L-Valine
type: SmallMoleculeReference
xref: Reference/CAS 72-18-4
xref: Reference/PubChem-compound 6287
xref: Reference/ChemSpider 6050
xref: Reference/HMDB HMDB0000883
xref: Reference/KEGG Compound C00183
xref: Reference/ChEBI CHEBI 16414
xref: Reference/BioCyc VAL
chemical formula: C5H11NO2
molecular weight: 117.07898
structure: ChemicalStructure/PW C000704

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

Reference/ChemSpider 1005
type: UnificationXref
id: 1005
db: ChemSpider

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

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/PubChem-compound 439161
type: UnificationXref
id: 439161
db: PubChem-compound

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

Reference/ChemSpider 388353
type: UnificationXref
id: 388353
db: ChemSpider

Reaction1254
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001459 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002942 Mitochondrial Matrix/Stoichiometry/1.0
right: 3-Hydroxypropionyl-CoA
spontaneous: false
left: Malonyl-CoA semialdehyde
name: Malonyl-CoA semialdehyde ↔ 3-Hydroxypropionyl-CoA
conversion direction: REVERSIBLE

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

Reference/ChemSpider 57
type: UnificationXref
id: 57
db: ChemSpider

Reference/BioCyc D-METHYL-MALONYL-COA
type: UnificationXref
id: D-METHYL-MALONYL-COA
db: BioCyc

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

Propinol adenylate
type: SmallMolecule
cellular location: Cytosol
entity reference: Propinol adenylate

Reference/UniProt D4A197
type: UnificationXref
id: D4A197
db: UniProt

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

Reference/PathWhiz PW000054
type: UnificationXref
id: PW000054
db: PathWhiz

Reference/KEGG Compound C00683
type: UnificationXref
id: C00683
db: KEGG Compound

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

Reference/PathWhiz PW000051
type: UnificationXref
id: PW000051
db: PathWhiz

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

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

Protein/16464 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
stoichiometric coefficient: 1.0

Reference/PubChem-compound 6287
type: UnificationXref
id: 6287
db: PubChem-compound

Compound/PW C000704 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Valine
stoichiometric coefficient: 1.0

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

SubPathwayInteraction108656
type: Interaction
name: SubPathwayReaction
name: SubPathway108656Reaction
participant: Hydroxypropionic acid
participant: Propionic acid

Reference/HMDB HMDB0002170
type: UnificationXref
id: HMDB0002170
db: HMDB

SubPathwayInteraction108657
type: Interaction
name: SubPathway108657Reaction
name: SubPathwayReaction
participant: Propionyl-CoA

SubPathwayInteraction108658
type: Interaction
name: SubPathway108658Reaction
name: SubPathwayReaction
participant: β-Alanine

Reference/BioCyc HCO3
type: UnificationXref
id: HCO3
db: BioCyc

SubPathwayInteraction108659
type: Interaction
name: SubPathwayReaction
name: SubPathway108659Reaction
participant: 2-Ketobutyric acid

Reference/ChEBI CHEBI 15380
type: UnificationXref
id: CHEBI:15380
db: ChEBI

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

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

Succinyl-CoA
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00091
xref: Reference/CAS 604-98-8
xref: Reference/ChemSpider 388307
xref: Reference/PubChem-compound 439161
xref: Reference/ChEBI CHEBI 15380
xref: Reference/BioCyc 3-METHYLBENZYLSUCCINYL-COA
xref: Reference/HMDB HMDB0001022
chemical formula: C25H40N7O19P3S
molecular weight: 867.1312
structure: ChemicalStructure/PW C000808

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

Reference/KEGG Compound C00332
type: UnificationXref
id: C00332
db: KEGG Compound

Reference/BioCyc ALPHA-GLUCOSE-16-BISPHOSPHATE
type: UnificationXref
id: ALPHA-GLUCOSE-16-BISPHOSPHATE
db: BioCyc

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

Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/15179 Cytosol/Stoichiometry/4.0
cellular location: Cytosol
component: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial

SubPathwayInteraction108660
type: Interaction
name: SubPathway108660Reaction
name: SubPathwayReaction
participant: 2-Ketobutyric acid

Reference/ChemSpider 388468
type: UnificationXref
id: 388468
db: ChemSpider

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

Reference/PubChem-compound 11266
type: UnificationXref
id: 11266
db: PubChem-compound

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

Reference/KEGG Compound C00109
type: UnificationXref
id: C00109
db: KEGG Compound

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

Reference/CAS 5776-58-9
type: UnificationXref
id: 5776-58-9
db: CAS

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

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

ChemicalStructure/PW C001099
type: ChemicalStructure
structure format: SMILES

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

Reference/ChEBI CHEBI 30768
type: UnificationXref
id: CHEBI:30768
db: ChEBI

Rattus norvegicus
type: BioSource
xref: Reference/TAXONOMY 10116

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

Reference/UniProt Q6P6R2
type: UnificationXref
id: Q6P6R2
db: UniProt

SubPathwayInteraction108654
type: Interaction
name: SubPathwayReaction
name: SubPathway108654Reaction
participant: Acetyl-CoA

SubPathwayInteraction108655
type: Interaction
name: SubPathway108655Reaction
name: SubPathwayReaction
participant: Succinyl-CoA

Malonyl-CoA semialdehyde
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Malonyl-CoA semialdehyde

ChemicalStructure/PW C000808
type: ChemicalStructure
structure format: SMILES

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

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

Enoyl-CoA hydratase, mitochondrial
type: Complex
component stoichiometry: Protein/15144 Cytosol/Stoichiometry/6.0
cellular location: Cytosol
component: Enoyl-CoA hydratase, mitochondrial

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

Reaction147432
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001441 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000940 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000463 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000911 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001034 Cytosol/Stoichiometry/1.0
right: Adenosine diphosphate
right: Malonyl-CoA
right: Phosphoric acid
spontaneous: false
left: Adenosine triphosphate
left: Acetyl-CoA
left: Hydrogen carbonate
name: Acetyl-CoA + Adenosine triphosphate + Hydrogen carbonate → Adenosine diphosphate + Malonyl-CoA + Phosphoric acid
conversion direction: LEFT_TO_RIGHT

Reference/KEGG Compound C01213
type: UnificationXref
id: C01213
db: KEGG Compound

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

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

Reference/CAS 503-66-2
type: UnificationXref
id: 503-66-2
db: CAS

Reference/ChEBI CHEBI 5086
type: UnificationXref
id: CHEBI:5086
db: ChEBI

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

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

2-Hydroxybutyric acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 2-Hydroxybutyric acid

Reaction2266
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000911 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001459 Cytosol/Stoichiometry/1.0
right: Malonyl-CoA semialdehyde
spontaneous: false
left: Malonyl-CoA
name: Malonyl-CoA ↔ Malonyl-CoA semialdehyde
conversion direction: REVERSIBLE

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

Reference/CAS 73173-92-9
type: UnificationXref
id: 73173-92-9
db: CAS

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial

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

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

Reference/UniProt P07633
type: UnificationXref
id: P07633
db: UniProt

Protein/14853 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
stoichiometric coefficient: 1.0

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

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

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

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

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

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

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

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

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

Reaction1281
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000704 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001500 Mitochondrial Matrix/Stoichiometry/1.0
right: (S)-Methylmalonic acid semialdehyde
spontaneous: false
left: L-Valine
name: L-Valine → (S)-Methylmalonic acid semialdehyde
conversion direction: LEFT_TO_RIGHT

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

Acetyl-CoA carboxylase 1
type: Protein
cellular location: Cytosol
entity reference: Acetyl-CoA carboxylase 1

Reference/ChemSpider 389623
type: UnificationXref
id: 389623
db: ChemSpider

ReactionCatalysis138167
type: Catalysis
controller: Methylmalonyl-CoA mutase, mitochondrial
controlled: 5.4.99.2
control type: ACTIVATION

Coenzyme A
type: SmallMolecule
cellular location: Cytosol
entity reference: Coenzyme A

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

ReactionCatalysis138169
type: Catalysis
controller: Enoyl-CoA hydratase, mitochondrial
controlled: 4.2.1.17
control type: ACTIVATION

Reference/CAS 73173-91-8
type: UnificationXref
id: 73173-91-8
db: CAS

ReactionCatalysis138168
type: Catalysis
controller: Malonyl-CoA decarboxylase, mitochondrial
controlled: 4.1.1.9
control type: ACTIVATION

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

Propionyl-CoA carboxylase alpha chain, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: Propionyl-CoA carboxylase alpha chain, mitochondrial

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

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

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/UniProt P08503
type: UnificationXref
id: P08503
db: UniProt

Reference/ChemSpider 388307
type: UnificationXref
id: 388307
db: ChemSpider

ReactionCatalysis138178
type: Catalysis
controller: L-lactate dehydrogenase A chain
controlled: 1.1.1.27
control type: ACTIVATION

ReactionCatalysis138177
type: Catalysis
controller: branched-chain alpha-keto dehydrogenase complex
controlled: 2.3.1.168
control type: ACTIVATION

Reference/PubChem-compound 22833590
type: UnificationXref
id: 22833590
db: PubChem-compound

Malonyl-CoA decarboxylase, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: Malonyl-CoA decarboxylase, mitochondrial

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

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

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

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

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

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

ChemicalStructure/PW C000911
type: ChemicalStructure
structure format: SMILES

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

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

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

Propionyl-CoA carboxylase
type: Complex
component stoichiometry: Protein/16501 Cytosol/Stoichiometry/1.0
component stoichiometry: Protein/16502 Cytosol/Stoichiometry/1.0
cellular location: Cytosol
component: Propionyl-CoA carboxylase beta chain, mitochondrial
component: Propionyl-CoA carboxylase alpha chain, mitochondrial

Compound/PW C002942 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxypropionyl-CoA
stoichiometric coefficient: 1.0

ReactionCatalysis138174
type: Catalysis
controller: Acyl-CoA synthetase short-chain family member 3
controlled: 6.2.1.1
control type: ACTIVATION

ReactionCatalysis138173
type: Catalysis
controller: Acyl-CoA synthetase short-chain family member 3
controlled: 6.2.1.1
control type: ACTIVATION

ReactionCatalysis138176
type: Catalysis
controller: Propionyl-CoA carboxylase
controlled: 6.4.1.3
control type: ACTIVATION

ReactionCatalysis138175
type: Catalysis
controller: Acetyl-coenzyme A synthetase
controlled: 6.2.1.1
control type: ACTIVATION

ReactionCatalysis138170
type: Catalysis
controller: 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
controlled: 3.1.2.4
control type: ACTIVATION

ReactionCatalysis138172
type: Catalysis
controller: Acetyl-coenzyme A synthetase
controlled: 6.2.1.1
control type: ACTIVATION

ReactionCatalysis138171
type: Catalysis
controller: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.7
control type: ACTIVATION

Acrylyl-CoA
type: SmallMolecule
cellular location: Cytosol
entity reference: Acrylyl-CoA

Reference/PubChem-compound 5462303
type: UnificationXref
id: 5462303
db: PubChem-compound

Reference/PubChem-compound 769
type: UnificationXref
id: 769
db: PubChem-compound

Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P08503
organism: Rattus norvegicus

Reference/ChEBI CHEBI 18276
type: UnificationXref
id: CHEBI:18276
db: ChEBI

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

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

ChemicalStructure/PW C001142
type: ChemicalStructure
structure format: SMILES

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

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

Compound/PW C001560 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-Methylmalonyl-CoA
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00282
type: UnificationXref
id: C00282
db: KEGG Compound

Hydroxypropionic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 68152
xref: Reference/ChemSpider 61460
xref: Reference/ChEBI CHEBI 33404
xref: Reference/HMDB HMDB0000700
xref: Reference/KEGG Compound C01013
xref: Reference/CAS 503-66-2
chemical formula: C3H6O3
molecular weight: 90.03169
structure: ChemicalStructure/PW C000552

Reference/HMDB HMDB0002310
type: UnificationXref
id: HMDB0002310
db: HMDB

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

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

ChemicalStructure/PW C000170
type: ChemicalStructure
structure format: SMILES
structure data: [O-]P([O-])(=O)OP([O-])([O-])=O

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

Compound/PW C000463 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydrogen carbonate
stoichiometric coefficient: 1.0

3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial

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

Methylmalonyl CoA epimerase
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Methylmalonyl CoA epimerase

Acetyl-CoA acetyltransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P17764
organism: Rattus norvegicus

Reference/KEGG Compound C01013
type: UnificationXref
id: C01013
db: KEGG Compound

R-Methylmalonyl-CoA
type: SmallMoleculeReference
xref: Reference/BioCyc METHYL-MALONYL-COA
xref: Reference/PubChem-compound 22833590
xref: Reference/ChemSpider 17216177
xref: Reference/CAS 73173-92-9
xref: Reference/KEGG Compound C01213
xref: Reference/HMDB HMDB0002255
chemical formula: C25H40N7O19P3S
molecular weight: 867.1312
structure: ChemicalStructure/PW C001523

Pantetheine
type: SmallMoleculeReference
xref: Reference/BioCyc PANTETHEINE-P
xref: Reference/PubChem-compound 479
xref: Reference/ChemSpider 466
xref: Reference/CAS 496-65-1
xref: Reference/KEGG Compound C00831
xref: Reference/ChEBI CHEBI 16753
xref: Reference/HMDB HMDB0003426
chemical formula: C11H22N2O4S
molecular weight: 278.13004
structure: ChemicalStructure/PW C001887

Reference/KEGG Compound C00163
type: UnificationXref
id: C00163
db: KEGG Compound

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

Reference/KEGG Compound C00288
type: UnificationXref
id: C00288
db: KEGG Compound

ChemicalStructure/PW C001034
type: ChemicalStructure
structure format: SMILES

Methylmalonyl CoA mutase
type: Protein
cellular location: Mitochondrion
entity reference: Methylmalonyl CoA mutase

(S)-Methylmalonic acid semialdehyde
type: SmallMolecule
cellular location: Cytosol
entity reference: (S)-Methylmalonic acid semialdehyde

Reference/CAS 604-98-8
type: UnificationXref
id: 604-98-8
db: CAS

Reference/HMDB HMDB0006806
type: UnificationXref
id: HMDB0006806
db: HMDB

Reference/ChemSpider 762
type: UnificationXref
id: 762
db: ChemSpider

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

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

Acyl-CoA synthetase short-chain family member 3
type: Protein
cellular location: Cytosol
entity reference: Acyl-CoA synthetase short-chain family member 3

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

Reference/ChemSpider 389700
type: UnificationXref
id: 389700
db: ChemSpider

Succinyl-CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Succinyl-CoA
name: SubPathwayOutput

Reaction147496
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001099 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000988 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000463 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001500 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Cytosol/Stoichiometry/1.0
right: Propionyl-CoA
right: NADH
right: Hydrogen carbonate
spontaneous: false
left: NAD
left: (S)-Methylmalonic acid semialdehyde
left: Coenzyme A
left: Water
name: (S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD + Water → Hydrogen carbonate + NADH + Propionyl-CoA
conversion direction: LEFT_TO_RIGHT

5.1.99.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001523 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001560 Mitochondrial Matrix/Stoichiometry/1.0
right: R-Methylmalonyl-CoA
spontaneous: false
EC number: 5.1.99.1
left: S-Methylmalonyl-CoA
name: S-Methylmalonyl-CoA → R-Methylmalonyl-CoA
conversion direction: LEFT_TO_RIGHT

Reference/ChemSpider 389702
type: UnificationXref
id: 389702
db: ChemSpider

Propinol adenylate
type: SmallMoleculeReference
xref: Reference/ChemSpider 389700
xref: Reference/HMDB HMDB0006806
xref: Reference/ChEBI CHEBI 62415
xref: Reference/KEGG Compound C05983
xref: Reference/PubChem-compound 440863
chemical formula: C13H18N5O8P
molecular weight: 403.0893
structure: ChemicalStructure/PW C002941

3-Hydroxypropionyl-CoA
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 27762
xref: Reference/ChemSpider 389623
xref: Reference/KEGG Compound C05668
xref: Reference/BioCyc 3-HYDROXY-PROPIONYL-COA
xref: Reference/HMDB HMDB0006807
xref: Reference/CAS 157786-88-4
xref: Reference/PubChem-compound 440753
chemical formula: C24H40N7O18P3S
molecular weight: 839.13635
structure: ChemicalStructure/PW C002942

Reference/KEGG Compound C05989
type: UnificationXref
id: C05989
db: KEGG Compound

Reference/HMDB HMDB0006807
type: UnificationXref
id: HMDB0006807
db: HMDB

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

Reference/KEGG Compound C05983
type: UnificationXref
id: C05983
db: KEGG Compound

Reference/KEGG Compound C05984
type: UnificationXref
id: C05984
db: KEGG Compound

Reference/CAS 72-18-4
type: UnificationXref
id: 72-18-4
db: CAS

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

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

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

ChemicalStructure/PW C000032
type: ChemicalStructure
structure format: SMILES

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

Reference/KEGG Compound C00183
type: UnificationXref
id: C00183
db: KEGG Compound

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

Reference/ChEBI CHEBI 33404
type: UnificationXref
id: CHEBI:33404
db: ChEBI

Reference/HMDB HMDB0003426
type: UnificationXref
id: HMDB0003426
db: HMDB

Reference/HMDB HMDB0002217
type: UnificationXref
id: HMDB0002217
db: HMDB

Reference/UniProt P17764
type: UnificationXref
id: P17764
db: UniProt

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 HMDB0001362
type: UnificationXref
id: HMDB0001362
db: HMDB

Reference/HMDB HMDB0001484
type: UnificationXref
id: HMDB0001484
db: HMDB

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

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

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

(S)-Methylmalonic acid semialdehyde
type: SmallMoleculeReference
xref: Reference/KEGG Compound C06002
xref: Reference/ChEBI CHEBI 27821
xref: Reference/PubChem-compound 5462303
xref: Reference/HMDB HMDB0002217
xref: Reference/ChemSpider 4575365
xref: Reference/CAS 99043-16-0
chemical formula: C4H6O3
molecular weight: 102.03169
structure: ChemicalStructure/PW C001500

Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial

ChemicalStructure/PW C000159
type: ChemicalStructure
structure format: SMILES
structure data: CCC(O)=O

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

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

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

S-Methylmalonyl-CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: S-Methylmalonyl-CoA

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

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

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

L-lactate dehydrogenase A chain
type: ProteinReference
xref: Reference/UniProt P04642
organism: Rattus norvegicus

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

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

Acrylyl-CoA
type: SmallMoleculeReference
xref: Reference/ChemSpider 388468
xref: Reference/BioCyc ACRYLYL-COA
xref: Reference/ChEBI CHEBI 15513
xref: Reference/HMDB HMDB0002307
xref: Reference/PubChem-compound 439340
xref: Reference/CAS 5776-58-9
xref: Reference/KEGG Compound C00894
chemical formula: C24H38N7O17P3S
molecular weight: 821.1258
structure: ChemicalStructure/PW C001558

Reference/UniProt P97902
type: UnificationXref
id: P97902
db: UniProt

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

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

S-Methylmalonyl-CoA
type: SmallMoleculeReference
xref: Reference/CAS 73173-91-8
xref: Reference/KEGG Compound C00683
xref: Reference/BioCyc D-METHYL-MALONYL-COA
xref: Reference/ChemSpider 13628334
xref: Reference/HMDB HMDB0002310
xref: Reference/PubChem-compound 21252287
chemical formula: C25H40N7O19P3S
molecular weight: 867.1312
structure: ChemicalStructure/PW C001560

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

Compound/PW C000940 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Acetyl-CoA
stoichiometric coefficient: 2.0

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

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

Compound/Generic70 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Reduced electron-transfer flavoprotein
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00083
type: UnificationXref
id: C00083
db: KEGG Compound

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

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
type: ProteinReference
xref: Reference/UniProt P35738
organism: Rattus norvegicus

Reference/BioCyc 3-HYDROXY-PROPIONYL-COA
type: UnificationXref
id: 3-HYDROXY-PROPIONYL-COA
db: BioCyc

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
type: ProteinReference
xref: Reference/UniProt P11960
organism: Rattus norvegicus

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

Compound/PW C001887 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pantetheine
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0001022
type: UnificationXref
id: HMDB0001022
db: HMDB

Reference/UniProt P04642
type: UnificationXref
id: P04642
db: UniProt

Enoyl-CoA hydratase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P14604
organism: Rattus norvegicus

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

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

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

Reference/CAS 99043-16-0
type: UnificationXref
id: 99043-16-0
db: CAS

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

Protein/16466 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
stoichiometric coefficient: 1.0

2-Ketobutyric acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 2-Ketobutyric acid

2-Ketobutyric acid
type: SmallMolecule
entity reference: 2-Ketobutyric acid
name: SubPathwayOutput

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

Reference/KEGG Compound C00091
type: UnificationXref
id: C00091
db: KEGG Compound

Reference/BioCyc METHYL-MALONYL-COA
type: UnificationXref
id: METHYL-MALONYL-COA
db: BioCyc

2-Ketobutyric acid
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 30831
xref: Reference/HMDB HMDB0000005
xref: Reference/CAS 600-18-0
xref: Reference/ChemSpider 57
xref: Reference/KEGG Compound C00109
xref: Reference/PubChem-compound 58
xref: Reference/BioCyc 2-OXOBUTANOATE
chemical formula: C4H6O3
molecular weight: 102.03169
structure: ChemicalStructure/PW C000003

Compound/PW C000004 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Hydroxybutyric acid
stoichiometric coefficient: 1.0

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

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

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

Compound/PW C001459 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Malonyl-CoA semialdehyde
stoichiometric coefficient: 1.0

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

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

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

Reference/ChemSpider 4575365
type: UnificationXref
id: 4575365
db: ChemSpider

Reference/KEGG Compound C05668
type: UnificationXref
id: C05668
db: KEGG Compound

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

Reference/PubChem-compound 10663
type: UnificationXref
id: 10663
db: PubChem-compound

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

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

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

Compound/PW C000911 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Malonyl-CoA
stoichiometric coefficient: 1.0

Acetyl-CoA carboxylase 1
type: ProteinReference
xref: Reference/UniProt P97902
organism: Rattus norvegicus

(S)-Methylmalonic acid semialdehyde
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: (S)-Methylmalonic acid semialdehyde

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

Malonyl-CoA decarboxylase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q9WUY2
organism: Rattus norvegicus

Reference/HMDB HMDB0002255
type: UnificationXref
id: HMDB0002255
db: HMDB

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

Reference/BioCyc CPD-3564
type: UnificationXref
id: CPD-3564
db: BioCyc

Malonyl-CoA semialdehyde
type: SmallMolecule
cellular location: Cytosol
entity reference: Malonyl-CoA semialdehyde

Reference/UniProt Q9WUY2
type: UnificationXref
id: Q9WUY2
db: UniProt

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

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

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

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

Reference/ChemSpider 749
type: UnificationXref
id: 749
db: ChemSpider

Reference/CAS 71-52-3
type: UnificationXref
id: 71-52-3
db: CAS

Compound/PW C001560 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-Methylmalonyl-CoA
stoichiometric coefficient: 1.0

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

3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q5XIE6
organism: Rattus norvegicus

Reference/ChemSpider 61460
type: UnificationXref
id: 61460
db: ChemSpider

Reference/KEGG Compound C02170
type: UnificationXref
id: C02170
db: KEGG Compound

Acyl-CoA synthetase short-chain family member 3
type: ProteinReference
organism: Rattus norvegicus

Hydrogen carbonate
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000595
xref: Reference/ChEBI CHEBI 17544
xref: Reference/PubChem-compound 769
xref: Reference/BioCyc HCO3
xref: Reference/ChemSpider 749
xref: Reference/CAS 71-52-3
xref: Reference/KEGG Compound C00288
chemical formula: CHO3
molecular weight: 60.99257
structure: ChemicalStructure/PW C000463

Reference/HMDB HMDB0001175
type: UnificationXref
id: HMDB0001175
db: HMDB

ChemicalStructure/PW C001051
type: ChemicalStructure
structure format: SMILES
structure data: [H][H]

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

Reference/PubChem-compound 487
type: UnificationXref
id: 487
db: PubChem-compound

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

Protein/15144 Cytosol/Stoichiometry/6.0
type: Stoichiometry
physical entity: Enoyl-CoA hydratase, mitochondrial
stoichiometric coefficient: 6.0

S-Methylmalonyl-CoA
type: SmallMolecule
cellular location: Cytosol
entity reference: S-Methylmalonyl-CoA

Valine, Leucine, and Isoleucine Degradation
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108657
pathway component: SubPathwayInteraction108657
xref: Reference/SMPDB SMP0000032
xref: Reference/PathWhiz PW000051
name: SubPathway

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

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

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

Propionyl-CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Propionyl-CoA
name: SubPathwayInput

Acetyl-CoA
type: SmallMolecule
cellular location: Cytosol
entity reference: Acetyl-CoA

Adenosine monophosphate
type: SmallMoleculeReference
xref: Reference/CAS 61-19-8
xref: Reference/ChemSpider 5858
xref: Reference/HMDB HMDB0000045
xref: Reference/BioCyc AMP
xref: Reference/ChEBI CHEBI 16027
xref: Reference/PubChem-compound 6083
xref: Reference/KEGG Compound C00020
chemical formula: C10H14N5O7P
molecular weight: 347.06308
structure: ChemicalStructure/PW C000032

Reference/PubChem-compound 58838673
type: UnificationXref
id: 58838673
db: PubChem-compound

Reference/PubChem-compound 479
type: UnificationXref
id: 479
db: PubChem-compound

Compound/PW C001142 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Acetoacetyl-CoA
stoichiometric coefficient: 1.0

Propionic acid
type: SmallMoleculeReference
xref: Reference/BioCyc PROPIONATE
xref: Reference/ChemSpider 1005
xref: Reference/HMDB HMDB0000237
xref: Reference/ChEBI CHEBI 30768
xref: Reference/CAS 79-09-4
xref: Reference/KEGG Compound C00163
xref: Reference/PubChem-compound 1032
chemical formula: C3H6O2
molecular weight: 74.03678
structure: ChemicalStructure/PW C000159

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

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

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

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

Compound/PW C001459 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Malonyl-CoA semialdehyde
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 27821
type: UnificationXref
id: CHEBI:27821
db: ChEBI

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

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

Compound/PW C000552 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydroxypropionic acid
stoichiometric coefficient: 1.0

Reference/UniProt P14882
type: UnificationXref
id: P14882
db: UniProt

ChemicalStructure/PW C000463
type: ChemicalStructure
structure format: SMILES
structure data: OC([O-])=O

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

2.3.1.9
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000940 Mitochondrion/Stoichiometry/2.0
participant stoichiometry: Compound/PW C001142 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
right: Acetoacetyl-CoA
right: Coenzyme A
spontaneous: false
EC number: 2.3.1.9
left: Acetyl-CoA
name: 2 Acetyl-CoA ↔ Acetoacetyl-CoA + Coenzyme A
conversion direction: REVERSIBLE

Compound/PW C000003 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Ketobutyric acid
stoichiometric coefficient: 1.0

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

Reference/PubChem-compound 58
type: UnificationXref
id: 58
db: PubChem-compound

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

Malonyl-CoA
type: SmallMolecule
cellular location: Cytosol
entity reference: Malonyl-CoA

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

ReactionCatalysis137674
type: Catalysis
controller: Acetyl-CoA carboxylase 1
controlled: Reaction147432
control type: ACTIVATION

2-Hydroxybutyric acid
type: SmallMoleculeReference
xref: Reference/BioCyc CPD-3564
xref: Reference/PubChem-compound 11266
xref: Reference/CAS 600-15-7
xref: Reference/HMDB HMDB0000008
xref: Reference/ChemSpider 10792
xref: Reference/ChEBI CHEBI 1148
xref: Reference/KEGG Compound C05984
chemical formula: C4H8O3
molecular weight: 104.04734
structure: ChemicalStructure/PW C000004

Malonyl-CoA semialdehyde
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0002170
xref: Reference/ChemSpider 389702
xref: Reference/CAS 6244-93-5
xref: Reference/KEGG Compound C05989
xref: Reference/PubChem-compound 440865
chemical formula: C24H38N7O18P3S
molecular weight: 837.12067
structure: ChemicalStructure/PW C001459

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

Reference/PubChem-compound 1032
type: UnificationXref
id: 1032
db: PubChem-compound

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

ChemicalStructure/PW C000552
type: ChemicalStructure
structure format: SMILES
structure data: OCCC(O)=O

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

Protein/15187 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Acetyl-CoA acetyltransferase, mitochondrial
stoichiometric coefficient: 4.0

Compound/PW C002942 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxypropionyl-CoA
stoichiometric coefficient: 1.0

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

Methylmalonic acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000202
xref: Reference/PubChem-compound 487
xref: Reference/CAS 516-05-2
xref: Reference/KEGG Compound C02170
xref: Reference/ChemSpider 473
xref: Reference/ChEBI CHEBI 30860
chemical formula: C4H6O4
molecular weight: 118.02661
structure: ChemicalStructure/PW C000130

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

Citric Acid Cycle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108655
pathway component: SubPathwayInteraction108655
xref: Reference/PathWhiz PW000005
xref: Reference/SMPDB SMP0000057
name: SubPathway

Compound/PW C001558 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Acrylyl-CoA
stoichiometric coefficient: 1.0

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

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

Protein/16502 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Propionyl-CoA carboxylase beta chain, mitochondrial
stoichiometric coefficient: 1.0

ChemicalStructure/PW C001887
type: ChemicalStructure
structure format: SMILES
structure data: CC(C)(CO)C(O)C(=O)NCCC(=O)NCCS

ChemicalStructure/PW C001523
type: ChemicalStructure
structure format: SMILES

Reference/PathWhiz PW122009
type: UnificationXref
id: PW122009
db: PathWhiz

Reference/HMDB HMDB0000202
type: UnificationXref
id: HMDB0000202
db: HMDB

Reference/CAS 600-18-0
type: UnificationXref
id: 600-18-0
db: CAS

Pyruvate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108654
pathway component: SubPathwayInteraction108654
xref: Reference/SMPDB SMP0000060
xref: Reference/PathWhiz PW000054
name: SubPathway

Protein/16501 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Propionyl-CoA carboxylase alpha chain, mitochondrial
stoichiometric coefficient: 1.0

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

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

Methylmalonyl CoA epimerase
type: ProteinReference
xref: Reference/UniProt D4A197
organism: Rattus norvegicus

Acetyl-coenzyme A synthetase
type: ProteinReference
xref: Reference/UniProt D3ZZN3
organism: Rattus norvegicus

Reference/UniProt Q5XIE6
type: UnificationXref
id: Q5XIE6
db: UniProt

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

ChemicalStructure/PW C001459
type: ChemicalStructure
structure format: SMILES

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

Compound/PW C000130 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Methylmalonic acid
stoichiometric coefficient: 1.0

Reference/PubChem-compound 440865
type: UnificationXref
id: 440865
db: PubChem-compound

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

Reference/PubChem-compound 440863
type: UnificationXref
id: 440863
db: PubChem-compound

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

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

ChemicalStructure/PW C000130
type: ChemicalStructure
structure format: SMILES
structure data: CC(C(O)=O)C(O)=O

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

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

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

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

Compound/PW C001051 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydrogen
stoichiometric coefficient: 1.0

Propionyl-CoA carboxylase alpha chain, mitochondrial
type: ProteinReference
xref: Reference/UniProt P14882
organism: Rattus norvegicus

Propionyl-CoA carboxylase beta chain, mitochondrial
type: ProteinReference
xref: Reference/UniProt P07633
organism: Rattus norvegicus

Reference/ChEBI CHEBI 15531
type: UnificationXref
id: CHEBI:15531
db: ChEBI

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

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

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

Malonic semialdehyde
type: SmallMolecule
cellular location: Cytosol
entity reference: Malonic semialdehyde

Reference/UniProt P14604
type: UnificationXref
id: P14604
db: UniProt

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

Reference/ChemSpider 6050
type: UnificationXref
id: 6050
db: ChemSpider

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

Protein/16510 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Methylmalonyl CoA mutase
stoichiometric coefficient: 2.0

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

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

Methylmalonyl CoA mutase
type: ProteinReference
xref: Reference/UniProt D3ZKG1
organism: Rattus norvegicus

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

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

Hydrogen carbonate
type: SmallMolecule
cellular location: Cytosol
entity reference: Hydrogen carbonate

Hydrogen
type: SmallMoleculeReference
xref: Reference/PubChem-compound 58838673
xref: Reference/CAS 1333-74-0
xref: Reference/ChemSpider 762
xref: Reference/BioCyc ALPHA-GLUCOSE-16-BISPHOSPHATE
xref: Reference/KEGG Compound C00282
xref: Reference/HMDB HMDB0001362
xref: Reference/ChEBI CHEBI 18276
chemical formula: H2
molecular weight: 2.01565
structure: ChemicalStructure/PW C001051

ReactionCatalysis137752
type: Catalysis
controller: Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
controlled: Reaction147496
control type: ACTIVATION

ReactionCatalysis137755
type: Catalysis
controller: Methylmalonyl CoA epimerase
controlled: 5.1.99.1
control type: ACTIVATION

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

Cysteine and Methionine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108659
pathway component: SubPathwayInteraction108659
name: SubPathway

Intestinal Microflora
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108656
pathway component: SubPathwayInteraction108656
name: SubPathway

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

ChemicalStructure/PW C001558
type: ChemicalStructure
structure format: SMILES

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

Reference/KEGG Compound C06002
type: UnificationXref
id: C06002
db: KEGG Compound

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

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

Acetyl-CoA acetyltransferase, mitochondrial
type: Complex
component stoichiometry: Protein/15187 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: Acetyl-CoA acetyltransferase, mitochondrial

Reference/HMDB HMDB0000595
type: UnificationXref
id: HMDB0000595
db: HMDB

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

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: O7P2
molecular weight: 173.91193
structure: ChemicalStructure/PW C000170

Glycine, Serine, and Threonine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108660
pathway component: SubPathwayInteraction108660
name: SubPathway

ChemicalStructure/PW C001560
type: ChemicalStructure
structure format: SMILES

Acetoacetyl-CoA
type: SmallMoleculeReference
xref: Reference/BioCyc ACETOACETYL-COA
xref: Reference/PubChem-compound 439214
xref: Reference/ChEBI CHEBI 15345
xref: Reference/CAS 1420-36-6
xref: Reference/HMDB HMDB0001484
xref: Reference/KEGG Compound C00332
xref: Reference/ChemSpider 388353
chemical formula: C25H40N7O18P3S
molecular weight: 851.13635
structure: ChemicalStructure/PW C001142

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

Reference/CAS 496-65-1
type: UnificationXref
id: 496-65-1
db: CAS

Reference/SMPDB SMP0000060
type: UnificationXref
id: SMP0000060
db: SMPDB

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

Reaction147008
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000134 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000040 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C006753 Cytosol/Stoichiometry/1.0
right: L-Glutamic acid
right: Malonic semialdehyde
spontaneous: false
left: Oxoglutaric acid
left: β-Alanine
name: Oxoglutaric acid + β-Alanine → L-Glutamic acid + Malonic semialdehyde
conversion direction: LEFT_TO_RIGHT

Reference/HMDB HMDB0000237
type: UnificationXref
id: HMDB0000237
db: HMDB

Reference/ChemSpider 473
type: UnificationXref
id: 473
db: ChemSpider

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

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

Reference/ChEBI CHEBI 1148
type: UnificationXref
id: CHEBI:1148
db: ChEBI

Compound/PW C001523 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: R-Methylmalonyl-CoA
stoichiometric coefficient: 1.0

3-Hydroxypropionyl-CoA
type: SmallMolecule
cellular location: Cytosol
entity reference: 3-Hydroxypropionyl-CoA

Reference/ChEBI CHEBI 15513
type: UnificationXref
id: CHEBI:15513
db: ChEBI

Compound/PW C000159 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Propionic acid
stoichiometric coefficient: 1.0

Compound/PW C001500 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: (S)-Methylmalonic acid semialdehyde
stoichiometric coefficient: 1.0

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

5.4.99.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000808 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001523 Mitochondrion/Stoichiometry/1.0
right: Succinyl-CoA
spontaneous: false
EC number: 5.4.99.2
left: R-Methylmalonyl-CoA
name: R-Methylmalonyl-CoA ↔ Succinyl-CoA
conversion direction: REVERSIBLE

4.1.1.9
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000940 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000911 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Cytosol/Stoichiometry/1.0
right: Carbon dioxide
right: Acetyl-CoA
spontaneous: false
EC number: 4.1.1.9
left: Malonyl-CoA
name: Malonyl-CoA → Acetyl-CoA + Carbon dioxide
conversion direction: LEFT_TO_RIGHT

Reference/UniProt D3ZZN3
type: UnificationXref
id: D3ZZN3
db: UniProt

Reference/PubChem-compound 440753
type: UnificationXref
id: 440753
db: PubChem-compound

4.2.1.17
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001558 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002942 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
right: Acrylyl-CoA
right: Water
spontaneous: false
EC number: 4.2.1.17
left: 3-Hydroxypropionyl-CoA
name: 3-Hydroxypropionyl-CoA ↔ Acrylyl-CoA + Water
conversion direction: REVERSIBLE

3.1.2.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001099 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000552 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002942 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
right: Coenzyme A
right: Hydroxypropionic acid
spontaneous: false
EC number: 3.1.2.4
left: 3-Hydroxypropionyl-CoA
left: Water
name: 3-Hydroxypropionyl-CoA + Water ↔ Coenzyme A + Hydroxypropionic acid
conversion direction: REVERSIBLE

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

1.3.8.7
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001558 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/Generic70 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000988 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/Generic69 Cytosol/Stoichiometry/1.0
right: Propionyl-CoA
right: Reduced electron-transfer flavoprotein
spontaneous: false
EC number: 1.3.8.7
left: Acrylyl-CoA
left: electron-transfer flavoprotein
name: Acrylyl-CoA + electron-transfer flavoprotein ↔ Propionyl-CoA + Reduced electron-transfer flavoprotein
conversion direction: REVERSIBLE

6.2.1.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000414 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000988 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002941 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000032 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001887 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000170 Cytosol/Stoichiometry/1.0
right: Propinol adenylate
right: Adenosine triphosphate
right: Pantetheine
spontaneous: false
EC number: 6.2.1.1
left: Adenosine monophosphate
left: Propionyl-CoA
left: Pyrophosphate
name: Adenosine monophosphate + Propionyl-CoA + Pyrophosphate ↔ Adenosine triphosphate + Pantetheine + Propinol adenylate
conversion direction: REVERSIBLE

Reference/ChemSpider 466
type: UnificationXref
id: 466
db: ChemSpider

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

Compound/PW C000808 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Succinyl-CoA
stoichiometric coefficient: 1.0

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

6.2.1.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000414 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000988 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002941 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001887 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000032 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000170 Cytosol/Stoichiometry/1.0
right: Adenosine monophosphate
right: Propionyl-CoA
right: Pyrophosphate
spontaneous: false
EC number: 6.2.1.1
left: Propinol adenylate
left: Adenosine triphosphate
left: Pantetheine
name: Adenosine triphosphate + Pantetheine + Propinol adenylate ↔ Adenosine monophosphate + Propionyl-CoA + Pyrophosphate
conversion direction: REVERSIBLE

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

6.2.1.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002941 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000159 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000032 Cytosol/Stoichiometry/1.0
right: Adenosine monophosphate
right: Propionic acid
spontaneous: false
EC number: 6.2.1.1
left: Propinol adenylate
name: Propinol adenylate ↔ Adenosine monophosphate + Propionic acid
conversion direction: REVERSIBLE

ChemicalStructure/PW C000004
type: ChemicalStructure
structure format: SMILES
structure data: CCC(O)C(O)=O

Reference/HMDB HMDB0002307
type: UnificationXref
id: HMDB0002307
db: HMDB

6.2.1.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002941 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000159 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000032 Cytosol/Stoichiometry/1.0
right: Adenosine monophosphate
right: Propionic acid
spontaneous: false
EC number: 6.2.1.1
left: Propinol adenylate
name: Propinol adenylate ↔ Adenosine monophosphate + Propionic acid
conversion direction: REVERSIBLE

Reference/HMDB HMDB0000005
type: UnificationXref
id: HMDB0000005
db: HMDB

ChemicalStructure/PW C000003
type: ChemicalStructure
structure format: SMILES
structure data: CCC(=O)C(O)=O

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

Reference/HMDB HMDB0000008
type: UnificationXref
id: HMDB0000008
db: HMDB

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

Reference/CAS 79-09-4
type: UnificationXref
id: 79-09-4
db: CAS

Methylmalonic Aciduria Due to Cobalamin-Related Disorders
type: Pathway
pathway order: PathwayEnzymeReaction/2114982
pathway order: PathwayEnzymeReaction/2114981
pathway order: PathwayEnzymeReaction/2114980
pathway order: PathwayEnzymeReaction/2114986
pathway order: PathwayEnzymeReaction/2114985
pathway order: PathwayEnzymeReaction/2114984
pathway order: PathwayEnzymeReaction/2114983
pathway order: PathwayEnzymeReaction/2114968
pathway order: PathwaySubPathway/108657
pathway order: PathwayEnzymeReaction/2114967
pathway order: PathwaySubPathway/108658
pathway order: PathwayEnzymeReaction/2114966
pathway order: PathwaySubPathway/108659
pathway order: PathwayEnzymeReaction/2114965
pathway order: PathwaySubPathway/108654
pathway order: PathwaySubPathway/108655
pathway order: PathwayEnzymeReaction/2114969
pathway order: PathwaySubPathway/108656
pathway order: PathwayEnzymeReaction/2114971
pathway order: PathwaySubPathway/108660
pathway order: PathwayEnzymeReaction/2114970
pathway order: PathwayEnzymeReaction/2114975
pathway order: PathwayEnzymeReaction/2114974
pathway order: PathwayEnzymeReaction/2114973
pathway order: PathwayEnzymeReaction/2114972
pathway order: PathwayEnzymeReaction/2114979
pathway order: PathwayEnzymeReaction/2114978
pathway order: PathwayEnzymeReaction/2114977
pathway order: PathwayEnzymeReaction/2114976
pathway component: ReactionCatalysis137752
pathway component: ReactionCatalysis137755
pathway component: ReactionCatalysis138167
pathway component: Reaction147496
pathway component: 5.1.99.1
pathway component: Reaction147432
pathway component: ReactionCatalysis138169
pathway component: ReactionCatalysis138168
pathway component: ReactionCatalysis137674
pathway component: ReactionCatalysis137256
pathway component: Cysteine and Methionine Metabolism
pathway component: Intestinal Microflora
pathway component: 6.4.1.3
pathway component: 2.3.1.168
pathway component: 1.1.1.27
pathway component: Reaction1254
pathway component: Reaction2266
pathway component: Glycine, Serine, and Threonine Metabolism
pathway component: Valine, Leucine, and Isoleucine Degradation
pathway component: Reaction147008
pathway component: ReactionCatalysis137172
pathway component: Citric Acid Cycle
pathway component: ReactionCatalysis138178
pathway component: ReactionCatalysis138177
pathway component: ReactionCatalysis137322
pathway component: Reaction147501
pathway component: 5.4.99.2
pathway component: 4.1.1.9
pathway component: 4.2.1.17
pathway component: 3.1.2.4
pathway component: 1.3.8.7
pathway component: 6.2.1.1
pathway component: Reaction147116
pathway component: Reaction1281
pathway component: Pyruvate Metabolism
pathway component: ReactionCatalysis138174
pathway component: ReactionCatalysis138173
pathway component: 2.3.1.9
pathway component: ReactionCatalysis138176
pathway component: ReactionCatalysis138175
pathway component: ReactionCatalysis138170
pathway component: Pantothenate and CoA Biosynthesis
pathway component: ReactionCatalysis138172
pathway component: ReactionCatalysis138171
xref: Reference/SMPDB SMP0120748
xref: Reference/PathWhiz PW122009
organism: Rattus norvegicus

Pantothenate and CoA Biosynthesis
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway108658
pathway component: SubPathwayInteraction108658
xref: Reference/PathWhiz PW000032
xref: Reference/SMPDB SMP0000027
name: SubPathway

Reference/KEGG Compound C00831
type: UnificationXref
id: C00831
db: KEGG Compound

Reference/ChEBI CHEBI 16414
type: UnificationXref
id: CHEBI:16414
db: ChEBI

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

R-Methylmalonyl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: R-Methylmalonyl-CoA

electron-transfer flavoprotein
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 5086

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

6.4.1.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001560 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000988 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000463 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001051 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001034 Cytosol/Stoichiometry/1.0
right: S-Methylmalonyl-CoA
right: Adenosine diphosphate
right: Hydrogen
spontaneous: false
EC number: 6.4.1.3
left: Adenosine triphosphate
left: Propionyl-CoA
left: Hydrogen carbonate
name: Adenosine triphosphate + Hydrogen carbonate + Propionyl-CoA → Adenosine diphosphate + Hydrogen + S-Methylmalonyl-CoA
conversion direction: LEFT_TO_RIGHT

2.3.1.168
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000988 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
participant stoichiometry: Compound/PW C000003 Mitochondrial Matrix/Stoichiometry/1.0
right: Propionyl-CoA
right: NADH
spontaneous: false
EC number: 2.3.1.168
left: Coenzyme A
left: 2-Ketobutyric acid
left: NAD
name: 2-Ketobutyric acid + Coenzyme A + NAD → NADH + Propionyl-CoA
conversion direction: LEFT_TO_RIGHT

Reference/UniProt P35738
type: UnificationXref
id: P35738
db: UniProt

1.1.1.27
type: BiochemicalReaction
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 C000003 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000004 Mitochondrial Matrix/Stoichiometry/1.0
right: 2-Ketobutyric acid
right: NADH
right: Hydrogen Ion
spontaneous: false
EC number: 1.1.1.27
left: 2-Hydroxybutyric acid
left: NAD
name: 2-Hydroxybutyric acid + NAD ↔ 2-Ketobutyric acid + Hydrogen Ion + NADH
conversion direction: REVERSIBLE

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

Reduced electron-transfer flavoprotein
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 5086

Reference/ChEBI CHEBI 30831
type: UnificationXref
id: CHEBI:30831
db: ChEBI

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

Reference/UniProt Q3B7V7
type: UnificationXref
id: Q3B7V7
db: UniProt

Reference/ChemSpider 17216177
type: UnificationXref
id: 17216177
db: ChemSpider

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

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

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

Reference/PubChem-compound 21252287
type: UnificationXref
id: 21252287
db: PubChem-compound

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

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

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

Enoyl-CoA hydratase, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: Enoyl-CoA hydratase, mitochondrial

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

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

Methylmalonyl-CoA mutase, mitochondrial
type: Complex
component stoichiometry: Protein/16510 Mitochondrion/Stoichiometry/2.0
cellular location: Mitochondrion
component: Methylmalonyl CoA mutase

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

Reference/CAS 600-15-7
type: UnificationXref
id: 600-15-7
db: CAS

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

Reference/UniProt D3ZKG1
type: UnificationXref
id: D3ZKG1
db: UniProt

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

Reference/HMDB HMDB0000700
type: UnificationXref
id: HMDB0000700
db: HMDB

electron-transfer flavoprotein
type: SmallMolecule
cellular location: Cytosol
entity reference: electron-transfer flavoprotein

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

Reduced electron-transfer flavoprotein
type: SmallMolecule
cellular location: Cytosol
entity reference: Reduced electron-transfer flavoprotein

Reference/ChemSpider 10213
type: UnificationXref
id: 10213
db: ChemSpider

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

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

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

Reference/ChEBI CHEBI 16753
type: UnificationXref
id: CHEBI:16753
db: ChEBI

Coenzyme A
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Coenzyme A

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

Acetyl-CoA acetyltransferase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Acetyl-CoA acetyltransferase, mitochondrial

Compound/PW C002941 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Propinol adenylate
stoichiometric coefficient: 1.0

Reference/CAS 524-14-1
type: UnificationXref
id: 524-14-1
db: CAS

ChemicalStructure/PW C000940
type: ChemicalStructure
structure format: SMILES

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

ChemicalStructure/PW C000704
type: ChemicalStructure
structure format: SMILES
structure data: CC(C)[C@H](N)C(O)=O

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

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

Acetyl-coenzyme A synthetase
type: Protein
cellular location: Cytosol
entity reference: Acetyl-coenzyme A synthetase

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

Compound/PW C001523 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: R-Methylmalonyl-CoA
stoichiometric coefficient: 1.0

Reference/PubChem-compound 439214
type: UnificationXref
id: 439214
db: PubChem-compound

Compound/PW C001500 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: (S)-Methylmalonic acid semialdehyde
stoichiometric coefficient: 1.0

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

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

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

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

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

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

branched-chain alpha-keto dehydrogenase complex
type: Complex
component stoichiometry: Protein/14853 Mitochondrial Matrix/Stoichiometry/1.0
component stoichiometry: Protein/16464 Mitochondrial Matrix/Stoichiometry/1.0
component stoichiometry: Protein/16466 Mitochondrial Matrix/Stoichiometry/1.0
component stoichiometry: Protein/14636 Mitochondrial Matrix/Stoichiometry/1.0
cellular location: Mitochondrial Matrix
component: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
component: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
component: Dihydrolipoyl dehydrogenase, mitochondrial
component: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial

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

Reference/ChEBI CHEBI 27762
type: UnificationXref
id: CHEBI:27762
db: ChEBI

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

Reference/ChemSpider 10792
type: UnificationXref
id: 10792
db: ChemSpider

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

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

L-lactate dehydrogenase A chain
type: Protein
cellular location: Mitochondrial Matrix
entity reference: L-lactate dehydrogenase A chain

Reference/SMPDB SMP0000032
type: UnificationXref
id: SMP0000032
db: SMPDB

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

Reference/CAS 157786-88-4
type: UnificationXref
id: 157786-88-4
db: CAS

Reference/CAS 1333-74-0
type: UnificationXref
id: 1333-74-0
db: CAS

Reference/ChEBI CHEBI 17544
type: UnificationXref
id: CHEBI:17544
db: ChEBI

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

Reference/UniProt P11960
type: UnificationXref
id: P11960
db: UniProt

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

ReactionCatalysis137256
type: Catalysis
controller: Acetyl-CoA acetyltransferase, mitochondrial
controlled: 2.3.1.9
control type: ACTIVATION

Reference/CAS 6244-93-5
type: UnificationXref
id: 6244-93-5
db: CAS

Propionyl-CoA carboxylase beta chain, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: Propionyl-CoA carboxylase beta chain, mitochondrial

ChemicalStructure/PW C002942
type: ChemicalStructure
structure format: SMILES

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

Reference/SMPDB SMP0120748
type: UnificationXref
id: SMP0120748
db: SMPDB

ChemicalStructure/PW C001500
type: ChemicalStructure
structure format: SMILES
structure data: C[C@@H](C=O)C(O)=O

Reference/ChemSpider 13628334
type: UnificationXref
id: 13628334
db: ChemSpider

Reference/CAS 1420-36-6
type: UnificationXref
id: 1420-36-6
db: CAS

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

Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q3B7V7
organism: Rattus norvegicus

Malonyl-CoA
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15531
xref: Reference/KEGG Compound C00083
xref: Reference/BioCyc MALONYL-COA
xref: Reference/CAS 524-14-1
xref: Reference/HMDB HMDB0001175
xref: Reference/PubChem-compound 10663
xref: Reference/ChemSpider 10213
chemical formula: C24H38N7O19P3S
molecular weight: 853.1156
structure: ChemicalStructure/PW C000911

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

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

Reference/CAS 516-05-2
type: UnificationXref
id: 516-05-2
db: CAS

ChemicalStructure/PW C000414
type: ChemicalStructure
structure format: SMILES

R-Methylmalonyl-CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: R-Methylmalonyl-CoA

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

Protein/14636 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dihydrolipoyl dehydrogenase, mitochondrial
stoichiometric coefficient: 1.0

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

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

Reference/PubChem-compound 439340
type: UnificationXref
id: 439340
db: PubChem-compound

Reference/ChEBI CHEBI 15345
type: UnificationXref
id: CHEBI:15345
db: ChEBI

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

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

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

Reference/ChEBI CHEBI 30860
type: UnificationXref
id: CHEBI:30860
db: ChEBI

Reference/KEGG Compound C00894
type: UnificationXref
id: C00894
db: KEGG Compound

ChemicalStructure/PW C000988
type: ChemicalStructure
structure format: SMILES

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

Protein/15179 Cytosol/Stoichiometry/4.0
type: Stoichiometry
physical entity: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
stoichiometric coefficient: 4.0

Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial

Reference/BioCyc 3-METHYLBENZYLSUCCINYL-COA
type: UnificationXref
id: 3-METHYLBENZYLSUCCINYL-COA
db: BioCyc

Reaction147501
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001500 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000130 Mitochondrial Matrix/Stoichiometry/1.0
right: Methylmalonic acid
right: NADH
spontaneous: false
left: Water
left: (S)-Methylmalonic acid semialdehyde
left: NAD
name: (S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH
conversion direction: LEFT_TO_RIGHT

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

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

Reference/PubChem-compound 68152
type: UnificationXref
id: 68152
db: PubChem-compound

Reference/HMDB HMDB0000883
type: UnificationXref
id: HMDB0000883
db: HMDB

ChemicalStructure/PW C002941
type: ChemicalStructure
structure format: SMILES

Compound/Generic69 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: electron-transfer flavoprotein
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 62415
type: UnificationXref
id: CHEBI:62415
db: ChEBI

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

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

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

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

Dihydrolipoyl dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q6P6R2
organism: Rattus norvegicus