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

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

Compound/PW C000932 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: FADH
stoichiometric coefficient: 1.0

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 446013
type: UnificationXref
id: 446013
db: PubChem-compound

ReactionCatalysis208977
type: Catalysis
controller: Glycine N-acyltransferase
controlled: Reaction219598
control type: ACTIVATION

ReactionCatalysis208976
type: Catalysis
controller: Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
controlled: Reaction219597
control type: ACTIVATION

ReactionCatalysis208975
type: Catalysis
controller: branched-chain alpha-keto dehydrogenase complex
controlled: Reaction219596
control type: ACTIVATION

ReactionCatalysis208974
type: Catalysis
controller: Branched-chain-amino-acid aminotransferase, mitochondrial
controlled: 2.6.1.42
control type: ACTIVATION

Protein/294 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
stoichiometric coefficient: 1.0

Reference/UniProt P12694
type: UnificationXref
id: P12694
db: UniProt

Reference/CAS 35842-45-6
type: UnificationXref
id: 35842-45-6
db: CAS

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

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

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

Compound/PW C000766 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Tiglylglycine
stoichiometric coefficient: 1.0

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

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

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

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

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

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/HMDB HMDB0001197
type: UnificationXref
id: HMDB0001197
db: HMDB

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

branched-chain alpha-keto dehydrogenase complex
type: Complex
component stoichiometry: Protein/52 Mitochondrion/Stoichiometry/1.0
component stoichiometry: Protein/2232 Mitochondrion/Stoichiometry/1.0
component stoichiometry: Protein/293 Mitochondrion/Stoichiometry/1.0
component stoichiometry: Protein/294 Mitochondrion/Stoichiometry/1.0
cellular location: Mitochondrion
component: Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
component: Dihydrolipoyl dehydrogenase, mitochondrial
component: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
component: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

Reference/ChEBI CHEBI 16238
type: UnificationXref
id: CHEBI:16238
db: ChEBI

Reference/UniProt P09622
type: UnificationXref
id: P09622
db: UniProt

Kidney Damage
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway121678
pathway component: SubPathwayInteraction121678
name: SubPathway

Mitochondrial Fatty Acid Beta-Oxidation
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway121677
pathway component: SubPathwayInteraction121677
name: SubPathway

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

Reaction219596
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000373 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000820 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Mitochondrion/Stoichiometry/1.0
right: Carbon dioxide
right: 2-Methylbutyryl-CoA
spontaneous: false
left: 3-Methyl-2-oxovaleric acid
left: NAD
left: Coenzyme A
name: 3-Methyl-2-oxovaleric acid + Coenzyme A + NAD → 2-Methylbutyryl-CoA + Carbon dioxide
conversion direction: LEFT_TO_RIGHT

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

2.6.1.42
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000112 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000373 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Mitochondrion/Stoichiometry/1.0
right: L-Glutamic acid
right: 3-Methyl-2-oxovaleric 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

Protein/293 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
stoichiometric coefficient: 1.0

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

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

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

Reference/ChemSpider 393487
type: UnificationXref
id: 393487
db: ChemSpider

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

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

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

FADH
type: SmallMoleculeReference
xref: Reference/PubChem-compound 446013
xref: Reference/ChEBI CHEBI 17877
xref: Reference/BioCyc FADH2
xref: Reference/KEGG Compound C01352
xref: Reference/ChemSpider 393487
xref: Reference/HMDB HMDB0001197
xref: Reference/CAS 1910-41-4
chemical formula: C27H35N9O15P2
molecular weight: 787.1728
structure: ChemicalStructure/PW C000932

Reaction219598
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000078 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000766 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001051 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001378 Mitochondrion/Stoichiometry/1.0
right: Coenzyme A
right: Tiglylglycine
right: Hydrogen
spontaneous: false
left: Tiglyl-CoA
left: Glycine
name: Glycine + Tiglyl-CoA → Coenzyme A + Hydrogen + Tiglylglycine
conversion direction: LEFT_TO_RIGHT

Reaction219597
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000964 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000932 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001051 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000820 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001378 Mitochondrion/Stoichiometry/1.0
right: FADH
right: Tiglyl-CoA
spontaneous: false
left: 2-Methylbutyryl-CoA
left: FAD
left: Hydrogen
name: 2-Methylbutyryl-CoA + FAD + Hydrogen → FADH + Tiglyl-CoA
conversion direction: LEFT_TO_RIGHT

ChemicalStructure/PW C001099
type: ChemicalStructure
structure format: SMILES

Protein/52 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dihydrolipoyl dehydrogenase, mitochondrial
stoichiometric coefficient: 1.0

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
type: ProteinReference
xref: Reference/UniProt P12694
organism: Homo sapiens

2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
type: ProteinReference
xref: Reference/UniProt P21953
organism: Homo sapiens

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

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

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

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

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

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

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

Reference/UniProt Q6IB77
type: UnificationXref
id: Q6IB77
db: UniProt

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

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

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

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

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

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

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

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

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

Reference/ChemSpider 559059
type: UnificationXref
id: 559059
db: ChemSpider

ChemicalStructure/PW C000932
type: ChemicalStructure
structure format: SMILES

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

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

Reference/CAS 1910-41-4
type: UnificationXref
id: 1910-41-4
db: CAS

Tiglylglycine
type: SmallMolecule
entity reference: Tiglylglycine
name: SubPathwayInput

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

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

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

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

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

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

FAD
type: SmallMoleculeReference
xref: Reference/CAS 146-14-5
xref: Reference/HMDB HMDB0001248
xref: Reference/ChEBI CHEBI 16238
xref: Reference/BioCyc FAD
xref: Reference/PubChem-compound 643975
xref: Reference/ChemSpider 559059
xref: Reference/KEGG Compound C00016
chemical formula: C27H33N9O15P2
molecular weight: 785.1571
structure: ChemicalStructure/PW C000964

NAD
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15846
xref: Reference/HMDB HMDB0000902
xref: Reference/CAS 53-84-9
xref: Reference/KEGG Compound C00003
xref: Reference/PubChem-compound 5893
xref: Reference/BioCyc NAD
xref: Reference/ChemSpider 5682
chemical formula: C21H27N7O14P2
molecular weight: 663.10913
structure: ChemicalStructure/PW C000721

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

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

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

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

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

Reference/KEGG Compound C00016
type: UnificationXref
id: C00016
db: KEGG Compound

Metabolism and Physiological Effects of Tiglylglycine
type: Pathway
pathway order: PathwayEnzymeReaction/2539072
pathway order: PathwayTransport/2539028
pathway order: PathwayEnzymeReaction/2539069
pathway order: PathwaySubPathway/121678
pathway order: PathwayTransport/2539029
pathway order: PathwayEnzymeReaction/2539044
pathway order: PathwayEnzymeReaction/2539066
pathway order: PathwayTransport/2539074
pathway order: PathwayTransport/2539031
pathway order: PathwaySubPathway/121677
pathway component: Kidney Damage
pathway component: Mitochondrial Fatty Acid Beta-Oxidation
pathway component: Reaction219598
pathway component: Reaction219597
pathway component: ReactionCatalysis208977
pathway component: ReactionCatalysis208976
pathway component: ReactionCatalysis208975
pathway component: ReactionCatalysis208974
pathway component: Reaction219596
pathway component: 2.6.1.42
pathway component: Transport16198
pathway component: Transport16194
pathway component: Transport16192
pathway component: Transport16193
xref: Reference/SMPDB SMP0126840
xref: Reference/PathWhiz PW128445
organism: Homo sapiens

Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial

Reference/UniProt P21953
type: UnificationXref
id: P21953
db: UniProt

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

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

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

Reference/ChEBI CHEBI 73018
type: UnificationXref
id: CHEBI:73018
db: ChEBI

Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
type: ProteinReference
xref: Reference/UniProt P11182
organism: Homo sapiens

Reference/KEGG Compound C01352
type: UnificationXref
id: C01352
db: KEGG Compound

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

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

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

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

Glycine N-acyltransferase
type: ProteinReference
xref: Reference/UniProt Q6IB77
organism: Homo sapiens

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

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

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

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

Protein/2232 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
stoichiometric coefficient: 1.0

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

ChemicalStructure/PW C000964
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

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

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

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

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

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

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

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

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

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

Reference/ChemSpider 4945715
type: UnificationXref
id: 4945715
db: ChemSpider

Glycine N-acyltransferase
type: Protein
cellular location: Mitochondrion
entity reference: Glycine N-acyltransferase

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

Reference/ChEBI CHEBI 17877
type: UnificationXref
id: CHEBI:17877
db: ChEBI

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

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

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

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

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

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

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

SubPathwayInteraction121678
type: Interaction
name: SubPathwayReaction
name: SubPathway121678Reaction
participant: Tiglylglycine

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

SubPathwayInteraction121677
type: Interaction
name: SubPathway121677Reaction
name: SubPathwayReaction
participant: Tiglylglycine

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

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

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

Tiglylglycine
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 73018
xref: Reference/CAS 35842-45-6
xref: Reference/ChemSpider 4945715
xref: Reference/HMDB HMDB0000959
xref: Reference/PubChem-compound 6441567
chemical formula: C7H11NO3
molecular weight: 157.0739
structure: ChemicalStructure/PW C000766

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

Reference/PubChem-compound 643975
type: UnificationXref
id: 643975
db: PubChem-compound

Homo sapiens
type: BioSource
xref: Reference/TAXONOMY 9606

ChemicalStructure/PW C000820
type: ChemicalStructure
structure format: SMILES

Reference/UniProt O15382
type: UnificationXref
id: O15382
db: UniProt

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

Reference/HMDB HMDB0001248
type: UnificationXref
id: HMDB0001248
db: HMDB

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

Reference/HMDB HMDB0001362
type: UnificationXref
id: HMDB0001362
db: HMDB

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

Dihydrolipoyl dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P09622
organism: Homo sapiens

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

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

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

Reference/HMDB HMDB0000959
type: UnificationXref
id: HMDB0000959
db: HMDB

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

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

ChemicalStructure/PW C001378
type: ChemicalStructure
structure format: SMILES

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

Transport16198
type: Transport
participant stoichiometry: Compound/PW C000766 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000766/Stoichiometry/1.0
right: Tiglylglycine
spontaneous: false
left: Tiglylglycine
name: Tiglylglycine (→) Transport: Homo sapiens, Cell, Mitochondrion to Homo sapiens, Blood Vessel
conversion direction: LEFT_TO_RIGHT

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

Transport16194
type: Transport
participant stoichiometry: Compound/PW C000112 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000112/Stoichiometry/1.0
right: L-Isoleucine
spontaneous: false
left: L-Isoleucine
name: L-Isoleucine (→) Transport: Homo sapiens, Blood Vessel to Homo sapiens, Cell, Mitochondrion
conversion direction: LEFT_TO_RIGHT

Transport16192
type: Transport
participant stoichiometry: Compound/PW C000112 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000112/Stoichiometry/1.0
right: L-Isoleucine
spontaneous: false
left: L-Isoleucine
name: L-Isoleucine (→) Transport: Homo sapiens to Homo sapiens, Intestine, Epithelial Cell, Cytosol
conversion direction: LEFT_TO_RIGHT

Transport16193
type: Transport
participant stoichiometry: Compound/PW C000112 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000112/Stoichiometry/1.0
right: L-Isoleucine
spontaneous: false
left: L-Isoleucine
name: L-Isoleucine (→) Transport: Homo sapiens, Intestine, Epithelial Cell, Cytosol to Homo sapiens, Blood Vessel
conversion direction: LEFT_TO_RIGHT

Reference/SMPDB SMP0126840
type: UnificationXref
id: SMP0126840
db: SMPDB

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

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

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

Reference/PathWhiz PW128445
type: UnificationXref
id: PW128445
db: PathWhiz

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

ChemicalStructure/PW C000766
type: ChemicalStructure
structure format: SMILES
structure data: C\C=C(/C)C(=O)NCC(O)=O

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

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

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

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

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

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

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

Compound/PW C000766/Stoichiometry/1.0
type: Stoichiometry
physical entity: Tiglylglycine
stoichiometric coefficient: 1.0

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

Branched-chain-amino-acid aminotransferase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Branched-chain-amino-acid aminotransferase, mitochondrial

Reference/CAS 146-14-5
type: UnificationXref
id: 146-14-5
db: CAS

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

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

Reference/PubChem-compound 6441567
type: UnificationXref
id: 6441567
db: PubChem-compound

Compound/PW C000964 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: FAD
stoichiometric coefficient: 1.0

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

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

L-Isoleucine
type: SmallMolecule
entity reference: L-Isoleucine

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

Dihydrolipoyl dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Dihydrolipoyl dehydrogenase, mitochondrial

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

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

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

Reference/BioCyc FADH2
type: UnificationXref
id: FADH2
db: BioCyc

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

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

Reference/UniProt P11182
type: UnificationXref
id: P11182
db: UniProt

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

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

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

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

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

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

Branched-chain-amino-acid aminotransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt O15382
organism: Homo sapiens

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

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