: UnificationXref
id: 17473
db: PubChem-compound

Reference/HMDB HMDB0060877
type: UnificationXref
id: HMDB0060877
db: HMDB

Reference/PubChem-compound 3731686
type: UnificationXref
id: 3731686
db: PubChem-compound

Reference/UniProt Q08AH1
type: UnificationXref
id: Q08AH1
db: UniProt

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

Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P45954
organism: BiologicalState10

Compound/PW C008175 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pentanoyl-CoA
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

2,4-diene-Valproic acid-CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 2,4-diene-Valproic acid-CoA
name: SubPathwayActivator

Reference/ChemSpider 118528
type: UnificationXref
id: 118528
db: ChemSpider

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

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

Reference/HMDB HMDB0060723
type: UnificationXref
id: HMDB0060723
db: HMDB

1.14.13.-
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000146 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001249 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C008641 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000143 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
right: Water
right: 3-Hydroxyvalproic acid
right: NADP
spontaneous: false
EC number: 1.14.13.-
left: Oxygen
left: NADPH
left: Valproic acid
left: Hydrogen Ion
name: Hydrogen Ion + NADPH + Oxygen + Valproic acid → 3-Hydroxyvalproic acid + NADP + Water
conversion direction: LEFT_TO_RIGHT

Reference/ChemSpider 118522
type: UnificationXref
id: 118522
db: ChemSpider

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

Cytochrome P450 2B6
type: Protein
cellular location: Endoplasmic Reticulum Membrane
entity reference: Cytochrome P450 2B6

Compound/PW C000143 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADP
stoichiometric coefficient: 1.0

Valproic acid CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0060877
chemical formula: C29H50N7O17P3S
molecular weight: 893.21967
structure: ChemicalStructure/PW C040713

4-ene-Valproic acid CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0060762
chemical formula: C29H48N7O17P3S
molecular weight: 891.20404
structure: ChemicalStructure/PW C040714

2-ene-Valproic acid CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0060714
chemical formula: C29H48N7O17P3S
molecular weight: 891.20404
structure: ChemicalStructure/PW C040715

Valproic acid CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Valproic acid CoA

3-Hydroxyvalproic acid CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0060744
chemical formula: C29H50N7O18P3S
molecular weight: 909.2146
structure: ChemicalStructure/PW C040716

3-ene-Valproic acid CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0060740
xref: Reference/PubChem-compound 3121
chemical formula: C29H48N7O17P3S
molecular weight: 891.20404
structure: ChemicalStructure/PW C040717

3-oxo-Valproic acid CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0060749
xref: Reference/PubChem-compound 3121
chemical formula: C29H48N7O18P3S
molecular weight: 907.1989
structure: ChemicalStructure/PW C040718

2,3-diene-Valproic acid-CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0060723
xref: Reference/PubChem-compound 3121
chemical formula: C29H47N7O17P3S
molecular weight: 890.1962
structure: ChemicalStructure/PW C040719

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

Trifunctional enzyme, mitochondrial
type: Complex
component stoichiometry: Protein/381 Mitochondrion/Stoichiometry/4.0
component stoichiometry: Protein/46 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: Trifunctional enzyme subunit alpha, mitochondrial
component: Trifunctional enzyme subunit beta, mitochondrial

Hepatotoxicity
type: Pathway
name: ActivatingSubPathway

2-n-Propyl-4-oxopentanoic acid
type: SmallMoleculeReference
xref: Reference/ChemSpider 2962072
xref: Reference/HMDB HMDB0060683
xref: Reference/PubChem-compound 3731686
xref: Reference/KEGG Compound C16655
chemical formula: C8H14O3
molecular weight: 158.0943
structure: ChemicalStructure/PW C040565

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

2-Propyl-2,4-pentadienoic acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0060682
xref: Reference/CAS 72010-18-5
xref: Reference/PubChem-compound 6441809
xref: Reference/ChemSpider 4945939
xref: Reference/KEGG Compound C16656
chemical formula: C8H12O2
molecular weight: 140.08372
structure: ChemicalStructure/PW C040569

Endoplasmic Reticulum Membrane
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005789

Compound/PW C008642 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: 4-Hydroxyvalproic acid
stoichiometric coefficient: 1.0

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C000720
type: ChemicalStructure
structure format: SMILES

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

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

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

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

1.14.13.-
type: BiochemicalReaction
participant stoichiometry: Compound/PW C008640 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000146 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001249 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000143 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
right: Water
right: 5-Hydroxyvalproic acid
right: NADP
spontaneous: false
EC number: 1.14.13.-
left: Oxygen
left: NADPH
left: Valproic acid
left: Hydrogen Ion
name: Hydrogen Ion + NADPH + Oxygen + Valproic acid → 5-Hydroxyvalproic acid + NADP + Water
conversion direction: LEFT_TO_RIGHT

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

1.14.13.-
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000146 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001249 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C008642 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000143 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
right: Water
right: 4-Hydroxyvalproic acid
right: NADP
spontaneous: false
EC number: 1.14.13.-
left: Oxygen
left: NADPH
left: Valproic acid
left: Hydrogen Ion
name: Hydrogen Ion + NADPH + Oxygen + Valproic acid → 4-Hydroxyvalproic acid + NADP + Water
conversion direction: LEFT_TO_RIGHT

Reaction1894
type: BiochemicalReaction
participant stoichiometry: Compound/PW C008640/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040548/Stoichiometry/1.0
right: 2-Propylglutaric acid
spontaneous: false
left: 5-Hydroxyvalproic acid
name: 5-Hydroxyvalproic acid → 2-Propylglutaric acid
conversion direction: LEFT_TO_RIGHT

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

2-Propylsuccinic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 3031829
xref: Reference/KEGG Compound C16657
xref: Reference/HMDB HMDB0061239
xref: Reference/ChemSpider 10467181
chemical formula: C7H12O4
molecular weight: 160.07356
structure: ChemicalStructure/PW C040575

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

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

Reference/HMDB HMDB0000935
type: UnificationXref
id: HMDB0000935
db: HMDB

Reaction1881
type: BiochemicalReaction
participant stoichiometry: Compound/PW C008641/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040543/Stoichiometry/1.0
right: 3-Oxovalproic acid
spontaneous: false
left: 3-Hydroxyvalproic acid
name: 3-Hydroxyvalproic acid → 3-Oxovalproic acid
conversion direction: LEFT_TO_RIGHT

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

NAD
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: NAD

Reaction1888
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001249/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040545/Stoichiometry/1.0
right: 3-ene-valproic acid
spontaneous: false
left: Valproic acid
name: Valproic acid → 3-ene-valproic acid
conversion direction: LEFT_TO_RIGHT

Reaction1887
type: BiochemicalReaction
participant stoichiometry: Compound/PW C008641/Stoichiometry/1.0
participant stoichiometry: Compound/PW C008643/Stoichiometry/1.0
right: 3-Hydroxyvalproic acid
spontaneous: false
left: 2-ene-Valproic acid
name: 2-ene-Valproic acid → 3-Hydroxyvalproic acid
conversion direction: LEFT_TO_RIGHT

Reaction1882
type: BiochemicalReaction
participant stoichiometry: Compound/PW C008643/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001249/Stoichiometry/1.0
right: 2-ene-Valproic acid
spontaneous: false
left: Valproic acid
name: Valproic acid → 2-ene-Valproic acid
conversion direction: LEFT_TO_RIGHT

Compound/PW C000201 Endoplasmic Reticulum Lumen/Stoichiometry/1.0
type: Stoichiometry
physical entity: Uridine 5'-diphosphate
stoichiometric coefficient: 1.0

Trifunctional enzyme subunit beta, mitochondrial
type: ProteinReference
xref: Reference/UniProt P55084
organism: BiologicalState10

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

Reference/HMDB HMDB0013900
type: UnificationXref
id: HMDB0013900
db: HMDB

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

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

Valproic acid
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: Valproic acid

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

Reference/PubChem-compound 173719
type: UnificationXref
id: 173719
db: PubChem-compound

Cytochrome P450 2C9
type: Protein
cellular location: Endoplasmic Reticulum Membrane
entity reference: Cytochrome P450 2C9

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/GENE ONTOLOGY GO 0005789
type: UnificationXref
id: GO:0005789
db: GENE ONTOLOGY

Reference/ChEBI CHEBI 15539
type: UnificationXref
id: CHEBI:15539
db: ChEBI

Reference/GENE ONTOLOGY GO 0005788
type: UnificationXref
id: GO:0005788
db: GENE ONTOLOGY

Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/1496 Mitochondrial Matrix/Stoichiometry/4.0
cellular location: Mitochondrial Matrix
component: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial

Reference/PubChem-compound 439337
type: UnificationXref
id: 439337
db: PubChem-compound

Reference/UniProt P35503
type: UnificationXref
id: P35503
db: UniProt

Reference/CAS 53660-23-4
type: UnificationXref
id: 53660-23-4
db: CAS

Reference/ChEBI CHEBI 39867
type: UnificationXref
id: CHEBI:39867
db: ChEBI

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

Reference/HMDB HMDB0013902
type: UnificationXref
id: HMDB0013902
db: HMDB

Reference/GENE ONTOLOGY GO 0005777
type: UnificationXref
id: GO:0005777
db: GENE ONTOLOGY

Reference/PubChem-compound 135244
type: UnificationXref
id: 135244
db: PubChem-compound

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

Reference/CAS 72010-18-5
type: UnificationXref
id: 72010-18-5
db: CAS

Compound/PW C000721 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: NAD
stoichiometric coefficient: 1.0

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

3-hydroxyacyl-CoA dehydrogenase type-2
type: Protein
cellular location: Mitochondrial Matrix
entity reference: 3-hydroxyacyl-CoA dehydrogenase type-2

Valproic Acid Metabolism Pathway
type: Pathway
pathway order: PathwayEnzymeReaction/2386
pathway order: PathwayEnzymeReaction/3553
pathway order: PathwayEnzymeReaction/3554
pathway order: PathwayEnzymeReaction/3566
pathway order: PathwayEnzymeReaction/2390
pathway order: PathwaySubPathway/860
pathway order: PathwayEnzymeReaction/3562
pathway order: PathwayEnzymeReaction/3563
pathway order: PathwayEnzymeReaction/2299
pathway order: PathwayEnzymeReaction/3564
pathway order: PathwayEnzymeReaction/2311
pathway order: PathwayEnzymeReaction/3565
pathway order: PathwayEnzymeReaction/3620
pathway order: PathwayEnzymeReaction/3560
pathway order: PathwayEnzymeReaction/3559
pathway order: PathwayEnzymeReaction/3614
pathway order: PathwayEnzymeReaction/3615
pathway order: PathwayEnzymeReaction/2307
pathway order: PathwayEnzymeReaction/3616
pathway order: PathwayEnzymeReaction/3617
pathway order: PathwayEnzymeReaction/2301
pathway order: PathwayEnzymeReaction/2389
pathway order: PathwayEnzymeReaction/3555
pathway order: PathwayEnzymeReaction/2324
pathway order: PathwayEnzymeReaction/3611
pathway order: PathwayEnzymeReaction/3557
pathway order: PathwayEnzymeReaction/3579
pathway order: PathwayEnzymeReaction/3612
pathway order: PathwayEnzymeReaction/3558
pathway order: PathwayEnzymeReaction/3613
pathway order: PathwayEnzymeReaction/3618
pathway order: PathwayEnzymeReaction/3619
pathway component: 1.14.13.-
pathway component: SubPathwayControl860
pathway component: ReactionCatalysis1764
pathway component: 1.3.8.4
pathway component: ReactionCatalysis1763
pathway component: Reaction2321
pathway component: Reaction2328
pathway component: Reaction2329
pathway component: ReactionCatalysis2231
pathway component: ReactionCatalysis2230
pathway component: ReactionCatalysis1760
pathway component: Reaction2324
pathway component: Reaction2325
pathway component: ReactionCatalysis2232
pathway component: ReactionCatalysis1762
pathway component: ReactionCatalysis2213
pathway component: Reaction2322
pathway component: Reaction1894
pathway component: ReactionCatalysis1761
pathway component: Reaction2323
pathway component: Reaction1923
pathway component: 1.3.8.5
pathway component: ReactionCatalysis1707
pathway component: ReactionCatalysis2226
pathway component: Reaction1881
pathway component: 2.4.1.17
pathway component: ReactionCatalysis2203
pathway component: ReactionCatalysis1711
pathway component: ReactionCatalysis2228
pathway component: ReactionCatalysis2227
pathway component: Reaction2330
pathway component: ReactionCatalysis1713
pathway component: ReactionCatalysis1712
pathway component: ReactionCatalysis2229
pathway component: ReactionCatalysis1759
pathway component: Reaction1888
pathway component: Reaction1922
pathway component: Reaction1887
pathway component: Reaction1920
pathway component: Reaction1882
pathway component: 6.2.1.2
xref: Reference/PathWhiz PW000611
xref: Reference/SMPDB SMP0000635
organism: Homo sapiens

ReactionCatalysis2213
type: Catalysis
controller: Acyl-coenzyme A synthetase ACSM1, mitochondrial
controlled: 6.2.1.2
control type: ACTIVATION

Compound/PW C008641/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxyvalproic acid
stoichiometric coefficient: 1.0

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

3-Oxovalproic acid
type: SmallMolecule
entity reference: 3-Oxovalproic acid

Reference/KEGG Compound C00888
type: UnificationXref
id: C00888
db: KEGG Compound

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

Protein/381 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Trifunctional enzyme subunit alpha, mitochondrial
stoichiometric coefficient: 4.0

3-ene-valproic acid
type: SmallMolecule
entity reference: 3-ene-valproic acid

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

4-ene-Valproic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 104896
xref: Reference/HMDB HMDB0013897
xref: Reference/ChemSpider 94669
xref: Reference/ChEBI CHEBI 410867
xref: Reference/CAS 1575-72-0
xref: Reference/KEGG Compound C16648
chemical formula: C8H14O2
molecular weight: 142.09938
structure: ChemicalStructure/PW C008639

2-Propylglutaric acid
type: SmallMolecule
entity reference: 2-Propylglutaric acid

ReactionCatalysis2226
type: Catalysis
controller: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.5
control type: ACTIVATION

2-ene-Valproic acid
type: SmallMoleculeReference
xref: Reference/CAS 60218-41-9
xref: Reference/PubChem-compound 6437068
xref: Reference/HMDB HMDB0013902
xref: Reference/KEGG Compound C16653
xref: Reference/ChEBI CHEBI 288752
xref: Reference/ChemSpider 4941657
chemical formula: C8H14O2
molecular weight: 142.09938
structure: ChemicalStructure/PW C008643

ReactionCatalysis2228
type: Catalysis
controller: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.4
control type: ACTIVATION

4-Hydroxyvalproic acid
type: SmallMoleculeReference
xref: Reference/KEGG Compound C16649
xref: Reference/ChemSpider 118528
xref: Reference/CAS 60113-82-8
xref: Reference/HMDB HMDB0013900
xref: Reference/PubChem-compound 134467
chemical formula: C8H16O3
molecular weight: 160.10994
structure: ChemicalStructure/PW C008642

ReactionCatalysis2227
type: Catalysis
controller: Isovaleryl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.4
control type: ACTIVATION

2-ene-Valproic acid
type: SmallMolecule
entity reference: 2-ene-Valproic acid

Reference/ChemSpider 118936
type: UnificationXref
id: 118936
db: ChemSpider

ReactionCatalysis2229
type: Catalysis
controller: Peroxisomal bifunctional enzyme
controlled: Reaction2321
control type: ACTIVATION

Trifunctional enzyme subunit alpha, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Trifunctional enzyme subunit alpha, mitochondrial

Reference/PubChem-compound 6437068
type: UnificationXref
id: 6437068
db: PubChem-compound

4-Hydroxyvalproic acid
type: SmallMolecule
entity reference: 4-Hydroxyvalproic acid

3-Hydroxyvalproic acid
type: SmallMolecule
entity reference: 3-Hydroxyvalproic acid

5-Hydroxyvalproic acid
type: SmallMolecule
entity reference: 5-Hydroxyvalproic acid

Valproyl-dephospho-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Valproyl-dephospho-CoA

3-Hydroxyvalproic acid
type: SmallMoleculeReference
xref: Reference/CAS 58888-84-9
xref: Reference/KEGG Compound C16651
xref: Reference/PubChem-compound 134459
xref: Reference/HMDB HMDB0013899
xref: Reference/ChemSpider 118522
chemical formula: C8H16O3
molecular weight: 160.10994
structure: ChemicalStructure/PW C008641

Compound/PW C008641 Endoplasmic Reticulum Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxyvalproic acid
stoichiometric coefficient: 1.0

Compound/PW C000746 Endoplasmic Reticulum Lumen/Stoichiometry/1.0
type: Stoichiometry
physical entity: Uridine diphosphate glucuronic acid
stoichiometric coefficient: 1.0

5-Hydroxyvalproic acid
type: SmallMoleculeReference
xref: Reference/KEGG Compound C16650
xref: Reference/HMDB HMDB0013898
xref: Reference/CAS 53660-23-4
xref: Reference/ChemSpider 119147
xref: Reference/PubChem-compound 135244
chemical formula: C8H16O3
molecular weight: 160.10994
structure: ChemicalStructure/PW C008640

Compound/PW C040565/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-n-Propyl-4-oxopentanoic acid
stoichiometric coefficient: 1.0

2-Propylsuccinic acid
type: SmallMolecule
entity reference: 2-Propylsuccinic acid

Reference/CAS 72010-19-6
type: UnificationXref
id: 72010-19-6
db: CAS

3-Oxovalproic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 173719
xref: Reference/ChemSpider 151611
xref: Reference/CAS 60113-81-7
xref: Reference/KEGG Compound C16652
xref: Reference/HMDB HMDB0060685
chemical formula: C8H14O3
molecular weight: 158.0943
structure: ChemicalStructure/PW C040543

3-ene-valproic acid
type: SmallMoleculeReference
xref: Reference/CAS 72010-19-6
xref: Reference/HMDB HMDB0061342
xref: Reference/ChemSpider 30791778
xref: Reference/PubChem-compound 24892812
xref: Reference/KEGG Compound C16654
chemical formula: C8H14O2
molecular weight: 142.09938
structure: ChemicalStructure/PW C040545

Reference/CAS 60113-83-9
type: UnificationXref
id: 60113-83-9
db: CAS

2-Propylglutaric acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0060684
xref: Reference/ChemSpider 118936
xref: Reference/CAS 32806-62-5
xref: Reference/KEGG Compound C16658
xref: Reference/PubChem-compound 134970
chemical formula: C8H14O4
molecular weight: 174.0892
structure: ChemicalStructure/PW C040548

Compound/PW C040545/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-ene-valproic acid
stoichiometric coefficient: 1.0

Reference/UniProt P11509
type: UnificationXref
id: P11509
db: UniProt

4-ene-Valproic acid
type: SmallMolecule
entity reference: 4-ene-Valproic acid

ChemicalStructure/PW C000746
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C000988
type: ChemicalStructure
structure format: SMILES

1.3.8.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C040715 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 C040713 Mitochondrial Matrix/Stoichiometry/1.0
right: 2-ene-Valproic acid CoA
right: NADH
spontaneous: false
EC number: 1.3.8.4
left: Valproic acid CoA
left: NAD
name: NAD + Valproic acid CoA → 2-ene-Valproic acid CoA + NADH
conversion direction: LEFT_TO_RIGHT

Reaction2321
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Peroxisome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040716 Peroxisome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040715 Peroxisome/Stoichiometry/1.0
right: 3-Hydroxyvalproic acid CoA
spontaneous: false
left: 2-ene-Valproic acid CoA
left: Water
name: 2-ene-Valproic acid CoA + Water → 3-Hydroxyvalproic acid CoA
conversion direction: LEFT_TO_RIGHT

Reaction2328
type: BiochemicalReaction
participant stoichiometry: Compound/PW C040715 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040717 Mitochondrion/Stoichiometry/1.0
right: 3-ene-Valproic acid CoA
spontaneous: false
left: 2-ene-Valproic acid CoA
name: 2-ene-Valproic acid CoA → 3-ene-Valproic acid CoA
conversion direction: LEFT_TO_RIGHT

Reaction2329
type: BiochemicalReaction
participant stoichiometry: Compound/PW C040719 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040717 Mitochondrion/Stoichiometry/1.0
right: 2,3-diene-Valproic acid-CoA
spontaneous: false
left: 3-ene-Valproic acid CoA
name: 3-ene-Valproic acid CoA → 2,3-diene-Valproic acid-CoA
conversion direction: LEFT_TO_RIGHT

ReactionCatalysis2231
type: Catalysis
controller: Trifunctional enzyme, mitochondrial
controlled: Reaction2324
control type: ACTIVATION

ReactionCatalysis2230
type: Catalysis
controller: 3-hydroxyacyl-CoA dehydrogenase type-2
controlled: Reaction2323
control type: ACTIVATION

Reaction2324
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000988 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040716 Mitochondrion/Stoichiometry/1.0
right: Propionyl-CoA
spontaneous: false
left: 3-Hydroxyvalproic acid CoA
name: 3-Hydroxyvalproic acid CoA → Propionyl-CoA
conversion direction: LEFT_TO_RIGHT

Reaction2325
type: BiochemicalReaction
participant stoichiometry: Compound/PW C040721 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040713 Mitochondrion/Stoichiometry/1.0
right: Valproyl-dephospho-CoA
spontaneous: false
left: Valproic acid CoA
name: Valproic acid CoA → Valproyl-dephospho-CoA
conversion direction: LEFT_TO_RIGHT

ReactionCatalysis2232
type: Catalysis
controller: Trifunctional enzyme, mitochondrial
controlled: Reaction2330
control type: ACTIVATION

Reaction2322
type: BiochemicalReaction
participant stoichiometry: Compound/PW C040720 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040714 Mitochondrial Matrix/Stoichiometry/1.0
right: 2,4-diene-Valproic acid-CoA
spontaneous: false
left: 4-ene-Valproic acid CoA
name: 4-ene-Valproic acid CoA → 2,4-diene-Valproic acid-CoA
conversion direction: LEFT_TO_RIGHT

NADP
type: SmallMolecule
cellular location: Endoplasmic Reticulum Membrane
entity reference: NADP

Reaction2323
type: BiochemicalReaction
participant stoichiometry: Compound/PW C040716 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 C040718 Mitochondrial Matrix/Stoichiometry/1.0
right: 3-oxo-Valproic acid CoA
right: NAD
spontaneous: false
left: 3-Hydroxyvalproic acid CoA
left: NADH
name: 3-Hydroxyvalproic acid CoA + NADH → 3-oxo-Valproic acid CoA + NAD
conversion direction: LEFT_TO_RIGHT

2-n-Propyl-4-oxopentanoic acid
type: SmallMolecule
entity reference: 2-n-Propyl-4-oxopentanoic acid

Reference/ChEBI CHEBI 17659
type: UnificationXref
id: CHEBI:17659
db: ChEBI

1.3.8.5
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001099 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040714 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C008639 Mitochondrial Matrix/Stoichiometry/1.0
right: 4-ene-Valproic acid CoA
spontaneous: false
EC number: 1.3.8.5
left: 4-ene-Valproic acid
left: Coenzyme A
name: 4-ene-Valproic acid + Coenzyme A → 4-ene-Valproic acid CoA
conversion direction: LEFT_TO_RIGHT

Reference/PubChem-compound 3121
type: UnificationXref
id: 3121
db: PubChem-compound

2-Propyl-2,4-pentadienoic acid
type: SmallMolecule
entity reference: 2-Propyl-2,4-pentadienoic acid

2.4.1.17
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001249 Endoplasmic Reticulum Lumen/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000720 Endoplasmic Reticulum Lumen/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000746 Endoplasmic Reticulum Lumen/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000201 Endoplasmic Reticulum Lumen/Stoichiometry/1.0
right: Uridine 5'-diphosphate
right: Valproic acid glucuronide
spontaneous: false
EC number: 2.4.1.17
left: Valproic acid
left: Uridine diphosphate glucuronic acid
name: Uridine diphosphate glucuronic acid + Valproic acid → Uridine 5'-diphosphate + Valproic acid glucuronide
conversion direction: LEFT_TO_RIGHT

Reference/KEGG Compound C07185
type: UnificationXref
id: C07185
db: KEGG Compound

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

6.2.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000032 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000170 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040713 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001249 Mitochondrial Matrix/Stoichiometry/1.0
right: Adenosine monophosphate
right: Valproic acid CoA
right: Pyrophosphate
spontaneous: false
EC number: 6.2.1.2
left: Coenzyme A
left: Valproic acid
left: Adenosine triphosphate
name: Adenosine triphosphate + Coenzyme A + Valproic acid → Adenosine monophosphate + Pyrophosphate + Valproic acid CoA
conversion direction: LEFT_TO_RIGHT