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

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

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

Mitochondrial pyruvate carrier 1
type: ProteinReference
xref: Reference/UniProt Q9Y5U8
organism: Homo sapiens

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

Reference/KEGG Compound C00311
type: UnificationXref
id: C00311
db: KEGG Compound

Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
type: ProteinReference
xref: Reference/UniProt P31040
organism: Homo sapiens

Reference/ChEBI CHEBI 30797
type: UnificationXref
id: CHEBI:30797
db: ChEBI

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

Compound/PW C001006 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: QH2
stoichiometric coefficient: 1.0

Reference/CAS 110-17-8
type: UnificationXref
id: 110-17-8
db: CAS

Reference/HMDB HMDB0000094
type: UnificationXref
id: HMDB0000094
db: HMDB

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

Compound/PW C000101 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Malic acid
stoichiometric coefficient: 1.0

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

Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial

Reference/BioCyc OXALACETIC ACID
type: UnificationXref
id: OXALACETIC_ACID
db: BioCyc

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

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

Protein/116 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
stoichiometric coefficient: 1.0

Protein/806 Mitochondrial Matrix/Stoichiometry/4.0
type: Stoichiometry
physical entity: Fumarate hydratase, mitochondrial
stoichiometric coefficient: 4.0

Reference/HMDB HMDB0001072
type: UnificationXref
id: HMDB0001072
db: HMDB

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

Reference/PathWhiz PW000046
type: UnificationXref
id: PW000046
db: PathWhiz

Reference/PathWhiz PW000165
type: UnificationXref
id: PW000165
db: PathWhiz

Reference/SMPDB SMP0000547
type: UnificationXref
id: SMP0000547
db: SMPDB

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

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

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

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

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

Reference/PathWhiz PW000163
type: UnificationXref
id: PW000163
db: PathWhiz

Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
type: Protein
cellular location: Mitochondrial Inner Membrane
entity reference: Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial

Reference/PathWhiz PW000162
type: UnificationXref
id: PW000162
db: PathWhiz

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

Protein/840 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial
stoichiometric coefficient: 1.0

Reference/PubChem-compound 5281915
type: UnificationXref
id: 5281915
db: PubChem-compound

Reference/CAS 97-67-6
type: UnificationXref
id: 97-67-6
db: CAS

Reference/PathWhiz PW000155
type: UnificationXref
id: PW000155
db: PathWhiz

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

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

Reference/ChemSpider 945
type: UnificationXref
id: 945
db: ChemSpider

Reference/PathWhiz PW000152
type: UnificationXref
id: PW000152
db: PathWhiz

Reference/PathWhiz PW000030
type: UnificationXref
id: PW000030
db: PathWhiz

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

Guanosine diphosphate
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001201
xref: Reference/BioCyc GDP-4-DEHYDRO-6-DEOXY-D-MANNOSE
xref: Reference/CAS 146-91-8
xref: Reference/PubChem-compound 8977
xref: Reference/ChEBI CHEBI 17552
xref: Reference/ChemSpider 8630
xref: Reference/KEGG Compound C00035
chemical formula: C10H15N5O11P2
molecular weight: 443.02432
structure: ChemicalStructure/PW C000936

Compound/PW C000063 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Citric acid
stoichiometric coefficient: 1.0

ChemicalStructure/PW C001099
type: ChemicalStructure
structure format: SMILES

Reference/ChEBI CHEBI 30769
type: UnificationXref
id: CHEBI:30769
db: ChEBI

Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
type: ProteinReference
xref: Reference/UniProt O14521
organism: Homo sapiens

Reference/ChEBI CHEBI 30887
type: UnificationXref
id: CHEBI:30887
db: ChEBI

Compound/PW C000125 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Isocitric acid
stoichiometric coefficient: 1.0

Reference/PathWhiz PW000029
type: UnificationXref
id: PW000029
db: PathWhiz

ChemicalStructure/PW C000808
type: ChemicalStructure
structure format: SMILES

SubPathwayInteraction356
type: Interaction
name: SubPathway356Reaction
name: SubPathwayReaction
participant: Oxalacetic acid

SubPathwayInteraction357
type: Interaction
name: SubPathwayReaction
name: SubPathway357Reaction
participant: Oxalacetic acid

SubPathwayInteraction354
type: Interaction
name: SubPathwayReaction
name: SubPathway354Reaction
participant: Pyruvic acid

SubPathwayInteraction355
type: Interaction
name: SubPathway355Reaction
name: SubPathwayReaction
participant: Oxalacetic acid

Guanosine triphosphate
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15996
xref: Reference/ChemSpider 6569
xref: Reference/KEGG Compound C00044
xref: Reference/PubChem-compound 6830
xref: Reference/HMDB HMDB0001273
xref: Reference/CAS 86-01-1
xref: Reference/BioCyc GTP
chemical formula: C10H16N5O14P3
molecular weight: 522.99066
structure: ChemicalStructure/PW C000986

SubPathwayInteraction358
type: Interaction
name: SubPathwayReaction
name: SubPathway358Reaction
participant: Oxalacetic acid

Protein/145 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
stoichiometric coefficient: 1.0

SubPathwayInteraction359
type: Interaction
name: SubPathway359Reaction
name: SubPathwayReaction
participant: Oxalacetic acid

Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial

Reference/ChemSpider 1161
type: UnificationXref
id: 1161
db: ChemSpider

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

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

Reference/UniProt O43837
type: UnificationXref
id: O43837
db: UniProt

Reaction390
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000101 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000148 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Mitochondrial Matrix/Stoichiometry/1.0
right: Oxalacetic acid
right: NADH
right: Hydrogen Ion
spontaneous: false
left: L-Malic acid
left: NAD
name: L-Malic acid + NAD ↔ Hydrogen Ion + NADH + Oxalacetic acid
conversion direction: REVERSIBLE

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

SubPathwayInteraction352
type: Interaction
name: SubPathwayReaction
name: SubPathway352Reaction
participant: Pyruvic acid

SubPathwayInteraction353
type: Interaction
name: SubPathway353Reaction
name: SubPathwayReaction
participant: Pyruvic acid

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

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

Succinate dehydrogenase
type: Complex
component stoichiometry: Protein/116 Mitochondrial Inner Membrane/Stoichiometry/1.0
component stoichiometry: Protein/145 Mitochondrial Inner Membrane/Stoichiometry/1.0
component stoichiometry: Protein/174 Mitochondrial Inner Membrane/Stoichiometry/1.0
component stoichiometry: Protein/188 Mitochondrial Inner Membrane/Stoichiometry/1.0
cellular location: Mitochondrial Inner Membrane
component: Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
component: Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
component: Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
component: Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial

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

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

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

Reference/KEGG Compound C00122
type: UnificationXref
id: C00122
db: KEGG Compound

ChemicalStructure/PW C000932
type: ChemicalStructure
structure format: SMILES

Protein/19 Mitochondrial Matrix/Stoichiometry/4.0
type: Stoichiometry
physical entity: Pyruvate carboxylase, mitochondrial
stoichiometric coefficient: 4.0

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

ChemicalStructure/PW C000936
type: ChemicalStructure
structure format: SMILES

Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q99643
organism: Homo sapiens

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

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

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

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

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

Reference/ChemSpider 1031
type: UnificationXref
id: 1031
db: ChemSpider

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

Protein/53 Mitochondrial Matrix/Stoichiometry/60.0
type: Stoichiometry
physical entity: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
stoichiometric coefficient: 60.0

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

Mitochondrial Inner Membrane
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005743

Reference/KEGG Compound C11378
type: UnificationXref
id: C11378
db: KEGG Compound

SubPathwayInteraction378
type: Interaction
name: SubPathway378Reaction
name: SubPathwayReaction
participant: Acetyl-CoA

Coenzyme Q10
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001072
xref: Reference/BioCyc UBIQUINONE-10
xref: Reference/PubChem-compound 5281915
xref: Reference/ChemSpider 4445197
xref: Reference/CAS 303-98-0
xref: Reference/KEGG Compound C11378
xref: Reference/ChEBI CHEBI 46245
chemical formula: C59H90O4
molecular weight: 862.6839
structure: ChemicalStructure/PW C000846

SubPathwayInteraction376
type: Interaction
name: SubPathwayReaction
name: SubPathway376Reaction
participant: Acetyl-CoA

SubPathwayInteraction377
type: Interaction
name: SubPathwayReaction
name: SubPathway377Reaction
participant: Acetyl-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

Pyruvic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Pyruvic acid
name: SubPathwayOutput
name: SubPathwayInput

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

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

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

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

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

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

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

FAD
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: FAD

Compound/PW C000174 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Succinic acid
stoichiometric coefficient: 1.0

SubPathwayInteraction370
type: Interaction
name: SubPathway370Reaction
name: SubPathwayReaction
participant: Oxoglutaric acid

SubPathwayInteraction371
type: Interaction
name: SubPathwayReaction
name: SubPathway371Reaction
participant: Acetyl-CoA

SubPathwayInteraction374
type: Interaction
name: SubPathway374Reaction
name: SubPathwayReaction
participant: Acetyl-CoA

SubPathwayInteraction375
type: Interaction
name: SubPathway375Reaction
name: SubPathwayReaction
participant: Acetyl-CoA

FADH
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: FADH
name: SubPathwayInput

SubPathwayInteraction372
type: Interaction
name: SubPathwayReaction
name: SubPathway372Reaction
participant: Acetyl-CoA

SubPathwayInteraction373
type: Interaction
name: SubPathway373Reaction
name: SubPathwayReaction
participant: Acetyl-CoA

SubPathwayInteraction367
type: Interaction
name: SubPathway367Reaction
name: SubPathwayReaction
participant: Oxoglutaric acid

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 dehydrogenase E1 component subunit beta, mitochondrial
type: ProteinReference
xref: Reference/UniProt P11177
organism: Homo sapiens

SubPathwayInteraction368
type: Interaction
name: SubPathway368Reaction
name: SubPathwayReaction
participant: Oxoglutaric acid

SubPathwayInteraction365
type: Interaction
name: SubPathwayReaction
name: SubPathway365Reaction
participant: FADH

Reference/KEGG Compound C00149
type: UnificationXref
id: C00149
db: KEGG Compound

SubPathwayInteraction366
type: Interaction
name: SubPathwayReaction
name: SubPathway366Reaction
participant: Oxoglutaric acid

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

SubPathwayInteraction369
type: Interaction
name: SubPathwayReaction
name: SubPathway369Reaction
participant: Oxoglutaric acid

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

Reference/KEGG Compound C00022
type: UnificationXref
id: C00022
db: KEGG Compound

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

Pyruvate carboxylase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P11498
organism: Homo sapiens

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

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

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

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

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

Reference/UniProt O14521
type: UnificationXref
id: O14521
db: UniProt

Reference/KEGG Compound C00390
type: UnificationXref
id: C00390
db: KEGG Compound

SubPathwayInteraction360
type: Interaction
name: SubPathway360Reaction
name: SubPathwayReaction
participant: Fumaric acid

SubPathwayInteraction363
type: Interaction
name: SubPathwayReaction
name: SubPathway363Reaction
participant: Fumaric acid

SubPathwayInteraction364
type: Interaction
name: SubPathway364Reaction
name: SubPathwayReaction
participant: Succinic acid

SubPathwayInteraction361
type: Interaction
name: SubPathway361Reaction
name: SubPathwayReaction
participant: Fumaric acid

SubPathwayInteraction362
type: Interaction
name: SubPathway362Reaction
name: SubPathwayReaction
participant: Fumaric acid

Compound/PW C000063 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Citric acid
stoichiometric coefficient: 1.0

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

Reference/CAS 56275-39-9
type: UnificationXref
id: 56275-39-9
db: CAS

Pyruvate carboxylase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Pyruvate carboxylase, mitochondrial

Reference/KEGG Compound C00158
type: UnificationXref
id: C00158
db: KEGG Compound

Reference/KEGG Compound C00036
type: UnificationXref
id: C00036
db: KEGG Compound

Transport1
type: Transport
participant stoichiometry: Compound/PW C000164 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000164 Mitochondrial Matrix/Stoichiometry/1.0
right: Pyruvic acid
spontaneous: false
left: Pyruvic acid
name: Pyruvic acid (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrial Matrix
conversion direction: LEFT_TO_RIGHT

Reference/KEGG Compound C00035
type: UnificationXref
id: C00035
db: KEGG Compound

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

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

Reference/HMDB HMDB0000134
type: UnificationXref
id: HMDB0000134
db: HMDB

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

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

Reference/UniProt P50213
type: UnificationXref
id: P50213
db: UniProt

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

ChemicalStructure/PW C000051
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)C\C(=C\C(O)=O)C(O)=O

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

Reference/UniProt P07954
type: UnificationXref
id: P07954
db: UniProt

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

Reference/KEGG Compound C00044
type: UnificationXref
id: C00044
db: KEGG Compound

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

Reference/ChemSpider 4445197
type: UnificationXref
id: 4445197
db: ChemSpider

Reference/CAS 303-98-0
type: UnificationXref
id: 303-98-0
db: CAS

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

ChemicalStructure/PW C001034
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C000063
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CC(O)(CC(O)=O)C(O)=O

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

Reference/UniProt P51553
type: UnificationXref
id: P51553
db: UniProt

cis-Aconitic acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: cis-Aconitic acid

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

Reference/ChemSpider 10197150
type: UnificationXref
id: 10197150
db: ChemSpider

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

ChemicalStructure/PW C000148
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CC(=O)C(O)=O

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

Aconitate hydratase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q99798
organism: Homo sapiens

Reference/ChEBI CHEBI 18012
type: UnificationXref
id: CHEBI:18012
db: ChEBI

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

Reference/HMDB HMDB0000156
type: UnificationXref
id: HMDB0000156
db: HMDB

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

Reference/UniProt Q99798
type: UnificationXref
id: Q99798
db: UniProt

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

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

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

Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial

Reference/UniProt P21912
type: UnificationXref
id: P21912
db: UniProt

Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
type: Protein
cellular location: Mitochondrial Inner Membrane
entity reference: Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial

Protein/52 Mitochondrial Matrix/Stoichiometry/24.0
type: Stoichiometry
physical entity: Dihydrolipoyl dehydrogenase, mitochondrial
stoichiometric coefficient: 24.0

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

ChemicalStructure/PW C001006
type: ChemicalStructure
structure format: SMILES
structure data: COC1=C(O)C(C)=C(C\C=C(/C)CCC=C(C)C)C(O)=C1OC

Reference/ChemSpider 305
type: UnificationXref
id: 305
db: ChemSpider

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

ChemicalStructure/PW C000164
type: ChemicalStructure
structure format: SMILES
structure data: CC(=O)C(O)=O

Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial

6.4.1.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001034 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000148 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001104 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000463 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000164 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Mitochondrial Matrix/Stoichiometry/1.0
right: Oxalacetic acid
right: Phosphate
right: Adenosine diphosphate
spontaneous: false
EC number: 6.4.1.1
left: Pyruvic acid
left: Adenosine triphosphate
left: Hydrogen carbonate
name: Adenosine triphosphate + Hydrogen carbonate + Pyruvic acid → Adenosine diphosphate + Oxalacetic acid + Phosphate
conversion direction: LEFT_TO_RIGHT

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

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

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

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 C00080
type: UnificationXref
id: C00080
db: KEGG Compound

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

Glutamate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway367
pathway component: SubPathwayInteraction367
xref: Reference/SMPDB SMP0000072
xref: Reference/PathWhiz PW000003
name: SubPathway

Ketone Body Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway375
pathway component: SubPathwayInteraction375
xref: Reference/SMPDB SMP0000071
xref: Reference/PathWhiz PW000028
name: SubPathway

Reference/UniProt Q9Y5U8
type: UnificationXref
id: Q9Y5U8
db: UniProt

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

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

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

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

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

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

Reference/BioCyc QH2
type: UnificationXref
id: QH2
db: BioCyc

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

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

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

Reference/CAS 585-84-2
type: UnificationXref
id: 585-84-2
db: CAS

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

Protein/4308 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial
stoichiometric coefficient: 1.0

Reference/UniProt P11177
type: UnificationXref
id: P11177
db: UniProt

Reference/HMDB HMDB0001273
type: UnificationXref
id: HMDB0001273
db: HMDB

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

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

Succinic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Succinic acid
name: SubPathwayInput

Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
type: ProteinReference
xref: Reference/UniProt P36957
organism: Homo sapiens

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

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

Protein/882 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
stoichiometric coefficient: 1.0

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

Succinic acid
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: Succinic acid

Reference/HMDB HMDB0000193
type: UnificationXref
id: HMDB0000193
db: HMDB

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

Reference/HMDB HMDB0000072
type: UnificationXref
id: HMDB0000072
db: HMDB

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

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

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

Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial

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

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

Reference/PubChem-compound 970
type: UnificationXref
id: 970
db: PubChem-compound

Reference/BioCyc CIS-ACONITATE
type: UnificationXref
id: CIS-ACONITATE
db: BioCyc

ChemicalStructure/PW C000088
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)\C=C\C(O)=O

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

Reference/UniProt Q99643
type: UnificationXref
id: Q99643
db: UniProt

Reference/UniProt O75390
type: UnificationXref
id: O75390
db: UniProt

Reference/CAS 146-91-8
type: UnificationXref
id: 146-91-8
db: CAS

Citrate synthase, mitochondrial
type: Complex
component stoichiometry: Protein/41 Mitochondrial Matrix/Stoichiometry/2.0
cellular location: Mitochondrial Matrix
component: Citrate synthase, mitochondrial

L-Malic acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000156
xref: Reference/ChEBI CHEBI 30797
xref: Reference/KEGG Compound C00149
xref: Reference/ChemSpider 193317
xref: Reference/PubChem-compound 222656
xref: Reference/CAS 97-67-6
chemical formula: C4H6O5
molecular weight: 134.02153
structure: ChemicalStructure/PW C000101

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: CH2O3
molecular weight: 62.000393
structure: ChemicalStructure/PW C000463

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

Oxalacetic acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000223
xref: Reference/CAS 328-42-7
xref: Reference/ChEBI CHEBI 30744
xref: Reference/BioCyc OXALACETIC ACID
xref: Reference/ChemSpider 945
xref: Reference/PubChem-compound 970
xref: Reference/KEGG Compound C00036
chemical formula: C4H4O5
molecular weight: 132.00587
structure: ChemicalStructure/PW C000148

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

Succinyl-CoA ligase
type: Complex
component stoichiometry: Protein/834 Mitochondrial Matrix/Stoichiometry/1.0
component stoichiometry: Protein/832 Mitochondrial Matrix/Stoichiometry/1.0
cellular location: Mitochondrial Matrix
component: Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial
component: Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial

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

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

Valine, Leucine, and Isoleucine Degradation
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway371
pathway component: SubPathwayInteraction371
xref: Reference/SMPDB SMP0000032
xref: Reference/PathWhiz PW000051
name: SubPathway

Protein/52 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Dihydrolipoyl dehydrogenase, mitochondrial
stoichiometric coefficient: 2.0

Oxidation of Branched-Chain Fatty Acids
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway377
pathway component: SubPathwayInteraction377
xref: Reference/PathWhiz PW000155
xref: Reference/SMPDB SMP0000030
name: SubPathway

Reference/PubChem-compound 21883788
type: UnificationXref
id: 21883788
db: PubChem-compound

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

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

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

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

Lysine Degradation
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway376
pathway component: SubPathwayInteraction376
xref: Reference/SMPDB SMP0000037
xref: Reference/PathWhiz PW000029
name: SubPathway

Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
type: Protein
cellular location: Mitochondrial Inner Membrane
entity reference: Succinate dehydrogenase cytochrome b560 subunit, mitochondrial

Reference/UniProt P11498
type: UnificationXref
id: P11498
db: UniProt

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

Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q96I99
organism: Homo sapiens

QH2
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001304
xref: Reference/BioCyc QH2
xref: Reference/PubChem-compound 5280344
xref: Reference/KEGG Compound C00390
xref: Reference/CAS 56275-39-9
xref: Reference/ChemSpider 4444052
chemical formula: (C5H8)nC14H20O4
structure: ChemicalStructure/PW C001006

TransportCatalysis6
type: Catalysis
controller: Mitochondrial pyruvate carrier 1
controlled: Transport1
control type: ACTIVATION

Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
type: ProteinReference
xref: Reference/UniProt P53597
organism: Homo sapiens

Compound/PW C000148 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Oxalacetic acid
stoichiometric coefficient: 1.0

Reference/CAS 328-42-7
type: UnificationXref
id: 328-42-7
db: CAS

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

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

Glycolysis
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway353
pathway component: SubPathwayInteraction353
xref: Reference/SMPDB SMP0000040
xref: Reference/PathWhiz PW000146
name: SubPathway

Protein/41 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Citrate synthase, mitochondrial
stoichiometric coefficient: 2.0

ChemicalStructure/PW C000101
type: ChemicalStructure
structure format: SMILES
structure data: O[C@@H](CC(O)=O)C(O)=O

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

Compound/PW C000936 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Guanosine diphosphate
stoichiometric coefficient: 1.0

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

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

Pyruvic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 1060
xref: Reference/HMDB HMDB0000243
xref: Reference/CAS 127-17-3
xref: Reference/BioCyc PYRUVATE
xref: Reference/KEGG Compound C00022
xref: Reference/ChEBI CHEBI 32816
xref: Reference/ChemSpider 1031
chemical formula: C3H4O3
molecular weight: 88.016045
structure: ChemicalStructure/PW C000164

Reference/ChEBI CHEBI 46245
type: UnificationXref
id: CHEBI:46245
db: ChEBI

Compound/PW C000986 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Guanosine triphosphate
stoichiometric coefficient: 1.0

Isocitric acid
type: SmallMoleculeReference
xref: Reference/CAS 320-77-4
xref: Reference/BioCyc threo-d(s)-iso-citrate
xref: Reference/ChEBI CHEBI 30887
xref: Reference/ChemSpider 1161
xref: Reference/HMDB HMDB0000193
xref: Reference/KEGG Compound C00311
xref: Reference/PubChem-compound 1198
chemical formula: C6H8O7
molecular weight: 192.02701
structure: ChemicalStructure/PW C000125

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

Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial
type: ProteinReference
xref: Reference/UniProt P50213
organism: Homo sapiens

Oxoglutarate dehydrogenase complex
type: Complex
component stoichiometry: Protein/52 Mitochondrial Matrix/Stoichiometry/2.0
component stoichiometry: Protein/4308 Mitochondrial Matrix/Stoichiometry/1.0
component stoichiometry: Protein/882 Mitochondrial Matrix/Stoichiometry/1.0
cellular location: Mitochondrial Matrix
component: Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial
component: Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
component: Dihydrolipoyl dehydrogenase, mitochondrial

Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
type: ProteinReference
xref: Reference/UniProt P51553
organism: Homo sapiens

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

Alanine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway357
pathway order: SubPathwayInteractionSteps/SubPathway368
pathway component: SubPathwayInteraction357
pathway component: SubPathwayInteraction368
xref: Reference/PathWhiz PW000001
xref: Reference/SMPDB SMP0000055
name: SubPathway

Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial
type: ProteinReference
xref: Reference/UniProt O43837
organism: Homo sapiens

Fatty Acid Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway374
pathway component: SubPathwayInteraction374
xref: Reference/SMPDB SMP0000051
xref: Reference/PathWhiz PW000023
name: SubPathway

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

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

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

Urea Cycle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway363
pathway component: SubPathwayInteraction363
xref: Reference/SMPDB SMP0000059
xref: Reference/PathWhiz PW000162
name: SubPathway

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

Pyruvate dehydrogenase complex
type: Complex
component stoichiometry: Protein/52 Mitochondrial Matrix/Stoichiometry/24.0
component stoichiometry: Protein/12 Mitochondrial Matrix/Stoichiometry/60.0
component stoichiometry: Protein/53 Mitochondrial Matrix/Stoichiometry/60.0
component stoichiometry: Protein/44 Mitochondrial Matrix/Stoichiometry/60.0
cellular location: Mitochondrial Matrix
component: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
component: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
component: Dihydrolipoyl dehydrogenase, mitochondrial
component: Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial

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

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: H3O4P
molecular weight: 97.9769
structure: ChemicalStructure/PW C001104

Reference/PubChem-compound 6830
type: UnificationXref
id: 6830
db: PubChem-compound

Reference/CAS 77-92-9
type: UnificationXref
id: 77-92-9
db: CAS

Reference/ChemSpider 558863
type: UnificationXref
id: 558863
db: ChemSpider

Aspartate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway358
pathway order: SubPathwayInteractionSteps/SubPathway360
pathway component: SubPathwayInteraction358
pathway component: SubPathwayInteraction360
xref: Reference/PathWhiz PW000002
xref: Reference/SMPDB SMP0000067
name: SubPathway

Tryptophan Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway372
pathway component: SubPathwayInteraction372
xref: Reference/PathWhiz PW000163
xref: Reference/SMPDB SMP0000063
name: SubPathway

Compound/PW C000051 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: cis-Aconitic acid
stoichiometric coefficient: 1.0

Pyruvate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway359
pathway order: SubPathwayInteractionSteps/SubPathway354
pathway order: SubPathwayInteractionSteps/SubPathway373
pathway component: SubPathwayInteraction354
pathway component: SubPathwayInteraction359
pathway component: SubPathwayInteraction373
xref: Reference/SMPDB SMP0000060
xref: Reference/PathWhiz PW000054
name: SubPathway

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

Reference/UniProt P40925
type: UnificationXref
id: P40925
db: UniProt

Reference/PubChem-compound 643757
type: UnificationXref
id: 643757
db: PubChem-compound

Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial

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

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

Reference/SMPDB SMP0000040
type: UnificationXref
id: SMP0000040
db: SMPDB

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

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

ChemicalStructure/PW C001104
type: ChemicalStructure
structure format: SMILES
structure data: OP(O)(O)=O

Reference/HMDB HMDB0001304
type: UnificationXref
id: HMDB0001304
db: HMDB

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

Reference/UniProt Q96HY7
type: UnificationXref
id: Q96HY7
db: UniProt

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

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

Guanosine diphosphate
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Guanosine diphosphate

Mitochondrial Electron Transport Chain
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway365
pathway component: SubPathwayInteraction365
xref: Reference/PathWhiz PW000165
xref: Reference/SMPDB SMP0000355
name: SubPathway

ReactionCatalysis69
type: Catalysis
controller: Pyruvate dehydrogenase complex
controlled: 1.2.4.1
control type: ACTIVATION

4.2.1.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000063 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000051 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
right: Water
right: cis-Aconitic acid
spontaneous: false
EC number: 4.2.1.3
left: Citric acid
name: Citric acid ↔ Water + cis-Aconitic acid
conversion direction: REVERSIBLE

Compound/PW C000088 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Fumaric acid
stoichiometric coefficient: 1.0

ReactionCatalysis4
type: Catalysis
controller: Pyruvate carboxylase, mitochondrial
controlled: 6.4.1.1
control type: ACTIVATION

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

ReactionCatalysis63
type: Catalysis
controller: Aconitate hydratase, mitochondrial
controlled: 4.2.1.3
control type: ACTIVATION

ReactionCatalysis64
type: Catalysis
controller: Aconitate hydratase, mitochondrial
controlled: 4.2.1.3
control type: ACTIVATION

ReactionCatalysis62
type: Catalysis
controller: Citrate synthase, mitochondrial
controlled: 2.3.3.1
control type: ACTIVATION

ReactionCatalysis67
type: Catalysis
controller: Oxoglutarate dehydrogenase complex
controlled: 1.2.4.2
control type: ACTIVATION

ReactionCatalysis68
type: Catalysis
controller: Succinyl-CoA ligase
controlled: Reaction593
control type: ACTIVATION

Reference/SMPDB SMP0000051
type: UnificationXref
id: SMP0000051
db: SMPDB

Reference/HMDB HMDB0000223
type: UnificationXref
id: HMDB0000223
db: HMDB

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

Reference/BioCyc GDP-4-DEHYDRO-6-DEOXY-D-MANNOSE
type: UnificationXref
id: GDP-4-DEHYDRO-6-DEOXY-D-MANNOSE
db: BioCyc

4.2.1.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000125 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000051 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
right: Isocitric acid
spontaneous: false
EC number: 4.2.1.3
left: Water
left: cis-Aconitic acid
name: Water + cis-Aconitic acid ↔ Isocitric acid
conversion direction: REVERSIBLE

Reference/UniProt Q96I99
type: UnificationXref
id: Q96I99
db: UniProt

Compound/PW C000164 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvic acid
stoichiometric coefficient: 1.0

Malate-Aspartate Shuttle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway355
pathway order: SubPathwayInteractionSteps/SubPathway366
pathway component: SubPathwayInteraction355
pathway component: SubPathwayInteraction366
xref: Reference/SMPDB SMP0000129
xref: Reference/PathWhiz PW000030
name: SubPathway

Reference/SMPDB SMP0000059
type: UnificationXref
id: SMP0000059
db: SMPDB

Compound/PW C000088 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Fumaric acid
stoichiometric coefficient: 1.0

Gluconeogenesis
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway369
pathway order: SubPathwayInteractionSteps/SubPathway356
pathway order: SubPathwayInteractionSteps/SubPathway352
pathway component: SubPathwayInteraction356
pathway component: SubPathwayInteraction369
pathway component: SubPathwayInteraction352
xref: Reference/SMPDB SMP0000128
xref: Reference/PathWhiz PW000152
name: SubPathway

Fumaric acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000134
xref: Reference/ChemSpider 10197150
xref: Reference/CAS 110-17-8
xref: Reference/PubChem-compound 21883788
xref: Reference/KEGG Compound C00122
xref: Reference/BioCyc FUM
xref: Reference/ChEBI CHEBI 18012
chemical formula: C4H4O4
molecular weight: 116.010956
structure: ChemicalStructure/PW C000088

Tyrosine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway361
pathway component: SubPathwayInteraction361
xref: Reference/PathWhiz PW000142
xref: Reference/SMPDB SMP0000006
name: SubPathway

Glucose-Alanine Cycle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway370
pathway component: SubPathwayInteraction370
xref: Reference/PathWhiz PW000046
xref: Reference/SMPDB SMP0000127
name: SubPathway

Reference/SMPDB SMP0000055
type: UnificationXref
id: SMP0000055
db: SMPDB

Reference/UniProt P31040
type: UnificationXref
id: P31040
db: UniProt

Aconitate hydratase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Aconitate hydratase, mitochondrial

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

Reference/PathWhiz PW000523
type: UnificationXref
id: PW000523
db: PathWhiz

Fumarate hydratase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P07954
organism: Homo sapiens

Malate dehydrogenase, cytoplasmic
type: ProteinReference
xref: Reference/UniProt P40925
organism: Homo sapiens

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

ReactionCatalysis75
type: Catalysis
controller: Succinate dehydrogenase
controlled: 1.3.5.1
control type: ACTIVATION

ReactionCatalysis78
type: Catalysis
controller: Fumarate hydratase, mitochondrial
controlled: 4.2.1.2
control type: ACTIVATION

Mitochondrial pyruvate carrier 1
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Mitochondrial pyruvate carrier 1

Reference/ChemSpider 4444052
type: UnificationXref
id: 4444052
db: ChemSpider

ReactionCatalysis79
type: Catalysis
controller: Malate dehydrogenase, cytoplasmic
controlled: Reaction390
control type: ACTIVATION

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

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

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

Reference/HMDB HMDB0001201
type: UnificationXref
id: HMDB0001201
db: HMDB

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

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

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

ReactionCatalysis70
type: Catalysis
controller: Isocitrate dehydrogenase
controlled: 1.1.1.41
control type: ACTIVATION

Reference/PubChem-compound 5280344
type: UnificationXref
id: 5280344
db: PubChem-compound

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

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

Reference/GENE ONTOLOGY GO 0005743
type: UnificationXref
id: GO:0005743
db: GENE ONTOLOGY

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

Propanoate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway378
pathway order: SubPathwayInteractionSteps/SubPathway364
pathway component: SubPathwayInteraction378
pathway component: SubPathwayInteraction364
xref: Reference/PathWhiz PW000149
xref: Reference/SMPDB SMP0000016
name: SubPathway

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

Reference/SMPDB SMP0000063
type: UnificationXref
id: SMP0000063
db: SMPDB

cis-Aconitic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 643757
xref: Reference/KEGG Compound C00417
xref: Reference/HMDB HMDB0000072
xref: Reference/ChEBI CHEBI 32805
xref: Reference/BioCyc CIS-ACONITATE
xref: Reference/ChemSpider 558863
xref: Reference/CAS 585-84-2
chemical formula: C6H6O6
molecular weight: 174.01643
structure: ChemicalStructure/PW C000051

Reference/CAS 320-77-4
type: UnificationXref
id: 320-77-4
db: CAS

Reference/BioCyc threo-d(s)-iso-citrate
type: UnificationXref
id: threo-d(s)-iso-citrate
db: BioCyc

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

Reference/ChEBI CHEBI 15996
type: UnificationXref
id: CHEBI:15996
db: ChEBI

Reference/SMPDB SMP0000072
type: UnificationXref
id: SMP0000072
db: SMPDB

Reference/HMDB HMDB0000243
type: UnificationXref
id: HMDB0000243
db: HMDB

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

Arginine and Proline Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway362
pathway component: SubPathwayInteraction362
xref: Reference/PathWhiz PW000010
xref: Reference/SMPDB SMP0000020
name: SubPathway

Reference/SMPDB SMP0000071
type: UnificationXref
id: SMP0000071
db: SMPDB

Protein/12 Mitochondrial Matrix/Stoichiometry/60.0
type: Stoichiometry
physical entity: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
stoichiometric coefficient: 60.0

ChemicalStructure/PW C000125
type: ChemicalStructure
structure format: SMILES
structure data: OC(C(CC(O)=O)C(O)=O)C(O)=O

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

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

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

Malate dehydrogenase, cytoplasmic
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Malate dehydrogenase, cytoplasmic

Citric acid
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 30769
xref: Reference/BioCyc CIT
xref: Reference/HMDB HMDB0000094
xref: Reference/PubChem-compound 19782904
xref: Reference/KEGG Compound C00158
xref: Reference/CAS 77-92-9
xref: Reference/ChemSpider 305
chemical formula: C6H8O7
molecular weight: 192.02701
structure: ChemicalStructure/PW C000063

Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q96HY7
organism: Homo sapiens

Reference/SMPDB SMP0000128
type: UnificationXref
id: SMP0000128
db: SMPDB

Reference/SMPDB SMP0000129
type: UnificationXref
id: SMP0000129
db: SMPDB

Reference/SMPDB SMP0000006
type: UnificationXref
id: SMP0000006
db: SMPDB

Reference/SMPDB SMP0000127
type: UnificationXref
id: SMP0000127
db: SMPDB

ChemicalStructure/PW C000964
type: ChemicalStructure
structure format: SMILES

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

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

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

Reference/PubChem-compound 19782904
type: UnificationXref
id: 19782904
db: PubChem-compound

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

ChemicalStructure/PW C000846
type: ChemicalStructure
structure format: SMILES
structure data: COC1=C(OC)C(=O)C(C\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CCC=C(C)C)=C(C)C1=O

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

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

1.2.4.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000808 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Mitochondrial Matrix/Stoichiometry/1.0
right: Succinyl-CoA
right: Carbon dioxide
right: NADH
right: Hydrogen Ion
spontaneous: false
EC number: 1.2.4.2
left: Coenzyme A
left: Oxoglutaric acid
left: NAD
name: Coenzyme A + NAD + Oxoglutaric acid → Carbon dioxide + Hydrogen Ion + NADH + Succinyl-CoA
conversion direction: LEFT_TO_RIGHT

2.3.3.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000063 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000148 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000940 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrial Matrix/Stoichiometry/1.0
right: Coenzyme A
right: Citric acid
spontaneous: false
EC number: 2.3.3.1
left: Acetyl-CoA
left: Water
left: Oxalacetic acid
name: Acetyl-CoA + Oxalacetic acid + Water → Citric acid + Coenzyme A
conversion direction: LEFT_TO_RIGHT

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

Compound/PW C000932 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: FADH
stoichiometric coefficient: 1.0

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

1.3.5.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000174 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000932 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000088 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001006 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000964 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000846 Mitochondrial Inner Membrane/Stoichiometry/1.0
right: Fumaric acid
right: FADH
right: QH2
spontaneous: false
EC number: 1.3.5.1
left: Coenzyme Q10
left: Succinic acid
left: FAD
name: Coenzyme Q10 + FAD + Succinic acid ↔ FADH + Fumaric acid + QH2
conversion direction: REVERSIBLE

Reaction593
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000808 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000986 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001104 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000936 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000174 Mitochondrial Matrix/Stoichiometry/1.0
right: Succinic acid
right: Coenzyme A
right: Guanosine triphosphate
spontaneous: false
left: Succinyl-CoA
left: Phosphate
left: Guanosine diphosphate
name: Guanosine diphosphate + Phosphate + Succinyl-CoA ↔ Coenzyme A + Guanosine triphosphate + Succinic acid
conversion direction: REVERSIBLE

1.2.4.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000940 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000164 Mitochondrial Matrix/Stoichiometry/1.0
right: Acetyl-CoA
right: Carbon dioxide
right: NADH
spontaneous: false
EC number: 1.2.4.1
left: Pyruvic acid
left: Coenzyme A
left: NAD
name: Coenzyme A + NAD + Pyruvic acid → Acetyl-CoA + Carbon dioxide + NADH
conversion direction: LEFT_TO_RIGHT

Fumarate hydratase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Fumarate hydratase, mitochondrial

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

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

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

Reference/PubChem-compound 222656
type: UnificationXref
id: 222656
db: PubChem-compound

Fumaric acid
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: Fumaric acid

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

Protein/842 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial
stoichiometric coefficient: 2.0

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

Protein/174 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
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

Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
type: ProteinReference
xref: Reference/UniProt P08559
organism: Homo sapiens

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

Citrate synthase, mitochondrial
type: ProteinReference
xref: Reference/UniProt O75390
organism: Homo sapiens

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

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

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

Homo sapiens
type: BioSource
xref: Reference/TAXONOMY 9606

ChemicalStructure/PW C000940
type: ChemicalStructure
structure format: SMILES

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

Reference/CAS 127-17-3
type: UnificationXref
id: 127-17-3
db: CAS

Coenzyme Q10
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: Coenzyme Q10

Reference/SMPDB SMP0000020
type: UnificationXref
id: SMP0000020
db: SMPDB

Reference/UniProt P36957
type: UnificationXref
id: P36957
db: UniProt

Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
type: ProteinReference
xref: Reference/UniProt P10515
organism: Homo sapiens

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

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

Reference/PubChem-compound 8977
type: UnificationXref
id: 8977
db: PubChem-compound

Protein/843 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
stoichiometric coefficient: 1.0

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

4.2.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000101 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000088 Mitochondrial Matrix/Stoichiometry/1.0
right: Fumaric acid
right: Water
spontaneous: false
EC number: 4.2.1.2
left: L-Malic acid
name: L-Malic acid ↔ Fumaric acid + Water
conversion direction: REVERSIBLE

Reference/SMPDB SMP0000037
type: UnificationXref
id: SMP0000037
db: SMPDB

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

Reference/SMPDB SMP0000030
type: UnificationXref
id: SMP0000030
db: SMPDB

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

Reference/PathWhiz PW000023
type: UnificationXref
id: PW000023
db: PathWhiz

Reference/UniProt P08559
type: UnificationXref
id: P08559
db: UniProt

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

Reference/PathWhiz PW000142
type: UnificationXref
id: PW000142
db: PathWhiz

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

Reference/PathWhiz PW000028
type: UnificationXref
id: PW000028
db: PathWhiz

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

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

Compound/PW C000964 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: FAD
stoichiometric coefficient: 1.0

Reference/PathWhiz PW000146
type: UnificationXref
id: PW000146
db: PathWhiz

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

Reference/ChemSpider 8630
type: UnificationXref
id: 8630
db: ChemSpider

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

Reference/UniProt P10515
type: UnificationXref
id: P10515
db: UniProt

Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
type: ProteinReference
xref: Reference/UniProt P21912
organism: Homo sapiens

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

Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
type: Protein
cellular location: Mitochondrial Inner Membrane
entity reference: Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial

Citrate synthase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Citrate synthase, mitochondrial

Reference/ChEBI CHEBI 32816
type: UnificationXref
id: CHEBI:32816
db: ChEBI

Protein/188 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
stoichiometric coefficient: 1.0

QH2
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: QH2

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

Fumarate hydratase, mitochondrial
type: Complex
component stoichiometry: Protein/806 Mitochondrial Matrix/Stoichiometry/4.0
cellular location: Mitochondrial Matrix
component: Fumarate hydratase, mitochondrial

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

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

Reference/PathWhiz PW000010
type: UnificationXref
id: PW000010
db: PathWhiz

Isocitrate dehydrogenase
type: Complex
component stoichiometry: Protein/840 Mitochondrial Matrix/Stoichiometry/1.0
component stoichiometry: Protein/842 Mitochondrial Matrix/Stoichiometry/2.0
component stoichiometry: Protein/843 Mitochondrial Matrix/Stoichiometry/1.0
cellular location: Mitochondrial Matrix
component: Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
component: Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial
component: Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial

Reference/ChemSpider 6569
type: UnificationXref
id: 6569
db: ChemSpider

ChemicalStructure/PW C000414
type: ChemicalStructure
structure format: SMILES

Protein/44 Mitochondrial Matrix/Stoichiometry/60.0
type: Stoichiometry
physical entity: Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
stoichiometric coefficient: 60.0

Reference/ChEBI CHEBI 17552
type: UnificationXref
id: CHEBI:17552
db: ChEBI

Pyruvate carboxylase, mitochondrial
type: Complex
component stoichiometry: Protein/19 Mitochondrial Matrix/Stoichiometry/4.0
cellular location: Mitochondrial Matrix
component: Pyruvate carboxylase, mitochondrial

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

Reference/ChEBI CHEBI 30744
type: UnificationXref
id: CHEBI:30744
db: ChEBI

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

Reference/ChEBI CHEBI 32805
type: UnificationXref
id: CHEBI:32805
db: ChEBI

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

Reference/PathWhiz PW000001
type: UnificationXref
id: PW000001
db: PathWhiz

Compound/PW C000125 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Isocitric acid
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00417
type: UnificationXref
id: C00417
db: KEGG Compound

Reference/PathWhiz PW000003
type: UnificationXref
id: PW000003
db: PathWhiz

Compound/PW C000846 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Coenzyme Q10
stoichiometric coefficient: 1.0

ChemicalStructure/PW C000986
type: ChemicalStructure
structure format: SMILES

Reference/PubChem-compound 1198
type: UnificationXref
id: 1198
db: PubChem-compound

Protein/832 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
stoichiometric coefficient: 1.0

Protein/834 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial
stoichiometric coefficient: 1.0

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

Reference/ChemSpider 193317
type: UnificationXref
id: 193317
db: ChemSpider

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

1.1.1.41
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001316 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000125 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 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
right: Carbon dioxide
right: Oxoglutaric acid
right: NADH
right: Hydrogen Ion
spontaneous: false
EC number: 1.1.1.41
left: Isocitric acid
left: NAD
name: Isocitric acid + NAD → Carbon dioxide + Hydrogen Ion + NADH + Oxoglutaric acid
conversion direction: LEFT_TO_RIGHT

Reference/CAS 86-01-1
type: UnificationXref
id: 86-01-1
db: CAS

Reference/PubChem-compound 1060
type: UnificationXref
id: 1060
db: PubChem-compound

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

Reference/UniProt P53597
type: UnificationXref
id: P53597
db: UniProt

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

Compound/PW C000164 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvic acid
stoichiometric coefficient: 1.0

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

Reference/SMPDB SMP0000355
type: UnificationXref
id: SMP0000355
db: SMPDB

Fumarase Deficiency
type: Pathway
pathway order: PathwaySubPathway/355
pathway order: PathwaySubPathway/377
pathway order: PathwaySubPathway/356
pathway order: PathwaySubPathway/378
pathway order: PathwaySubPathway/353
pathway order: PathwaySubPathway/375
pathway order: PathwayEnzymeReaction/79
pathway order: PathwaySubPathway/354
pathway order: PathwaySubPathway/376
pathway order: PathwaySubPathway/373
pathway order: PathwaySubPathway/352
pathway order: PathwaySubPathway/374
pathway order: PathwaySubPathway/371
pathway order: PathwaySubPathway/372
pathway order: PathwaySubPathway/359
pathway order: PathwayTransport/478
pathway order: PathwaySubPathway/357
pathway order: PathwaySubPathway/358
pathway order: PathwayEnzymeReaction/80
pathway order: PathwayEnzymeReaction/85
pathway order: PathwaySubPathway/370
pathway order: PathwayEnzymeReaction/88
pathway order: PathwayEnzymeReaction/87
pathway order: PathwaySubPathway/366
pathway order: PathwaySubPathway/367
pathway order: PathwaySubPathway/364
pathway order: PathwaySubPathway/365
pathway order: PathwaySubPathway/362
pathway order: PathwaySubPathway/363
pathway order: PathwaySubPathway/360
pathway order: PathwaySubPathway/361
pathway order: PathwayEnzymeReaction/3441
pathway order: PathwaySubPathway/368
pathway order: PathwayEnzymeReaction/3440
pathway order: PathwaySubPathway/369
pathway order: PathwayEnzymeReaction/90
pathway order: PathwayEnzymeReaction/71
pathway order: PathwayEnzymeReaction/78
pathway order: PathwayEnzymeReaction/77
pathway component: ReactionCatalysis75
pathway component: ReactionCatalysis78
pathway component: ReactionCatalysis79
pathway component: Transport1
pathway component: 1.2.4.2
pathway component: Alanine Metabolism
pathway component: Valine, Leucine, and Isoleucine Degradation
pathway component: 2.3.3.1
pathway component: ReactionCatalysis70
pathway component: Oxidation of Branched-Chain Fatty Acids
pathway component: Reaction390
pathway component: Fatty Acid Metabolism
pathway component: Glutamate Metabolism
pathway component: Ketone Body Metabolism
pathway component: Propanoate Metabolism
pathway component: 1.1.1.41
pathway component: 1.3.5.1
pathway component: Urea Cycle
pathway component: Lysine Degradation
pathway component: Reaction593
pathway component: Mitochondrial Electron Transport Chain
pathway component: 1.2.4.1
pathway component: ReactionCatalysis69
pathway component: 4.2.1.3
pathway component: ReactionCatalysis4
pathway component: ReactionCatalysis63
pathway component: ReactionCatalysis64
pathway component: ReactionCatalysis62
pathway component: ReactionCatalysis67
pathway component: TransportCatalysis6
pathway component: ReactionCatalysis68
pathway component: Aspartate Metabolism
pathway component: Arginine and Proline Metabolism
pathway component: Tryptophan Metabolism
pathway component: Glycolysis
pathway component: 6.4.1.1
pathway component: Pyruvate Metabolism
pathway component: 4.2.1.2
pathway component: Malate-Aspartate Shuttle

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

Reference/BioCyc UBIQUINONE-10
type: UnificationXref
id: UBIQUINONE-10
db: BioCyc