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

2-Methylbutyryl-CoA
type: SmallMoleculeReference
xref: Reference/CAS 6712-02-3
xref: Reference/ChEBI CHEBI 15477
xref: Reference/HMDB HMDB0001041
xref: Reference/KEGG Compound C01033
xref: Reference/ChemSpider 388491
xref: Reference/PubChem-compound 439371
xref: Reference/BioCyc 2-METHYL-BUTYRYL-COA
chemical formula: C26H44N7O17P3S
molecular weight: 851.1727
structure: ChemicalStructure/PW C000820

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

(S)-3-Hydroxyisobutyryl-CoA
type: SmallMoleculeReference
xref: Reference/CAS 319440-43-2
xref: Reference/KEGG Compound C06000
xref: Reference/ChEBI CHEBI 28259
xref: Reference/PubChem-compound 88
xref: Reference/HMDB HMDB0001052
xref: Reference/ChemSpider 86
xref: Reference/BioCyc 3-HYDROXY-ISOBUTYRYL-COA
chemical formula: C25H42N7O18P3S
molecular weight: 853.152
structure: ChemicalStructure/PW C000829

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

Reference/ChemSpider 52
type: UnificationXref
id: 52
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

Reference/BioCyc 2-METHYL-BUTYRYL-COA
type: UnificationXref
id: 2-METHYL-BUTYRYL-COA
db: BioCyc

Reference/CAS 73-32-5
type: UnificationXref
id: 73-32-5
db: CAS

ReactionCatalysis100730
type: Catalysis
controller: Dihydrolipoyl dehydrogenase E3
controlled: 1.8.1.4
control type: ACTIVATION

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: LEFT_TO_RIGHT

3-Methylglutaconyl-CoA
type: SmallMoleculeReference
xref: Reference/BioCyc TRANS-3-METHYL-GLUTACONYL-COA
xref: Reference/HMDB HMDB0001057
xref: Reference/KEGG Compound C03231
xref: Reference/ChEBI CHEBI 15488
xref: Reference/CAS 6247-73-0
xref: Reference/ChemSpider 11471767
xref: Reference/PubChem-compound 5462214
chemical formula: C27H42N7O19P3S
molecular weight: 893.1469
structure: ChemicalStructure/PW C000832

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

Reference/UniProt P45952
type: UnificationXref
id: P45952
db: UniProt

Reference/ChemSpider 48
type: UnificationXref
id: 48
db: ChemSpider

Reference/ChemSpider 46
type: UnificationXref
id: 46
db: ChemSpider

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

Compound/PW C000800 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Methacrylyl-CoA
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0002166
type: UnificationXref
id: HMDB0002166
db: HMDB

Methacrylyl-CoA
type: SmallMoleculeReference
xref: Reference/BioCyc METHACRYLYL-COA
xref: Reference/KEGG Compound C03460
xref: Reference/ChEBI CHEBI 27754
xref: Reference/PubChem-compound 440021
xref: Reference/CAS 6008-91-9
xref: Reference/ChemSpider 389035
xref: Reference/HMDB HMDB0001011
chemical formula: C25H40N7O17P3S
molecular weight: 835.1414
structure: ChemicalStructure/PW C000800

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

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

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

Pyruvate carboxylase, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: Pyruvate carboxylase, mitochondrial

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

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
name: SubPathwayInput

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

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

Reference/CAS 154-87-0
type: UnificationXref
id: 154-87-0
db: CAS

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

Reference/ChEBI CHEBI 17577
type: UnificationXref
id: CHEBI:17577
db: ChEBI

Thioredoxin reductase 2, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Thioredoxin reductase 2, mitochondrial

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

Protein/13679 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Alpha-aminoadipic semialdehyde dehydrogenase
stoichiometric coefficient: 4.0

ChemicalStructure/PW C001099
type: ChemicalStructure
structure format: SMILES

Reaction2230
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000130 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001523 Mitochondrion/Stoichiometry/1.0
right: R-Methylmalonyl-CoA
spontaneous: false
left: Methylmalonic acid
name: Methylmalonic acid → R-Methylmalonyl-CoA
conversion direction: LEFT_TO_RIGHT

Reference/ChemSpider 69
type: UnificationXref
id: 69
db: ChemSpider

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

Compound/PW C000373 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Methyl-2-oxovaleric acid
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 28692
type: UnificationXref
id: CHEBI:28692
db: ChEBI

ChemicalStructure/PW C000808
type: ChemicalStructure
structure format: SMILES

3-Hydroxy-3-methylglutaryl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 3-Hydroxy-3-methylglutaryl-CoA

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

ChemicalStructure/PW C000800
type: ChemicalStructure
structure format: SMILES

Reference/PubChem-compound 6106
type: UnificationXref
id: 6106
db: PubChem-compound

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

Reference/KEGG Compound C00233
type: UnificationXref
id: C00233
db: KEGG Compound

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

Reference/CAS 6712-02-3
type: UnificationXref
id: 6712-02-3
db: CAS

Reference/PathWhiz PW121770
type: UnificationXref
id: PW121770
db: PathWhiz

Compound/PW C001150 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Methylcrotonyl-CoA
stoichiometric coefficient: 1.0

Isovaleryl-CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001113
xref: Reference/ChEBI CHEBI 15487
xref: Reference/ChemSpider 388897
xref: Reference/KEGG Compound C02939
xref: Reference/BioCyc ISOVALERYL-COA
xref: Reference/CAS 6244-91-3
xref: Reference/PubChem-compound 439855
chemical formula: C26H44N7O17P3S
molecular weight: 851.1727
structure: ChemicalStructure/PW C000870

Reference/ChemSpider 94
type: UnificationXref
id: 94
db: ChemSpider

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

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

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

Reference/KEGG Compound C00248
type: UnificationXref
id: C00248
db: KEGG Compound

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

Protein/13719 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Thioredoxin reductase 2, mitochondrial
stoichiometric coefficient: 2.0

Reference/UniProt P54869
type: UnificationXref
id: P54869
db: UniProt

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

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

3-Methylglutaconyl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 3-Methylglutaconyl-CoA

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

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

Reference/ChemSpider 86
type: UnificationXref
id: 86
db: ChemSpider

Reference/KEGG Compound C15976
type: UnificationXref
id: C15976
db: KEGG Compound

Reference/KEGG Compound C15974
type: UnificationXref
id: C15974
db: KEGG Compound

Dihydrolipoyl dehydrogenase E3
type: Complex
component stoichiometry: Protein/13719 Mitochondrial Matrix/Stoichiometry/2.0
cellular location: Mitochondrial Matrix
component: Thioredoxin reductase 2, mitochondrial

Reference/ChemSpider 388897
type: UnificationXref
id: 388897
db: ChemSpider

Short-chain specific acyl-CoA dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/13906 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: Short-chain specific acyl-CoA dehydrogenase, mitochondrial

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

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

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

Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial

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

Protein/13906 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Short-chain specific acyl-CoA dehydrogenase, mitochondrial
stoichiometric coefficient: 4.0

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

Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial

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

Pyruvate carboxylase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q05920
organism: Mus musculus

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

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

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

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

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

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

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

Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial

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

Reference/ChEBI CHEBI 28259
type: UnificationXref
id: CHEBI:28259
db: ChEBI

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

Reference/ChemSpider 26333171
type: UnificationXref
id: 26333171
db: ChemSpider

Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q9D051
organism: Mus musculus

Compound/PW C000870 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Isovaleryl-CoA
stoichiometric coefficient: 1.0

Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
type: ProteinReference
xref: Reference/UniProt P35487
organism: Mus musculus

Propionyl-CoA carboxylase beta chain, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q99MN9
organism: Mus musculus

Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q9D2G2
organism: Mus musculus

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

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

Reference/CAS 793193-48-3
type: UnificationXref
id: 793193-48-3
db: CAS

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

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

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

Compound/PW C002981 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxyisovaleryl-CoA
stoichiometric coefficient: 1.0

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

ReactionCatalysis100579
type: Catalysis
controller: Aldehyde dehydrogenase, mitochondrial
controlled: 1.2.1.3
control type: ACTIVATION

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

ReactionCatalysis100578
type: Catalysis
controller: Unknown
controlled: 5.1.99.1
control type: ACTIVATION

Reference/PubChem-compound 11953876
type: UnificationXref
id: 11953876
db: PubChem-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

Reference/BioCyc 3-METHYL-CROTONYL-COA
type: UnificationXref
id: 3-METHYL-CROTONYL-COA
db: BioCyc

Reference/KEGG Compound C04405
type: UnificationXref
id: C04405
db: KEGG Compound

Compound/PW C000832 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Methylglutaconyl-CoA
stoichiometric coefficient: 1.0

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

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

ChemicalStructure/PW C001034
type: ChemicalStructure
structure format: SMILES

Reference/ChemSpider 643
type: UnificationXref
id: 643
db: ChemSpider

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

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

Protein/14117 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Hydroxymethylglutaryl-CoA lyase, mitochondrial
stoichiometric coefficient: 2.0

Reference/ChEBI CHEBI 17191
type: UnificationXref
id: CHEBI:17191
db: ChEBI

Protein/13905 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
stoichiometric coefficient: 4.0

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

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

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

Reference/UniProt Q07417
type: UnificationXref
id: Q07417
db: UniProt

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

Enoyl-CoA hydratase, mitochondrial
type: Complex
component stoichiometry: Protein/13909 Mitochondrion/Stoichiometry/6.0
cellular location: Mitochondrion
component: Enoyl-CoA hydratase, mitochondrial

ReactionCatalysis100590
type: Catalysis
controller: Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
controlled: Reaction110278
control type: ACTIVATION

Reference/ChemSpider 389707
type: UnificationXref
id: 389707
db: ChemSpider

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

Reference/HMDB HMDB0001243
type: UnificationXref
id: HMDB0001243
db: HMDB

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

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

Compound/PW C001150 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Methylcrotonyl-CoA
stoichiometric coefficient: 1.0

2-Methylacetoacetyl-CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001157
xref: Reference/ChEBI CHEBI 15476
xref: Reference/CAS 6712-01-2
xref: Reference/BioCyc 2-METHYL-ACETO-ACETYL-COA
xref: Reference/PubChem-compound 53
xref: Reference/ChemSpider 52
xref: Reference/KEGG Compound C03344
chemical formula: C26H42N7O18P3S
molecular weight: 865.152
structure: ChemicalStructure/PW C000897

Reference/ChEBI CHEBI 28291
type: UnificationXref
id: CHEBI:28291
db: ChEBI

Reference/KEGG Compound C05998
type: UnificationXref
id: C05998
db: KEGG Compound

(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

Reference/KEGG Compound C04424
type: UnificationXref
id: C04424
db: KEGG Compound

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

Compound/PW C001060 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Thiamine pyrophosphate
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00068
type: UnificationXref
id: C00068
db: KEGG Compound

Reference/BioCyc 2K-4CH3-PENTANOATE
type: UnificationXref
id: 2K-4CH3-PENTANOATE
db: BioCyc

Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial

Reference/HMDB HMDB0001493
type: UnificationXref
id: HMDB0001493
db: HMDB

Reference/HMDB HMDB0001011
type: UnificationXref
id: HMDB0001011
db: HMDB

Compound/PW C002980 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-(2-Methylbutanoyl)-dihydrolipoamide
stoichiometric coefficient: 1.0

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

1.1.1.31
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001500 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000016 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
right: (S)-Methylmalonic acid semialdehyde
right: Hydrogen Ion
right: NADH
spontaneous: false
EC number: 1.1.1.31
left: NAD
left: (S)-3-Hydroxyisobutyric acid
name: (S)-3-Hydroxyisobutyric acid + NAD → (S)-Methylmalonic acid semialdehyde + Hydrogen Ion + NADH
conversion direction: LEFT_TO_RIGHT

Reference/UniProt Q8BH95
type: UnificationXref
id: Q8BH95
db: UniProt

Reaction110705
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001500 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001455 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Mitochondrion/Stoichiometry/1.0
right: L-Glutamic acid
right: (S)-Methylmalonic acid semialdehyde
spontaneous: false
left: Oxoglutaric acid
left: (S)-beta-Aminoisobutyric acid
name: (S)-beta-Aminoisobutyric acid + Oxoglutaric acid ↔ (S)-Methylmalonic acid semialdehyde + L-Glutamic acid
conversion direction: REVERSIBLE

4.2.1.17
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000800 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000829 Mitochondrion/Stoichiometry/1.0
right: (S)-3-Hydroxyisobutyryl-CoA
spontaneous: false
EC number: 4.2.1.17
left: Water
left: Methacrylyl-CoA
name: Methacrylyl-CoA + Water → (S)-3-Hydroxyisobutyryl-CoA
conversion direction: LEFT_TO_RIGHT

3.1.2.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000016 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000829 Mitochondrion/Stoichiometry/1.0
right: Coenzyme A
right: (S)-3-Hydroxyisobutyric acid
spontaneous: false
EC number: 3.1.2.4
left: Water
left: (S)-3-Hydroxyisobutyryl-CoA
name: (S)-3-Hydroxyisobutyryl-CoA + Water → (S)-3-Hydroxyisobutyric acid + Coenzyme A
conversion direction: LEFT_TO_RIGHT

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

4.2.1.17
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001047 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001378 Mitochondrion/Stoichiometry/1.0
right: 2-Methyl-3-hydroxybutyryl-CoA
spontaneous: false
EC number: 4.2.1.17
left: Water
left: Tiglyl-CoA
name: Tiglyl-CoA + Water → 2-Methyl-3-hydroxybutyryl-CoA
conversion direction: LEFT_TO_RIGHT

Reaction110709
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000897 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001047 Mitochondrion/Stoichiometry/1.0
right: 2-Methylacetoacetyl-CoA
right: NADH
spontaneous: false
left: NAD
left: 2-Methyl-3-hydroxybutyryl-CoA
name: 2-Methyl-3-hydroxybutyryl-CoA + NAD ↔ 2-Methylacetoacetyl-CoA + NADH
conversion direction: REVERSIBLE

1.3.8.7
type: BiochemicalReaction
participant stoichiometry: Compound/Generic69 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/Generic70 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000820 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001378 Mitochondrion/Stoichiometry/1.0
right: Tiglyl-CoA
right: Reduced electron-transfer flavoprotein
spontaneous: false
EC number: 1.3.8.7
left: 2-Methylbutyryl-CoA
left: electron-transfer flavoprotein
name: 2-Methylbutyryl-CoA + electron-transfer flavoprotein ↔ Reduced electron-transfer flavoprotein + Tiglyl-CoA
conversion direction: REVERSIBLE

1.3.8.1
type: BiochemicalReaction
participant stoichiometry: Compound/Generic69 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/Generic70 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000820 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001378 Mitochondrion/Stoichiometry/1.0
right: Tiglyl-CoA
right: Reduced electron-transfer flavoprotein
spontaneous: false
EC number: 1.3.8.1
left: 2-Methylbutyryl-CoA
left: electron-transfer flavoprotein
name: 2-Methylbutyryl-CoA + electron-transfer flavoprotein → Reduced electron-transfer flavoprotein + Tiglyl-CoA
conversion direction: LEFT_TO_RIGHT

Protein/13680 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Aldehyde dehydrogenase family 16 member A1
stoichiometric coefficient: 4.0

3-Methyl-1-hydroxybutyl-ThPP
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 3-Methyl-1-hydroxybutyl-ThPP

Reference/BioCyc 2-KETO-3-METHYL-VALERATE
type: UnificationXref
id: 2-KETO-3-METHYL-VALERATE
db: BioCyc

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

Protein/13823 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
stoichiometric coefficient: 1.0

Reference/KEGG Compound C03344
type: UnificationXref
id: C03344
db: KEGG Compound

Reference/KEGG Compound C03345
type: UnificationXref
id: C03345
db: KEGG Compound

Protein/13824 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
stoichiometric coefficient: 1.0

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

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

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

Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial

2-Methyl-3-hydroxybutyryl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 2-Methyl-3-hydroxybutyryl-CoA

Reference/HMDB HMDB0000172
type: UnificationXref
id: HMDB0000172
db: HMDB

ChemicalStructure/PW C001063
type: ChemicalStructure
structure format: SMILES

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

1.3.8.1
type: BiochemicalReaction
participant stoichiometry: Compound/Generic69 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/Generic70 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000960 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000800 Mitochondrion/Stoichiometry/1.0
right: Reduced electron-transfer flavoprotein
right: Methacrylyl-CoA
spontaneous: false
EC number: 1.3.8.1
left: Isobutyryl-CoA
left: electron-transfer flavoprotein
name: Isobutyryl-CoA + electron-transfer flavoprotein → Methacrylyl-CoA + Reduced electron-transfer flavoprotein
conversion direction: LEFT_TO_RIGHT

1.3.8.7
type: BiochemicalReaction
participant stoichiometry: Compound/Generic69 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/Generic70 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000960 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000800 Mitochondrion/Stoichiometry/1.0
right: Reduced electron-transfer flavoprotein
right: Methacrylyl-CoA
spontaneous: false
EC number: 1.3.8.7
left: Isobutyryl-CoA
left: electron-transfer flavoprotein
name: Isobutyryl-CoA + electron-transfer flavoprotein ↔ Methacrylyl-CoA + Reduced electron-transfer flavoprotein
conversion direction: REVERSIBLE

ChemicalStructure/PW C001060
type: ChemicalStructure
structure format: SMILES
structure data: CC1=C(CCO[P@](O)(=O)OP(O)(O)=O)SC=[N+]1CC1=CN=C(C)N=C1N

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

2.8.3.5
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000808 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001142 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000174 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000042 Mitochondrion/Stoichiometry/1.0
right: Acetoacetyl-CoA
right: Succinic acid
spontaneous: false
EC number: 2.8.3.5
left: Succinyl-CoA
left: Acetoacetic acid
name: Acetoacetic acid + Succinyl-CoA → Acetoacetyl-CoA + Succinic acid
conversion direction: LEFT_TO_RIGHT

4.2.1.18
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001063 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000832 Mitochondrion/Stoichiometry/1.0
right: 3-Hydroxy-3-methylglutaryl-CoA
spontaneous: false
EC number: 4.2.1.18
left: Water
left: 3-Methylglutaconyl-CoA
name: 3-Methylglutaconyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA
conversion direction: LEFT_TO_RIGHT

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

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: LEFT_TO_RIGHT

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

Reference/PubChem-compound 11953835
type: UnificationXref
id: 11953835
db: PubChem-compound

Reference/UniProt Q9ESL0
type: UnificationXref
id: Q9ESL0
db: UniProt

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

Reference/ChemSpider 840
type: UnificationXref
id: 840
db: ChemSpider

Reference/CAS 6247-73-0
type: UnificationXref
id: 6247-73-0
db: CAS

Methylmalonyl-CoA mutase, mitochondrial
type: Complex
component stoichiometry: Protein/7725 Mitochondrion/Stoichiometry/2.0
cellular location: Mitochondrion
component: Unknown

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

Reference/UniProt P35487
type: UnificationXref
id: P35487
db: UniProt

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

6.4.1.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000463 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000988 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001034 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001560 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001104 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Mitochondrion/Stoichiometry/1.0
right: S-Methylmalonyl-CoA
right: Adenosine diphosphate
right: Phosphate
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 + Phosphate + S-Methylmalonyl-CoA
conversion direction: LEFT_TO_RIGHT

Reaction110712
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001500 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001783 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000130 Mitochondrion/Stoichiometry/1.0
right: Methylmalonic acid
right: Hydrogen peroxide
spontaneous: false
left: Water
left: (S)-Methylmalonic acid semialdehyde
left: Oxygen
name: (S)-Methylmalonic acid semialdehyde + Oxygen + Water → Hydrogen peroxide + Methylmalonic acid
conversion direction: LEFT_TO_RIGHT

2.3.1.16
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000940 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000897 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000988 Mitochondrion/Stoichiometry/1.0
right: Propionyl-CoA
right: Acetyl-CoA
spontaneous: false
EC number: 2.3.1.16
left: Coenzyme A
left: 2-Methylacetoacetyl-CoA
name: 2-Methylacetoacetyl-CoA + Coenzyme A → Acetyl-CoA + Propionyl-CoA
conversion direction: LEFT_TO_RIGHT

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

α-Ketoisovaleric acid
type: SmallMolecule
cellular location: Cytosol
entity reference: α-Ketoisovaleric acid

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

Reference/ChemSpider 1100
type: UnificationXref
id: 1100
db: ChemSpider

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

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

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

ChemicalStructure/PW C001047
type: ChemicalStructure
structure format: SMILES

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

Reference/HMDB HMDB0001041
type: UnificationXref
id: HMDB0001041
db: HMDB

Compound/PW C000042 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Acetoacetic acid
stoichiometric coefficient: 1.0

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

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

Protein/13924 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: 3-ketoacyl-CoA thiolase, mitochondrial
stoichiometric coefficient: 4.0

Compound/PW C002976 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Methyl-1-hydroxybutyl-ThPP
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0001057
type: UnificationXref
id: HMDB0001057
db: HMDB

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 HMDB0001052
type: UnificationXref
id: HMDB0001052
db: HMDB

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

Reference/PubChem-compound 1132
type: UnificationXref
id: 1132
db: PubChem-compound

ReactionCatalysis101026
type: Catalysis
controller: 2-oxoisovalerate dehydrogenase
controlled: 1.2.4.4
control type: ACTIVATION

ReactionCatalysis101025
type: Catalysis
controller: Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
controlled: 2.3.1.168
control type: ACTIVATION

ReactionCatalysis101024
type: Catalysis
controller: Propionyl-CoA carboxylase beta chain, mitochondrial
controlled: 6.4.1.4
control type: ACTIVATION

ReactionCatalysis101023
type: Catalysis
controller: Pyruvate carboxylase, mitochondrial
controlled: 6.4.1.4
control type: ACTIVATION

ReactionCatalysis101022
type: Catalysis
controller: Enoyl-CoA hydratase, mitochondrial
controlled: 4.2.1.17
control type: ACTIVATION

ReactionCatalysis101021
type: Catalysis
controller: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.7
control type: ACTIVATION

ReactionCatalysis101020
type: Catalysis
controller: Unknown
controlled: 1.3.8.4
control type: ACTIVATION

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

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

ReactionCatalysis101029
type: Catalysis
controller: Short-chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.1
control type: ACTIVATION

Reference/ChemSpider 22378483
type: UnificationXref
id: 22378483
db: ChemSpider

ReactionCatalysis101028
type: Catalysis
controller: Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
controlled: 2.3.1.168
control type: ACTIVATION

Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/13903 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial

ReactionCatalysis101027
type: Catalysis
controller: 2-oxoisovalerate dehydrogenase
controlled: 1.2.4.4
control type: ACTIVATION

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

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

Reference/CAS 52227-66-4
type: UnificationXref
id: 52227-66-4
db: CAS

ReactionCatalysis101015
type: Catalysis
controller: Unknown
controlled: 2.6.1.42
control type: ACTIVATION

ReactionCatalysis101014
type: Catalysis
controller: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
controlled: 1.2.4.4
control type: ACTIVATION

ReactionCatalysis101013
type: Catalysis
controller: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
controlled: 1.2.4.4
control type: ACTIVATION

ReactionCatalysis101012
type: Catalysis
controller: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
controlled: 1.2.4.4
control type: ACTIVATION

ReactionCatalysis101011
type: Catalysis
controller: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
controlled: 1.2.4.4
control type: ACTIVATION

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

ReactionCatalysis101010
type: Catalysis
controller: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
controlled: 1.2.4.4
control type: ACTIVATION

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

Reference/ChemSpider 11471767
type: UnificationXref
id: 11471767
db: ChemSpider

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

Tiglyl-CoA
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15478
xref: Reference/ChemSpider 4444187
xref: Reference/PubChem-compound 5280564
xref: Reference/BioCyc TIGLYL-COA
xref: Reference/HMDB HMDB0002054
xref: Reference/KEGG Compound C03345
chemical formula: C26H42N7O17P3S
molecular weight: 849.15704
structure: ChemicalStructure/PW C001378

Reference/PubChem-compound 53
type: UnificationXref
id: 53
db: PubChem-compound

ReactionCatalysis101019
type: Catalysis
controller: Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
controlled: 2.3.1.168
control type: ACTIVATION

Acetoacetic acid
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15344
xref: Reference/HMDB HMDB0000060
xref: Reference/BioCyc 3-KETOBUTYRATE
xref: Reference/KEGG Compound C00164
xref: Reference/PubChem-compound 96
xref: Reference/CAS 541-50-4
xref: Reference/ChemSpider 94
chemical formula: C4H6O3
molecular weight: 102.03169
structure: ChemicalStructure/PW C000042

ReactionCatalysis101018
type: Catalysis
controller: 2-oxoisovalerate dehydrogenase
controlled: 1.2.4.4
control type: ACTIVATION

ReactionCatalysis101017
type: Catalysis
controller: Unknown
controlled: 2.6.1.42
control type: ACTIVATION

ReactionCatalysis101016
type: Catalysis
controller: Unknown
controlled: 2.6.1.42
control type: ACTIVATION

3-Methyl-2-oxovaleric acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 3-Methyl-2-oxovaleric acid

Dihydrolipoyl dehydrogenase E3
type: Complex
component stoichiometry: Protein/13719 Mitochondrion/Stoichiometry/2.0
cellular location: Mitochondrion
component: Thioredoxin reductase 2, mitochondrial

ReactionCatalysis101040
type: Catalysis
controller: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.1
control type: ACTIVATION

Reference/UniProt Q9D2G2
type: UnificationXref
id: Q9D2G2
db: UniProt

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

ReactionCatalysis101048
type: Catalysis
controller: Acetyl-CoA acetyltransferase, mitochondrial
controlled: 2.3.1.9
control type: ACTIVATION

ReactionCatalysis101047
type: Catalysis
controller: Unknown
controlled: 4.2.1.18
control type: ACTIVATION

Reference/ChEBI CHEBI 48430
type: UnificationXref
id: CHEBI:48430
db: ChEBI

ReactionCatalysis101046
type: Catalysis
controller: Unknown
controlled: Reaction110712
control type: ACTIVATION

ReactionCatalysis101045
type: Catalysis
controller: Propionyl-CoA carboxylase beta chain, mitochondrial
controlled: 6.4.1.3
control type: ACTIVATION

ReactionCatalysis101044
type: Catalysis
controller: Pyruvate carboxylase, mitochondrial
controlled: 6.4.1.3
control type: ACTIVATION

ReactionCatalysis101043
type: Catalysis
controller: 3-ketoacyl-CoA thiolase, mitochondrial
controlled: 2.3.1.16
control type: ACTIVATION

ReactionCatalysis101042
type: Catalysis
controller: 3-hydroxyacyl-CoA dehydrogenase type-2
controlled: Reaction110709
control type: ACTIVATION

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

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

ReactionCatalysis101041
type: Catalysis
controller: Enoyl-CoA hydratase, mitochondrial
controlled: 4.2.1.17
control type: ACTIVATION

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

Hydrogen Ion
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Hydrogen Ion

Reference/PubChem-compound 88
type: UnificationXref
id: 88
db: PubChem-compound

α-Ketoisovaleric acid
type: SmallMoleculeReference
xref: Reference/CAS 759-05-7
xref: Reference/PubChem-compound 49
xref: Reference/ChemSpider 48
xref: Reference/HMDB HMDB0000019
xref: Reference/BioCyc 2-KETO-ISOVALERATE
xref: Reference/KEGG Compound C00141
xref: Reference/ChEBI CHEBI 16530
chemical formula: C5H8O3
molecular weight: 116.04734
structure: ChemicalStructure/PW C000012

ReactionCatalysis101049
type: Catalysis
controller: Succinyl-CoA:3-ketoacid coenzyme A transferase 2B, mitochondrial
controlled: 2.8.3.5
control type: ACTIVATION

(S)-3-Hydroxyisobutyric acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 440873
xref: Reference/HMDB HMDB0000023
xref: Reference/CAS 2068-83-9
xref: Reference/KEGG Compound C06001
xref: Reference/BioCyc 3-HYDROXY-ISOBUTYRATE
xref: Reference/ChEBI CHEBI 37373
xref: Reference/ChemSpider 389707
chemical formula: C4H8O3
molecular weight: 104.04734
structure: ChemicalStructure/PW C000016

Reference/KEGG Compound C05118
type: UnificationXref
id: C05118
db: KEGG Compound

Phosphate
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00009
xref: Reference/PubChem-compound 1061
xref: Reference/CAS 14265-44-2
xref: Reference/HMDB HMDB0001429
xref: Reference/BioCyc CPD-8587
xref: Reference/ChEBI CHEBI 18367
xref: Reference/ChemSpider 1032
chemical formula: O4P
molecular weight: 94.95342
structure: ChemicalStructure/PW C001104

Reference/KEGG Compound C05119
type: UnificationXref
id: C05119
db: KEGG Compound

ChemicalStructure/PW C001523
type: ChemicalStructure
structure format: SMILES

ReactionCatalysis101037
type: Catalysis
controller: 4-aminobutyrate aminotransferase, mitochondrial
controlled: Reaction110705
control type: ACTIVATION

ReactionCatalysis101036
type: Catalysis
controller: Enoyl-CoA hydratase, mitochondrial
controlled: 1.1.1.31
control type: ACTIVATION

ReactionCatalysis101035
type: Catalysis
controller: Unknown
controlled: 1.1.1.31
control type: ACTIVATION

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

Compound/PW C001063 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxy-3-methylglutaryl-CoA
stoichiometric coefficient: 1.0

ReactionCatalysis101034
type: Catalysis
controller: Unknown
controlled: 3.1.2.4
control type: ACTIVATION

ReactionCatalysis101033
type: Catalysis
controller: Enoyl-CoA hydratase, mitochondrial
controlled: 4.2.1.17
control type: ACTIVATION

ReactionCatalysis101032
type: Catalysis
controller: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.7
control type: ACTIVATION

Reference/PubChem-compound 70
type: UnificationXref
id: 70
db: PubChem-compound

ReactionCatalysis101031
type: Catalysis
controller: Unknown
controlled: 1.3.8.1
control type: ACTIVATION

ReactionCatalysis101030
type: Catalysis
controller: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.1
control type: ACTIVATION

Reference/BioCyc 2-KETO-ISOVALERATE
type: UnificationXref
id: 2-KETO-ISOVALERATE
db: BioCyc

3-Methyl-2-oxovaleric acid
type: SmallMolecule
cellular location: Cytosol
entity reference: 3-Methyl-2-oxovaleric acid

Protein/14117 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Hydroxymethylglutaryl-CoA lyase, mitochondrial
stoichiometric coefficient: 2.0

ReactionCatalysis101039
type: Catalysis
controller: Short-chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.1
control type: ACTIVATION

ReactionCatalysis101038
type: Catalysis
controller: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.7
control type: ACTIVATION

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

Compound/PW C000704 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Valine
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

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

Reference/ChEBI CHEBI 27754
type: UnificationXref
id: CHEBI:27754
db: ChEBI

ChemicalStructure/PW C001104
type: ChemicalStructure
structure format: SMILES
structure data: [O-]P([O-])([O-])=O

ChemicalStructure/PW C000012
type: ChemicalStructure
structure format: SMILES
structure data: CC(C)C(=O)C(O)=O

Maple Syrup Urine Disease
type: Pathway
pathway order: PathwayEnzymeReaction/1190960
pathway order: PathwayEnzymeReaction/1190961
pathway order: PathwaySubPathway/57709
pathway order: PathwayEnzymeReaction/1190919
pathway order: PathwayEnzymeReaction/1190915
pathway order: PathwayEnzymeReaction/1190959
pathway order: PathwaySubPathway/57712
pathway order: PathwayEnzymeReaction/1190916
pathway order: PathwaySubPathway/57711
pathway order: PathwayEnzymeReaction/1190917
pathway order: PathwayEnzymeReaction/1190918
pathway order: PathwayEnzymeReaction/1190911
pathway order: PathwayEnzymeReaction/1190955
pathway order: PathwayEnzymeReaction/1190912
pathway order: PathwayEnzymeReaction/1190956
pathway order: PathwayEnzymeReaction/1190913
pathway order: PathwayEnzymeReaction/1190957
pathway order: PathwaySubPathway/57710
pathway order: PathwayEnzymeReaction/1190914
pathway order: PathwayEnzymeReaction/1190958
pathway order: PathwayEnzymeReaction/1190951
pathway order: PathwayEnzymeReaction/1190952
pathway order: PathwayEnzymeReaction/1190953
pathway order: PathwayEnzymeReaction/1190910
pathway order: PathwayEnzymeReaction/1190954
pathway order: PathwayEnzymeReaction/1190950
pathway order: PathwayEnzymeReaction/1190908
pathway order: PathwayEnzymeReaction/1190909
pathway order: PathwayEnzymeReaction/1190948
pathway order: PathwayEnzymeReaction/1190949
pathway order: PathwayEnzymeReaction/1190907
pathway order: PathwayEnzymeReaction/1190944
pathway order: PathwayEnzymeReaction/1190945
pathway order: PathwayEnzymeReaction/1190946
pathway order: PathwayEnzymeReaction/1190947
pathway order: PathwayEnzymeReaction/1190940
pathway order: PathwayEnzymeReaction/1190941
pathway order: PathwayEnzymeReaction/1190942
pathway order: PathwayEnzymeReaction/1190943
pathway order: PathwayEnzymeReaction/1190937
pathway order: PathwayEnzymeReaction/1190938
pathway order: PathwayEnzymeReaction/1190939
pathway order: PathwayEnzymeReaction/1190933
pathway order: PathwayEnzymeReaction/1190934
pathway order: PathwayEnzymeReaction/1190935
pathway order: PathwayEnzymeReaction/1190936
pathway order: PathwayEnzymeReaction/1190930
pathway order: PathwayEnzymeReaction/1190931
pathway order: PathwayEnzymeReaction/1190932
pathway order: PathwayEnzymeReaction/1190926
pathway order: PathwayEnzymeReaction/1190927
pathway order: PathwayEnzymeReaction/1190928
pathway order: PathwayEnzymeReaction/1190929
pathway order: PathwayEnzymeReaction/1190922
pathway order: PathwayEnzymeReaction/1190923
pathway order: PathwayEnzymeReaction/1190924
pathway order: PathwayEnzymeReaction/1190925
pathway order: PathwayEnzymeReaction/1190920
pathway order: PathwayEnzymeReaction/1190921
pathway component: ReactionCatalysis101026
pathway component: ReactionCatalysis101025
pathway component: ReactionCatalysis101024
pathway component: ReactionCatalysis101023
pathway component: ReactionCatalysis101022
pathway component: ReactionCatalysis101021
pathway component: ReactionCatalysis101020
pathway component: ReactionCatalysis100579
pathway component: ReactionCatalysis101029
pathway component: ReactionCatalysis100578
pathway component: ReactionCatalysis101028
pathway component: ReactionCatalysis100730
pathway component: ReactionCatalysis101027
pathway component: 5.4.99.2
pathway component: ReactionCatalysis101015
pathway component: ReactionCatalysis101014
pathway component: ReactionCatalysis101013
pathway component: ReactionCatalysis101012
pathway component: ReactionCatalysis101011
pathway component: ReactionCatalysis101010

Hydroxymethylglutaryl-CoA lyase, mitochondrial
type: Complex
component stoichiometry: Protein/14117 Mitochondrion/Stoichiometry/2.0
cellular location: Mitochondrion
component: Hydroxymethylglutaryl-CoA lyase, mitochondrial

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

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

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

Reference/ChemSpider 4444187
type: UnificationXref
id: 4444187
db: ChemSpider

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

ChemicalStructure/PW C000016
type: ChemicalStructure
structure format: SMILES
structure data: C[C@@H](CO)C(O)=O

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

Reference/PubChem-compound 5462214
type: UnificationXref
id: 5462214
db: PubChem-compound

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

Reference/PubChem-compound 49
type: UnificationXref
id: 49
db: PubChem-compound

2-Methylbutyryl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 2-Methylbutyryl-CoA

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

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

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

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

ReactionCatalysis101004
type: Catalysis
controller: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
controlled: 1.2.4.4
control type: ACTIVATION

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

ReactionCatalysis101003
type: Catalysis
controller: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
controlled: 1.2.4.4
control type: ACTIVATION

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

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

Reference/KEGG Compound C06000
type: UnificationXref
id: C06000
db: KEGG Compound

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/KEGG Compound C06001
type: UnificationXref
id: C06001
db: KEGG Compound

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

Succinyl-CoA:3-ketoacid coenzyme A transferase 2B, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Succinyl-CoA:3-ketoacid coenzyme A transferase 2B, mitochondrial

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

2-oxoisovalerate dehydrogenase
type: Complex
component stoichiometry: Protein/13824 Mitochondrion/Stoichiometry/1.0
component stoichiometry: Protein/13823 Mitochondrion/Stoichiometry/1.0
cellular location: Mitochondrion
component: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
component: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial

ReactionCatalysis101009
type: Catalysis
controller: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
controlled: 1.2.4.4
control type: ACTIVATION

ReactionCatalysis101008
type: Catalysis
controller: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
controlled: 1.2.4.4
control type: ACTIVATION

Reference/PubChem-compound 47
type: UnificationXref
id: 47
db: PubChem-compound

ReactionCatalysis101007
type: Catalysis
controller: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
controlled: 1.2.4.4
control type: ACTIVATION

ReactionCatalysis101006
type: Catalysis
controller: Hydroxymethylglutaryl-CoA lyase, mitochondrial
controlled: 4.1.3.4
control type: ACTIVATION

ReactionCatalysis101005
type: Catalysis
controller: Hydroxymethylglutaryl-CoA lyase, mitochondrial
controlled: 4.1.3.4
control type: ACTIVATION

Compound/PW C001063 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxy-3-methylglutaryl-CoA
stoichiometric coefficient: 1.0

Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/13905 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial

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

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

3-Methylcrotonyl-CoA
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15486
xref: Reference/CAS 793193-48-3
xref: Reference/PubChem-compound 439869
xref: Reference/BioCyc 3-METHYL-CROTONYL-COA
xref: Reference/ChemSpider 388909
xref: Reference/HMDB HMDB0001493
xref: Reference/KEGG Compound C03069
chemical formula: C26H42N7O17P3S
molecular weight: 849.15704
structure: ChemicalStructure/PW C001150

ReactionCatalysis100147
type: Catalysis
controller: Hydroxymethylglutaryl-CoA synthase, mitochondrial
controlled: 2.3.3.10
control type: ACTIVATION

Reference/ChemSpider 2300823
type: UnificationXref
id: 2300823
db: ChemSpider

Reference/CAS 319440-43-2
type: UnificationXref
id: 319440-43-2
db: CAS

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

Reference/BioCyc 3-KETOBUTYRATE
type: UnificationXref
id: 3-KETOBUTYRATE
db: BioCyc

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

2-Methyl-1-hydroxypropyl-ThPP
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 2-Methyl-1-hydroxypropyl-ThPP

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

Reference/PubChem-compound 23724625
type: UnificationXref
id: 23724625
db: PubChem-compound

Reference/PubChem-compound 23724626
type: UnificationXref
id: 23724626
db: PubChem-compound

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

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

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

Reference/BioCyc 2-(alpha-lactyl)-thpp
type: UnificationXref
id: 2-(alpha-lactyl)-thpp
db: BioCyc

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

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

Reference/ChemSpider 389295
type: UnificationXref
id: 389295
db: ChemSpider

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

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

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

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

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

SubPathwayInteraction57710
type: Interaction
name: SubPathway57710Reaction
name: SubPathwayReaction
participant: Acetyl-CoA

SubPathwayInteraction57711
type: Interaction
name: SubPathwayReaction
name: SubPathway57711Reaction
participant: Propionyl-CoA

SubPathwayInteraction57712
type: Interaction
name: SubPathway57712Reaction
name: SubPathwayReaction
participant: Succinyl-CoA
participant: Acetyl-CoA

Compound/PW C002977 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Methyl-1-hydroxypropyl-ThPP
stoichiometric coefficient: 1.0

ChemicalStructure/PW C000829
type: ChemicalStructure
structure format: SMILES

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

ChemicalStructure/PW C000820
type: ChemicalStructure
structure format: SMILES

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

Reference/PubChem-compound 440326
type: UnificationXref
id: 440326
db: PubChem-compound

Reference/PubChem-compound 440566
type: UnificationXref
id: 440566
db: PubChem-compound

Reference/PubChem-compound 440565
type: UnificationXref
id: 440565
db: PubChem-compound

Reference/CAS 6712-01-2
type: UnificationXref
id: 6712-01-2
db: CAS

SubPathwayInteraction57709
type: Interaction
name: SubPathway57709Reaction
name: SubPathwayReaction
participant: (S)-beta-Aminoisobutyric acid

Compound/PW C000960 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Isobutyryl-CoA
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

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

Thiamine pyrophosphate
type: SmallMolecule
cellular location: Cytosol
entity reference: Thiamine pyrophosphate

ChemicalStructure/PW C000832
type: ChemicalStructure
structure format: SMILES

Reference/CAS 816-66-0
type: UnificationXref
id: 816-66-0
db: CAS

Reference/ChemSpider 389035
type: UnificationXref
id: 389035
db: ChemSpider

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

Reference/HMDB HMDB0000962
type: UnificationXref
id: HMDB0000962
db: HMDB

Compound/PW C000373 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Methyl-2-oxovaleric acid
stoichiometric coefficient: 1.0

3-Methylcrotonyl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 3-Methylcrotonyl-CoA

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

Reference/KEGG Compound C02939
type: UnificationXref
id: C02939
db: KEGG Compound

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

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

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

Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
type: Complex
component stoichiometry: Protein/13679 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: Alpha-aminoadipic semialdehyde dehydrogenase

Reference/CAS 61-90-5
type: UnificationXref
id: 61-90-5
db: CAS

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

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

Compound/PW C001060 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Thiamine pyrophosphate
stoichiometric coefficient: 1.0

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

1.2.1.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001500 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000130 Mitochondrion/Stoichiometry/1.0
right: Methylmalonic acid
right: NADH
spontaneous: false
EC number: 1.2.1.3
left: NAD
left: Water
left: (S)-Methylmalonic acid semialdehyde
name: (S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH
conversion direction: LEFT_TO_RIGHT

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

5.1.99.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001560 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001523 Mitochondrion/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

Protein/7725 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Unknown
stoichiometric coefficient: 2.0

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

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

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

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

ChemicalStructure/PW C000414
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C000897
type: ChemicalStructure
structure format: SMILES

S-(3-Methylbutanoyl)-dihydrolipoamide-E
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: S-(3-Methylbutanoyl)-dihydrolipoamide-E

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

Reference/HMDB HMDB0000985
type: UnificationXref
id: HMDB0000985
db: HMDB

Reaction110278
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001500 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000463 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000988 Mitochondrion/Stoichiometry/1.0
right: Propionyl-CoA
right: Hydrogen carbonate
right: NADH
spontaneous: false
left: NAD
left: Water
left: (S)-Methylmalonic acid semialdehyde
left: Coenzyme A
name: (S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD + Water → Hydrogen carbonate + NADH + Propionyl-CoA
conversion direction: LEFT_TO_RIGHT

Acetyl-CoA acetyltransferase, mitochondrial
type: Complex
cellular location: Mitochondrion

Thiamine pyrophosphate
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Thiamine pyrophosphate

Reference/ChEBI CHEBI 15344
type: UnificationXref
id: CHEBI:15344
db: ChEBI

Reference/ChEBI CHEBI 37373
type: UnificationXref
id: CHEBI:37373
db: ChEBI

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

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

Reference/ChemSpider 389466
type: UnificationXref
id: 389466
db: ChemSpider

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

Reference/ChemSpider 389465
type: UnificationXref
id: 389465
db: ChemSpider

Protein/13909 Mitochondrion/Stoichiometry/6.0
type: Stoichiometry
physical entity: Enoyl-CoA hydratase, mitochondrial
stoichiometric coefficient: 6.0

ChemicalStructure/PW C000870
type: ChemicalStructure
structure format: SMILES

Thiamine pyrophosphate
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Thiamine pyrophosphate

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

Hydroxymethylglutaryl-CoA synthase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Hydroxymethylglutaryl-CoA synthase, mitochondrial

(S)-beta-Aminoisobutyric acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: (S)-beta-Aminoisobutyric acid
name: SubPathwayInput

Reference/PubChem-compound 439371
type: UnificationXref
id: 439371
db: PubChem-compound

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

Protein/13824 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
stoichiometric coefficient: 1.0

Reference/CAS 3884-47-7
type: UnificationXref
id: 3884-47-7
db: CAS

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

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/ChemSpider 388357
type: UnificationXref
id: 388357
db: ChemSpider

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

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

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

Reference/ChEBI CHEBI 17460
type: UnificationXref
id: CHEBI:17460
db: ChEBI

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

2-oxoisovalerate dehydrogenase
type: Complex
component stoichiometry: Protein/13824 Cytosol/Stoichiometry/1.0
component stoichiometry: Protein/13823 Cytosol/Stoichiometry/1.0
cellular location: Cytosol
component: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
component: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial

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

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

Reference/PubChem-compound 440021
type: UnificationXref
id: 440021
db: PubChem-compound

Thioredoxin reductase 2, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q9JLT4
organism: Mus musculus

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

Reference/UniProt Q9D051
type: UnificationXref
id: Q9D051
db: UniProt

Reference/HMDB HMDB0006870
type: UnificationXref
id: HMDB0006870
db: HMDB

Compound/PW C002977 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Methyl-1-hydroxypropyl-ThPP
stoichiometric coefficient: 1.0

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

Reference/CAS 4249-19-8
type: UnificationXref
id: 4249-19-8
db: CAS

3-hydroxyacyl-CoA dehydrogenase type-2
type: Complex
component stoichiometry: Protein/13917 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: 3-hydroxyacyl-CoA dehydrogenase type-2

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

Isobutyryl-CoA
type: SmallMoleculeReference
xref: Reference/PubChem-compound 3036931
xref: Reference/ChemSpider 2300823
xref: Reference/BioCyc ISOBUTYRYL-COA
xref: Reference/ChEBI CHEBI 15479
xref: Reference/CAS 15621-60-0
xref: Reference/HMDB HMDB0001243
xref: Reference/KEGG Compound C00630
chemical formula: C25H42N7O17P3S
molecular weight: 837.15704
structure: ChemicalStructure/PW C000960

Reference/CAS 6008-91-9
type: UnificationXref
id: 6008-91-9
db: CAS

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

Reference/KEGG Compound C00579
type: UnificationXref
id: C00579
db: KEGG Compound

Reference/BioCyc 2-METHYL-ACETO-ACETYL-COA
type: UnificationXref
id: 2-METHYL-ACETO-ACETYL-COA
db: BioCyc

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

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

4.1.3.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000940 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001063 Mitochondrion/Stoichiometry/1.0
right: Acetyl-CoA
spontaneous: false
EC number: 4.1.3.4
left: 3-Hydroxy-3-methylglutaryl-CoA
name: 3-Hydroxy-3-methylglutaryl-CoA → Acetyl-CoA
conversion direction: LEFT_TO_RIGHT

Reference/UniProt P38060
type: UnificationXref
id: P38060
db: UniProt

4.1.3.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001063 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000042 Mitochondrial Matrix/Stoichiometry/1.0
right: Acetoacetic acid
spontaneous: false
EC number: 4.1.3.4
left: 3-Hydroxy-3-methylglutaryl-CoA
name: 3-Hydroxy-3-methylglutaryl-CoA → Acetoacetic acid
conversion direction: LEFT_TO_RIGHT

1.2.4.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002978 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000769 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002976 Mitochondrial Matrix/Stoichiometry/1.0
right: S-(3-Methylbutanoyl)-dihydrolipoamide-E
spontaneous: false
EC number: 1.2.4.4
left: 3-Methyl-1-hydroxybutyl-ThPP
left: Lipoamide
name: 3-Methyl-1-hydroxybutyl-ThPP + Lipoamide → S-(3-Methylbutanoyl)-dihydrolipoamide-E
conversion direction: LEFT_TO_RIGHT

1.2.4.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002980 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002977 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000769 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001060 Cytosol/Stoichiometry/1.0
right: Thiamine pyrophosphate
right: S-(2-Methylbutanoyl)-dihydrolipoamide
spontaneous: false
EC number: 1.2.4.4
left: Lipoamide
left: 2-Methyl-1-hydroxypropyl-ThPP
name: 2-Methyl-1-hydroxypropyl-ThPP + Lipoamide → S-(2-Methylbutanoyl)-dihydrolipoamide + Thiamine pyrophosphate
conversion direction: LEFT_TO_RIGHT

1.2.4.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002979 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000769 Mitochondrial Matrix/Stoichiometry/1.0
right: S-(2-Methylpropionyl)-dihydrolipoamide-E
spontaneous: false
EC number: 1.2.4.4
left: Lipoamide
name: Lipoamide → S-(2-Methylpropionyl)-dihydrolipoamide-E
conversion direction: LEFT_TO_RIGHT

S-(2-Methylpropionyl)-dihydrolipoamide-E
type: SmallMolecule
cellular location: Mitochondrion
entity reference: S-(2-Methylpropionyl)-dihydrolipoamide-E

1.2.4.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002977 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001060 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002976 Mitochondrial Matrix/Stoichiometry/1.0
right: Thiamine pyrophosphate
spontaneous: false
EC number: 1.2.4.4
left: 3-Methyl-1-hydroxybutyl-ThPP
left: 2-Methyl-1-hydroxypropyl-ThPP
name: 2-Methyl-1-hydroxypropyl-ThPP + 3-Methyl-1-hydroxybutyl-ThPP → Thiamine pyrophosphate
conversion direction: LEFT_TO_RIGHT

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

1.2.4.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002979 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002977 Mitochondrial Matrix/Stoichiometry/1.0
right: S-(2-Methylpropionyl)-dihydrolipoamide-E
spontaneous: false
EC number: 1.2.4.4
left: 2-Methyl-1-hydroxypropyl-ThPP
name: 2-Methyl-1-hydroxypropyl-ThPP → S-(2-Methylpropionyl)-dihydrolipoamide-E
conversion direction: LEFT_TO_RIGHT

2.6.1.42
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000112 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000373 Cytosol/Stoichiometry/1.0
right: 3-Methyl-2-oxovaleric acid
right: L-Glutamic acid
spontaneous: false
EC number: 2.6.1.42
left: Oxoglutaric acid
left: L-Isoleucine
name: L-Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + L-Glutamic acid
conversion direction: LEFT_TO_RIGHT

2.6.1.42
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000012 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000704 Cytosol/Stoichiometry/1.0
right: α-Ketoisovaleric acid
right: L-Glutamic acid
spontaneous: false
EC number: 2.6.1.42
left: Oxoglutaric acid
left: L-Valine
name: L-Valine + Oxoglutaric acid → L-Glutamic acid + α-Ketoisovaleric acid
conversion direction: LEFT_TO_RIGHT

Compound/PW C000547 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Ketoleucine
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0002054
type: UnificationXref
id: HMDB0002054
db: HMDB

2.6.1.42
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000540 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000547 Cytosol/Stoichiometry/1.0
right: Ketoleucine
right: L-Glutamic acid
spontaneous: false
EC number: 2.6.1.42
left: Oxoglutaric acid
left: L-Leucine
name: L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic acid
conversion direction: LEFT_TO_RIGHT

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

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

Reference/ChEBI CHEBI 17694
type: UnificationXref
id: CHEBI:17694
db: ChEBI

1.3.8.4
type: BiochemicalReaction
participant stoichiometry: Compound/Generic69 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/Generic70 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001150 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000870 Mitochondrion/Stoichiometry/1.0
right: Reduced electron-transfer flavoprotein
right: 3-Methylcrotonyl-CoA
spontaneous: false
EC number: 1.3.8.4
left: electron-transfer flavoprotein
left: Isovaleryl-CoA
name: Isovaleryl-CoA + electron-transfer flavoprotein → 3-Methylcrotonyl-CoA + Reduced electron-transfer flavoprotein
conversion direction: LEFT_TO_RIGHT

Reference/UniProt Q99MN9
type: UnificationXref
id: Q99MN9
db: UniProt

1.3.8.7
type: BiochemicalReaction
participant stoichiometry: Compound/Generic69 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/Generic70 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001150 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000870 Mitochondrion/Stoichiometry/1.0
right: Reduced electron-transfer flavoprotein
right: 3-Methylcrotonyl-CoA
spontaneous: false
EC number: 1.3.8.7
left: electron-transfer flavoprotein
left: Isovaleryl-CoA
name: Isovaleryl-CoA + electron-transfer flavoprotein ↔ 3-Methylcrotonyl-CoA + Reduced electron-transfer flavoprotein
conversion direction: REVERSIBLE

1.2.4.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002976 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000547 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001060 Mitochondrion/Stoichiometry/1.0
right: Carbon dioxide
right: 3-Methyl-1-hydroxybutyl-ThPP
spontaneous: false
EC number: 1.2.4.4
left: Ketoleucine
left: Thiamine pyrophosphate
name: Ketoleucine + Thiamine pyrophosphate ? 3-Methyl-1-hydroxybutyl-ThPP + Carbon dioxide

2.3.1.168
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000782 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002978 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000870 Mitochondrion/Stoichiometry/1.0
right: Isovaleryl-CoA
right: Dihydrolipoamide
spontaneous: false
EC number: 2.3.1.168
left: S-(3-Methylbutanoyl)-dihydrolipoamide-E
left: Coenzyme A
name: Coenzyme A + S-(3-Methylbutanoyl)-dihydrolipoamide-E → Dihydrolipoamide + Isovaleryl-CoA
conversion direction: LEFT_TO_RIGHT

2.3.1.168
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002979 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000782 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000960 Mitochondrion/Stoichiometry/1.0
right: Isobutyryl-CoA
right: Dihydrolipoamide
spontaneous: false
EC number: 2.3.1.168
left: Coenzyme A
left: S-(2-Methylpropionyl)-dihydrolipoamide-E
name: Coenzyme A + S-(2-Methylpropionyl)-dihydrolipoamide-E → Dihydrolipoamide + Isobutyryl-CoA
conversion direction: LEFT_TO_RIGHT

1.2.4.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000012 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002977 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001060 Cytosol/Stoichiometry/1.0
right: Carbon dioxide
right: 2-Methyl-1-hydroxypropyl-ThPP
spontaneous: false
EC number: 1.2.4.4
left: α-Ketoisovaleric acid
left: Thiamine pyrophosphate
name: Thiamine pyrophosphate + α-Ketoisovaleric acid → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
conversion direction: LEFT_TO_RIGHT

4.2.1.17
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002981 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001150 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
right: Water
right: 3-Methylcrotonyl-CoA
spontaneous: false
EC number: 4.2.1.17
left: 3-Hydroxyisovaleryl-CoA
name: 3-Hydroxyisovaleryl-CoA → 3-Methylcrotonyl-CoA + Water
conversion direction: LEFT_TO_RIGHT

Mus musculus
type: BioSource
xref: Reference/TAXONOMY 10090

6.4.1.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000414 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001150 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000463 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000832 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001034 Cytosol/Stoichiometry/1.0
right: 3-Methylglutaconyl-CoA
right: Adenosine diphosphate
spontaneous: false
EC number: 6.4.1.4
left: Adenosine triphosphate
left: 3-Methylcrotonyl-CoA
left: Hydrogen carbonate
name: 3-Methylcrotonyl-CoA + Adenosine triphosphate + Hydrogen carbonate → 3-Methylglutaconyl-CoA + Adenosine diphosphate
conversion direction: LEFT_TO_RIGHT

1.2.4.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000373 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001060 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002977 Mitochondrion/Stoichiometry/1.0
right: Carbon dioxide
right: 2-Methyl-1-hydroxypropyl-ThPP
spontaneous: false
EC number: 1.2.4.4
left: 3-Methyl-2-oxovaleric acid
left: Thiamine pyrophosphate
name: 3-Methyl-2-oxovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
conversion direction: LEFT_TO_RIGHT

2.3.1.168
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000782 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000820 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002980 Mitochondrion/Stoichiometry/1.0
right: 2-Methylbutyryl-CoA
right: Dihydrolipoamide
spontaneous: false
EC number: 2.3.1.168
left: Coenzyme A
left: S-(2-Methylbutanoyl)-dihydrolipoamide
name: Coenzyme A + S-(2-Methylbutanoyl)-dihydrolipoamide → 2-Methylbutyryl-CoA + Dihydrolipoamide
conversion direction: LEFT_TO_RIGHT

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

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

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

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/KEGG Compound C00356
type: UnificationXref
id: C00356
db: KEGG Compound

Reference/ChemSpider 5880
type: UnificationXref
id: 5880
db: ChemSpider

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

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

3-hydroxyacyl-CoA dehydrogenase type-2
type: ProteinReference
xref: Reference/UniProt O08756
organism: Mus musculus

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

S-(3-Methylbutanoyl)-dihydrolipoamide-E
type: SmallMolecule
cellular location: Mitochondrion
entity reference: S-(3-Methylbutanoyl)-dihydrolipoamide-E

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

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

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

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

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

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

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

2-Methyl-1-hydroxypropyl-ThPP
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 2-Methyl-1-hydroxypropyl-ThPP

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

Reference/KEGG Compound C00123
type: UnificationXref
id: C00123
db: KEGG Compound

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

3-ketoacyl-CoA thiolase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q8BWT1
organism: Mus musculus

Reference/UniProt Q571I9
type: UnificationXref
id: Q571I9
db: UniProt

4-aminobutyrate aminotransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P61922
organism: Mus musculus

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

S-(2-Methylpropionyl)-dihydrolipoamide-E
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: S-(2-Methylpropionyl)-dihydrolipoamide-E

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

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

Reference/ChemSpider 388543
type: UnificationXref
id: 388543
db: ChemSpider

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

Thioredoxin reductase 2, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Thioredoxin reductase 2, mitochondrial

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

Reference/KEGG Compound C00141
type: UnificationXref
id: C00141
db: KEGG Compound

Reference/BioCyc TRANS-3-METHYL-GLUTACONYL-COA
type: UnificationXref
id: TRANS-3-METHYL-GLUTACONYL-COA
db: BioCyc

Reference/CAS 940-69-2
type: UnificationXref
id: 940-69-2
db: CAS

Compound/PW C001060 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Thiamine pyrophosphate
stoichiometric coefficient: 1.0

1.8.1.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000769 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000782 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000769 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000782 Mitochondrial Matrix/Stoichiometry/1.0
right: Lipoamide
right: Hydrogen Ion
right: NADH
spontaneous: false
EC number: 1.8.1.4
left: NAD
left: Dihydrolipoamide
name: Dihydrolipoamide + NAD → Hydrogen Ion + Lipoamide + NADH
conversion direction: LEFT_TO_RIGHT

Ketoleucine
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000695
xref: Reference/ChEBI CHEBI 48430
xref: Reference/ChemSpider 69
xref: Reference/PubChem-compound 70
xref: Reference/BioCyc 2K-4CH3-PENTANOATE
xref: Reference/CAS 816-66-0
xref: Reference/KEGG Compound C00233
chemical formula: C6H10O3
molecular weight: 130.06299
structure: ChemicalStructure/PW C000547

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

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

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

ChemicalStructure/PW C001142
type: ChemicalStructure
structure format: SMILES

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

Reference/HMDB HMDB0000019
type: UnificationXref
id: HMDB0000019
db: HMDB

Unknown
type: Protein
cellular location: Mitochondrion
entity reference: Unknown

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

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

Reference/HMDB HMDB0000491
type: UnificationXref
id: HMDB0000491
db: HMDB

2-Methyl-1-hydroxypropyl-ThPP
type: SmallMolecule
cellular location: Cytosol
entity reference: 2-Methyl-1-hydroxypropyl-ThPP

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

Protein/13719 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Thioredoxin reductase 2, mitochondrial
stoichiometric coefficient: 2.0

3-Methyl-1-hydroxybutyl-ThPP
type: SmallMoleculeReference
xref: Reference/PubChem-compound 23724625
xref: Reference/HMDB HMDB0006865
xref: Reference/ChemSpider 22378483
xref: Reference/KEGG Compound C15974
chemical formula: C17H29N4O8P2S
molecular weight: 511.11813
structure: ChemicalStructure/PW C002976

Reference/KEGG Compound C00164
type: UnificationXref
id: C00164
db: KEGG Compound

2-Methyl-1-hydroxypropyl-ThPP
type: SmallMoleculeReference
xref: Reference/PubChem-compound 23724626
xref: Reference/HMDB HMDB0006866
xref: Reference/ChemSpider 26333171
xref: Reference/KEGG Compound C15976
xref: Reference/ChEBI CHEBI 48522
chemical formula: C16H27N4O8P2S
molecular weight: 497.10248
structure: ChemicalStructure/PW C002977

S-(3-Methylbutanoyl)-dihydrolipoamide-E
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0006867
xref: Reference/KEGG Compound C05119
xref: Reference/ChemSpider 389466
xref: Reference/ChEBI CHEBI 27462
xref: Reference/PubChem-compound 440566
chemical formula: C13H25NO2S2
molecular weight: 291.13266
structure: ChemicalStructure/PW C002978

S-(2-Methylpropionyl)-dihydrolipoamide-E
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 17577
xref: Reference/HMDB HMDB0006868
xref: Reference/KEGG Compound C04424
xref: Reference/PubChem-compound 11953835
xref: Reference/ChemSpider 10128135
chemical formula: C12H23NO2S2
molecular weight: 277.11703
structure: ChemicalStructure/PW C002979

Reference/UniProt Q9JLT4
type: UnificationXref
id: Q9JLT4
db: UniProt

Reference/HMDB HMDB0001113
type: UnificationXref
id: HMDB0001113
db: HMDB

Compound/PW C000112 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Isoleucine
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000023
type: UnificationXref
id: HMDB0000023
db: HMDB

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

S-(2-Methylbutanoyl)-dihydrolipoamide
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0006869
xref: Reference/PubChem-compound 440565
xref: Reference/KEGG Compound C05118
xref: Reference/ChEBI CHEBI 28692
xref: Reference/ChemSpider 389465
chemical formula: C13H25NO2S2
molecular weight: 291.13266
structure: ChemicalStructure/PW C002980

Reference/HMDB HMDB0001356
type: UnificationXref
id: HMDB0001356
db: HMDB

3-Hydroxyisovaleryl-CoA
type: SmallMoleculeReference
xref: Reference/ChemSpider 10128175
xref: Reference/ChEBI CHEBI 28291
xref: Reference/KEGG Compound C05998
xref: Reference/PubChem-compound 11953876
xref: Reference/HMDB HMDB0006870
chemical formula: C26H44N7O18P3S
molecular weight: 867.16766
structure: ChemicalStructure/PW C002981

Reference/ChEBI CHEBI 9532
type: UnificationXref
id: CHEBI:9532
db: ChEBI

ChemicalStructure/PW C001150
type: ChemicalStructure
structure format: SMILES

Lipoamide
type: SmallMoleculeReference
xref: Reference/PubChem-compound 863
xref: Reference/ChemSpider 840
xref: Reference/KEGG Compound C00248
xref: Reference/HMDB HMDB0000962
xref: Reference/BioCyc LIPOAMIDE
xref: Reference/CAS 940-69-2
xref: Reference/ChEBI CHEBI 17460
chemical formula: C8H15NOS2
molecular weight: 205.05951
structure: ChemicalStructure/PW C000769

Reference/PubChem-compound 663
type: UnificationXref
id: 663
db: PubChem-compound

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

Compound/PW C000782 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dihydrolipoamide
stoichiometric coefficient: 1.0

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

Protein/13903 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
stoichiometric coefficient: 4.0

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

Compound/PW C000782 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dihydrolipoamide
stoichiometric coefficient: 1.0

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

Compound/PW C002978 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-(3-Methylbutanoyl)-dihydrolipoamide-E
stoichiometric coefficient: 1.0

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

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

Compound/PW C000829 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: (S)-3-Hydroxyisobutyryl-CoA
stoichiometric coefficient: 1.0

Compound/PW C000540 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Leucine
stoichiometric coefficient: 1.0

Alpha-aminoadipic semialdehyde dehydrogenase
type: Protein
cellular location: Mitochondrion
entity reference: Alpha-aminoadipic semialdehyde dehydrogenase

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

Reference/KEGG Compound C01033
type: UnificationXref
id: C01033
db: KEGG Compound

Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial

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

2.3.3.10
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000940 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001142 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001063 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
right: Coenzyme A
right: 3-Hydroxy-3-methylglutaryl-CoA
spontaneous: false
EC number: 2.3.3.10
left: Acetoacetyl-CoA
left: Water
left: Acetyl-CoA
name: Acetoacetyl-CoA + Acetyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA + Coenzyme A
conversion direction: LEFT_TO_RIGHT

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

Reference/KEGG Compound C03460
type: UnificationXref
id: C03460
db: KEGG Compound

Hydroxymethylglutaryl-CoA lyase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Hydroxymethylglutaryl-CoA lyase, mitochondrial

ChemicalStructure/PW C000042
type: ChemicalStructure
structure format: SMILES
structure data: CC(=O)CC(O)=O

Reference/BioCyc 3-HYDROXY-ISOBUTYRATE
type: UnificationXref
id: 3-HYDROXY-ISOBUTYRATE
db: BioCyc

Reference/ChEBI CHEBI 35932
type: UnificationXref
id: CHEBI:35932
db: ChEBI

Enoyl-CoA hydratase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q8BH95
organism: Mus musculus

ChemicalStructure/PW C001378
type: ChemicalStructure
structure format: SMILES

Short-chain specific acyl-CoA dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q07417
organism: Mus musculus

Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q9DBL1
organism: Mus musculus

Dihydrolipoamide
type: SmallMoleculeReference
xref: Reference/ChemSpider 643
xref: Reference/CAS 3884-47-7
xref: Reference/KEGG Compound C00579
xref: Reference/PubChem-compound 663
xref: Reference/HMDB HMDB0000985
xref: Reference/BioCyc DIHYDROLIPOAMIDE
xref: Reference/ChEBI CHEBI 17694
chemical formula: C8H17NOS2
molecular weight: 207.07515
structure: ChemicalStructure/PW C000782

L-Leucine
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000687
xref: Reference/ChEBI CHEBI 15603
xref: Reference/CAS 61-90-5
xref: Reference/BioCyc LEU
xref: Reference/PubChem-compound 6106
xref: Reference/KEGG Compound C00123
xref: Reference/ChemSpider 5880
chemical formula: C6H13NO2
molecular weight: 131.09464
structure: ChemicalStructure/PW C000540

Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P45952
organism: Mus musculus

Reference/HMDB HMDB0001372
type: UnificationXref
id: HMDB0001372
db: HMDB

Reference/HMDB HMDB0001375
type: UnificationXref
id: HMDB0001375
db: HMDB

Compound/PW C000016 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: (S)-3-Hydroxyisobutyric acid
stoichiometric coefficient: 1.0

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

S-(2-Methylbutanoyl)-dihydrolipoamide
type: SmallMolecule
cellular location: Mitochondrion
entity reference: S-(2-Methylbutanoyl)-dihydrolipoamide

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

Reference/KEGG Compound C03465
type: UnificationXref
id: C03465
db: KEGG Compound

Reference/KEGG Compound C03231
type: UnificationXref
id: C03231
db: KEGG Compound

L-Isoleucine
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00407
xref: Reference/ChEBI CHEBI 17191
xref: Reference/PubChem-compound 6306
xref: Reference/HMDB HMDB0000172
xref: Reference/BioCyc ILE
xref: Reference/ChemSpider 6067
xref: Reference/CAS 73-32-5
chemical formula: C6H13NO2
molecular weight: 131.09464
structure: ChemicalStructure/PW C000112

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

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

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

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

Protein/13917 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: 3-hydroxyacyl-CoA dehydrogenase type-2
stoichiometric coefficient: 4.0

Reference/HMDB HMDB0001157
type: UnificationXref
id: HMDB0001157
db: HMDB

Reference/CAS 2068-83-9
type: UnificationXref
id: 2068-83-9
db: CAS

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

(S)-beta-Aminoisobutyric acid
type: SmallMoleculeReference
xref: Reference/KEGG Compound C03284
xref: Reference/ChEBI CHEBI 33094
xref: Reference/ChemSpider 388543
xref: Reference/PubChem-compound 439434
xref: Reference/CAS 4249-19-8
xref: Reference/HMDB HMDB0002166
chemical formula: C4H9NO2
molecular weight: 103.06333
structure: ChemicalStructure/PW C001455

Reference/HMDB HMDB0000060
type: UnificationXref
id: HMDB0000060
db: HMDB

Reference/PubChem-compound 96
type: UnificationXref
id: 96
db: PubChem-compound

Reference/PubChem-compound 863
type: UnificationXref
id: 863
db: PubChem-compound

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

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

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

(S)-3-Hydroxyisobutyryl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: (S)-3-Hydroxyisobutyryl-CoA

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

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

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

Hydroxymethylglutaryl-CoA lyase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Hydroxymethylglutaryl-CoA lyase, mitochondrial

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

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

3-Hydroxyisovaleryl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 3-Hydroxyisovaleryl-CoA

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

Reference/HMDB HMDB0006869
type: UnificationXref
id: HMDB0006869
db: HMDB

Reference/HMDB HMDB0006865
type: UnificationXref
id: HMDB0006865
db: HMDB

Reference/HMDB HMDB0006866
type: UnificationXref
id: HMDB0006866
db: HMDB

Reference/HMDB HMDB0006867
type: UnificationXref
id: HMDB0006867
db: HMDB

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

Reference/HMDB HMDB0006868
type: UnificationXref
id: HMDB0006868
db: HMDB

Reference/UniProt Q9DBL1
type: UnificationXref
id: Q9DBL1
db: UniProt

2-Methylacetoacetyl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 2-Methylacetoacetyl-CoA

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

Reference/ChEBI CHEBI 15603
type: UnificationXref
id: CHEBI:15603
db: ChEBI

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

Compound/PW C002978 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-(3-Methylbutanoyl)-dihydrolipoamide-E
stoichiometric coefficient: 1.0

Hydroxymethylglutaryl-CoA lyase, mitochondrial
type: Complex
component stoichiometry: Protein/14117 Mitochondrial Matrix/Stoichiometry/2.0
cellular location: Mitochondrial Matrix
component: Hydroxymethylglutaryl-CoA lyase, mitochondrial

Reference/KEGG Compound C03284
type: UnificationXref
id: C03284
db: KEGG Compound

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

Compound/PW C000820 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Methylbutyryl-CoA
stoichiometric coefficient: 1.0

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

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

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

Pyrimidine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway57709
pathway component: SubPathwayInteraction57709
xref: Reference/SMPDB SMP0000046
xref: Reference/PathWhiz PW000160
name: SubPathway

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

Reference/TAXONOMY 10090
type: UnificationXref
id: 10090
db: TAXONOMY

Reference/CAS 1553-55-5
type: UnificationXref
id: 1553-55-5
db: CAS

ChemicalStructure/PW C000547
type: ChemicalStructure
structure format: SMILES
structure data: CC(C)CC(=O)C(O)=O

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

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

Reference/CAS 759-05-7
type: UnificationXref
id: 759-05-7
db: CAS

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

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

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

3-Methyl-2-oxovaleric acid
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 35932
xref: Reference/ChemSpider 46
xref: Reference/CAS 1460-34-0
xref: Reference/BioCyc 2-KETO-3-METHYL-VALERATE
xref: Reference/HMDB HMDB0000491
xref: Reference/PubChem-compound 47
xref: Reference/KEGG Compound C03465
chemical formula: C6H10O3
molecular weight: 130.06299
structure: ChemicalStructure/PW C000373

Reference/PubChem-compound 439855
type: UnificationXref
id: 439855
db: PubChem-compound

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

Compound/PW C001378 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Tiglyl-CoA
stoichiometric coefficient: 1.0

Citric Acid Cycle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway57710
pathway order: SubPathwayInteractionSteps/SubPathway57712
pathway component: SubPathwayInteraction57710
pathway component: SubPathwayInteraction57712
xref: Reference/PathWhiz PW000005
xref: Reference/SMPDB SMP0000057
name: SubPathway

Hydroxymethylglutaryl-CoA synthase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P54869
organism: Mus musculus

Compound/PW C000897 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Methylacetoacetyl-CoA
stoichiometric coefficient: 1.0

ChemicalStructure/PW C002979
type: ChemicalStructure
structure format: SMILES
structure data: CC(C)C(=O)SCCC(S)CCCCC(N)=O

ChemicalStructure/PW C002976
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C002977
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C002978
type: ChemicalStructure
structure format: SMILES
structure data: CC(C)CC(=O)SCCC(S)CCCCC(N)=O

Reference/UniProt Q8BWT1
type: UnificationXref
id: Q8BWT1
db: UniProt

Reference/SMPDB SMP0120516
type: UnificationXref
id: SMP0120516
db: SMPDB

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

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

Reference/HMDB HMDB0000687
type: UnificationXref
id: HMDB0000687
db: HMDB

ChemicalStructure/PW C002980
type: ChemicalStructure
structure format: SMILES
structure data: CCC(C)C(=O)SCCC(S)CCCCC(N)=O

ChemicalStructure/PW C002981
type: ChemicalStructure
structure format: SMILES

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

Reference/ChemSpider 388909
type: UnificationXref
id: 388909
db: ChemSpider

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

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

Compound/PW C000832 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Methylglutaconyl-CoA
stoichiometric coefficient: 1.0

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

Pyruvate carboxylase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Pyruvate carboxylase, mitochondrial

Reference/KEGG Compound C03069
type: UnificationXref
id: C03069
db: KEGG Compound

Reference/HMDB HMDB0000695
type: UnificationXref
id: HMDB0000695
db: HMDB

ChemicalStructure/PW C000373
type: ChemicalStructure
structure format: SMILES
structure data: CCC(C)C(=O)C(O)=O

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

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

Reference/PubChem-compound 5280564
type: UnificationXref
id: 5280564
db: PubChem-compound

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

2-Methyl-3-hydroxybutyryl-CoA
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001356
xref: Reference/ChEBI CHEBI 15449
xref: Reference/KEGG Compound C04405
xref: Reference/ChemSpider 389295
xref: Reference/PubChem-compound 440326
xref: Reference/CAS 52227-66-4
xref: Reference/BioCyc 2-METHYL-3-HYDROXY-BUTYRYL-COA
chemical formula: C26H44N7O18P3S
molecular weight: 867.16766
structure: ChemicalStructure/PW C001047

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

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

Reference/CAS 1460-34-0
type: UnificationXref
id: 1460-34-0
db: CAS

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

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

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

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

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

Reference/PubChem-compound 439869
type: UnificationXref
id: 439869
db: PubChem-compound

Reference/UniProt Q9DBF1
type: UnificationXref
id: Q9DBF1
db: UniProt

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

Reference/ChemSpider 10128175
type: UnificationXref
id: 10128175
db: ChemSpider

Reference/UniProt P61922
type: UnificationXref
id: P61922
db: UniProt

Compound/PW C000769 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Lipoamide
stoichiometric coefficient: 1.0

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

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

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

ChemicalStructure/PW C000112
type: ChemicalStructure
structure format: SMILES
structure data: CC[C@H](C)[C@H](N)C(O)=O

Propanoate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway57711
pathway component: SubPathwayInteraction57711
xref: Reference/PathWhiz PW000149
xref: Reference/SMPDB SMP0000016
name: SubPathway

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

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

Hydroxymethylglutaryl-CoA lyase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P38060
organism: Mus musculus

Reference/PubChem-compound 3036931
type: UnificationXref
id: 3036931
db: PubChem-compound

(S)-3-Hydroxyisobutyric acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: (S)-3-Hydroxyisobutyric acid

Reference/ChemSpider 6067
type: UnificationXref
id: 6067
db: ChemSpider

3-Methyl-1-hydroxybutyl-ThPP
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 3-Methyl-1-hydroxybutyl-ThPP

Reference/PubChem-compound 440873
type: UnificationXref
id: 440873
db: PubChem-compound

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

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

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

ChemicalStructure/PW C001455
type: ChemicalStructure
structure format: SMILES
structure data: C[C@@H](CN)C(O)=O

Short-chain specific acyl-CoA dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Short-chain specific acyl-CoA dehydrogenase, mitochondrial

Reference/ChEBI CHEBI 48522
type: UnificationXref
id: CHEBI:48522
db: ChEBI

Compound/PW C002977 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Methyl-1-hydroxypropyl-ThPP
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 15449
type: UnificationXref
id: CHEBI:15449
db: ChEBI

Reference/PubChem-compound 6306
type: UnificationXref
id: 6306
db: PubChem-compound

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

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

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

Reference/ChEBI CHEBI 16530
type: UnificationXref
id: CHEBI:16530
db: ChEBI

Reference/BioCyc 2-METHYL-3-HYDROXY-BUTYRYL-COA
type: UnificationXref
id: 2-METHYL-3-HYDROXY-BUTYRYL-COA
db: BioCyc

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

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

3-ketoacyl-CoA thiolase, mitochondrial
type: Complex
component stoichiometry: Protein/13924 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: 3-ketoacyl-CoA thiolase, mitochondrial

Reference/PubChem-compound 439434
type: UnificationXref
id: 439434
db: PubChem-compound

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

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

Alpha-aminoadipic semialdehyde dehydrogenase
type: ProteinReference
xref: Reference/UniProt Q9DBF1
organism: Mus musculus

Aldehyde dehydrogenase family 16 member A1
type: ProteinReference
xref: Reference/UniProt Q571I9
organism: Mus musculus

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

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

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

Reference/UniProt O08756
type: UnificationXref
id: O08756
db: UniProt

Reference/ChEBI CHEBI 33094
type: UnificationXref
id: CHEBI:33094
db: ChEBI

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

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

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

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

ChemicalStructure/PW C000940
type: ChemicalStructure
structure format: SMILES

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

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

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

3-Hydroxy-3-methylglutaryl-CoA
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15467
xref: Reference/ChemSpider 388357
xref: Reference/CAS 1553-55-5
xref: Reference/BioCyc 3-HYDROXY-3-METHYL-GLUTARYL-COA
xref: Reference/KEGG Compound C00356
xref: Reference/HMDB HMDB0001375
xref: Reference/PubChem-compound 439218
chemical formula: C27H44N7O20P3S
molecular weight: 911.1575
structure: ChemicalStructure/PW C001063

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

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

ReactionCatalysis100912
type: Catalysis
controller: Methylmalonyl-CoA mutase, mitochondrial
controlled: 5.4.99.2
control type: ACTIVATION

Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial

Thiamine pyrophosphate
type: SmallMoleculeReference
xref: Reference/BioCyc 2-(alpha-lactyl)-thpp
xref: Reference/ChEBI CHEBI 9532
xref: Reference/CAS 154-87-0
xref: Reference/ChemSpider 1100
xref: Reference/HMDB HMDB0001372
xref: Reference/PubChem-compound 1132
xref: Reference/KEGG Compound C00068
chemical formula: C12H19N4O7P2S
molecular weight: 425.04498
structure: ChemicalStructure/PW C001060

Reference/CAS 15621-60-0
type: UnificationXref
id: 15621-60-0
db: CAS

Reference/PubChem-compound 439218
type: UnificationXref
id: 439218
db: PubChem-compound

Compound/PW C002979 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-(2-Methylpropionyl)-dihydrolipoamide-E
stoichiometric coefficient: 1.0

Protein/13823 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
stoichiometric coefficient: 1.0

Unknown
type: ProteinReference
organism: Mus musculus

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

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

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

Reference/UniProt Q05920
type: UnificationXref
id: Q05920
db: UniProt

ChemicalStructure/PW C000960
type: ChemicalStructure
structure format: SMILES

Reference/ChemSpider 10128135
type: UnificationXref
id: 10128135
db: ChemSpider

Reference/ChEBI CHEBI 15487
type: UnificationXref
id: CHEBI:15487
db: ChEBI

Reference/ChEBI CHEBI 15486
type: UnificationXref
id: CHEBI:15486
db: ChEBI

Reference/ChEBI CHEBI 15488
type: UnificationXref
id: CHEBI:15488
db: ChEBI

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

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

ChemicalStructure/PW C000769
type: ChemicalStructure
structure format: SMILES
structure data: NC(=O)CCCCC1CCSS1

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

Reference/KEGG Compound C00630
type: UnificationXref
id: C00630
db: KEGG Compound

Unknown
type: Protein
cellular location: Cytosol
entity reference: Unknown

Aldehyde dehydrogenase family 16 member A1
type: Protein
cellular location: Mitochondrion
entity reference: Aldehyde dehydrogenase family 16 member A1

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

Compound/PW C002979 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-(2-Methylpropionyl)-dihydrolipoamide-E
stoichiometric coefficient: 1.0

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

Reference/KEGG Compound C00407
type: UnificationXref
id: C00407
db: KEGG Compound

Compound/PW C002976 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Methyl-1-hydroxybutyl-ThPP
stoichiometric coefficient: 1.0

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

3-ketoacyl-CoA thiolase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: 3-ketoacyl-CoA thiolase, mitochondrial

Compound/PW C000769 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Lipoamide
stoichiometric coefficient: 1.0

Compound/PW C000042 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Acetoacetic acid
stoichiometric coefficient: 1.0

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

ChemicalStructure/PW C000782
type: ChemicalStructure
structure format: SMILES
structure data: OC(=N)CCCCC(S)CCS

ChemicalStructure/PW C000540
type: ChemicalStructure
structure format: SMILES
structure data: CC(C)C[C@H](N)C(O)=O

Reference/ChEBI CHEBI 27462
type: UnificationXref
id: CHEBI:27462
db: ChEBI

Reference/ChEBI CHEBI 15467
type: UnificationXref
id: CHEBI:15467
db: ChEBI

Succinyl-CoA:3-ketoacid coenzyme A transferase 2B, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q9ESL0
organism: Mus musculus

Reference/ChemSpider 388491
type: UnificationXref
id: 388491
db: ChemSpider

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

ChemicalStructure/PW C000988
type: ChemicalStructure
structure format: SMILES

Reference/CAS 541-50-4
type: UnificationXref
id: 541-50-4
db: CAS

S-(2-Methylbutanoyl)-dihydrolipoamide
type: SmallMolecule
cellular location: Cytosol
entity reference: S-(2-Methylbutanoyl)-dihydrolipoamide

Compound/PW C000547 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Ketoleucine
stoichiometric coefficient: 1.0

Compound/PW C000012 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: α-Ketoisovaleric acid
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 15476
type: UnificationXref
id: CHEBI:15476
db: ChEBI

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

Reference/ChEBI CHEBI 15478
type: UnificationXref
id: CHEBI:15478
db: ChEBI

Reference/ChEBI CHEBI 15477
type: UnificationXref
id: CHEBI:15477
db: ChEBI

Reference/ChEBI CHEBI 15479
type: UnificationXref
id: CHEBI:15479
db: ChEBI

Aldehyde dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/13680 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: Aldehyde dehydrogenase family 16 member A1

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

Compound/PW C002980 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-(2-Methylbutanoyl)-dihydrolipoamide
stoichiometric coefficient: 1.0

Compound/PW C001455 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: (S)-beta-Aminoisobutyric acid
stoichiometric coefficient: 1.0

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

Compound/PW C000769 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Lipoamide
stoichiometric coefficient: 1.0

Reference/CAS 6244-91-3
type: UnificationXref
id: 6244-91-3
db: CAS

Compound/PW C001047 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Methyl-3-hydroxybutyryl-CoA
stoichiometric coefficient: 1.0