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

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

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

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

Protein/380 Mitochondrion/Stoichiometry/6.0
type: Stoichiometry
physical entity: Enoyl-CoA hydratase, mitochondrial
stoichiometric coefficient: 6.0

Mitochondrial ornithine transporter 2
type: Protein
entity reference: Mitochondrial ornithine transporter 2

Allysine
type: SmallMolecule
entity reference: Allysine

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

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

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

Unknown
type: ProteinReference

Enoyl-CoA hydratase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P30084
organism: Homo sapiens

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

Reference/KEGG Compound C00449
type: UnificationXref
id: C00449
db: KEGG Compound

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

Reference/ChemSpider 388365
type: UnificationXref
id: 388365
db: ChemSpider

Reference/KEGG Compound C00322
type: UnificationXref
id: C00322
db: KEGG Compound

1.3.8.6
type: BiochemicalReaction
participant stoichiometry: Compound/Generic69 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/Generic70 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001345 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001032 Mitochondrion/Stoichiometry/1.0
right: Carbon dioxide
right: Reduced electron-transfer flavoprotein
right: Crotonoyl-CoA
spontaneous: false
EC number: 1.3.8.6
left: electron-transfer flavoprotein
left: Glutaryl-CoA
name: Glutaryl-CoA + electron-transfer flavoprotein → Carbon dioxide + Crotonoyl-CoA + Reduced electron-transfer flavoprotein
conversion direction: LEFT_TO_RIGHT

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

Transport10393
type: Transport
participant stoichiometry: Compound/PW C000976 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000976 Cytoplasm/Stoichiometry/1.0
right: Allysine
spontaneous: false
left: Allysine
name: Allysine (?) Transport: Homo sapiens, Liver, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrion
conversion direction: LEFT_TO_RIGHT

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

Reference/ChemSpider 5747
type: UnificationXref
id: 5747
db: ChemSpider

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

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

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

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

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

Alpha-aminoadipic semialdehyde dehydrogenase
type: ProteinReference
xref: Reference/UniProt P49419
organism: Homo sapiens

L-Lysine
type: SmallMolecule
cellular location: Cytoplasm
entity reference: L-Lysine

Reference/PubChem-compound 440045
type: UnificationXref
id: 440045
db: PubChem-compound

Low affinity cationic amino acid transporter 2
type: Protein
entity reference: Low affinity cationic amino acid transporter 2

Reference/ChemSpider 70
type: UnificationXref
id: 70
db: ChemSpider

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

Compound/PW C000143 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADP
stoichiometric coefficient: 1.0

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

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

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

Reference/UniProt Q9BXI2
type: UnificationXref
id: Q9BXI2
db: UniProt

ChemicalStructure/PW C001099
type: ChemicalStructure
structure format: SMILES

(S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate
type: SmallMoleculeReference
xref: Reference/CAS 3038-89-9
xref: Reference/PubChem-compound 165067
xref: Reference/ChemSpider 144715
xref: Reference/HMDB HMDB0012130
xref: Reference/ChEBI CHEBI 49015
chemical formula: C6H9NO2
molecular weight: 127.06333
structure: ChemicalStructure/PW C007691

Reference/PathWhiz PW127266
type: UnificationXref
id: PW127266
db: PathWhiz

(S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate
type: SmallMolecule
entity reference: (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate

Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
type: Complex
component stoichiometry: Protein/370 Mitochondrion/Stoichiometry/2.0
cellular location: Mitochondrion
component: Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial

Reference/ChEBI CHEBI 17027
type: UnificationXref
id: CHEBI:17027
db: ChEBI

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

Reference/PubChem-compound 165067
type: UnificationXref
id: 165067
db: PubChem-compound

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

Reference/CAS 56-87-1
type: UnificationXref
id: 56-87-1
db: CAS

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

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

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

Reference/ChEBI CHEBI 18009
type: UnificationXref
id: CHEBI:18009
db: ChEBI

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

Reference/UniProt P52569
type: UnificationXref
id: P52569
db: UniProt

SubPathwayInteraction118990
type: Interaction
name: SubPathway118990Reaction
name: SubPathwayReaction
participant: L-Lysine

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

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

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

Acetyl-CoA acetyltransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P24752
organism: Homo sapiens

Peroxisomal sarcosine oxidase
type: ProteinReference
xref: Reference/UniProt Q9P0Z9
organism: Homo sapiens

Reference/CAS 103192-48-9
type: UnificationXref
id: 103192-48-9
db: CAS

Protein/373 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
stoichiometric coefficient: 2.0

SubPathwayInteraction118989
type: Interaction
name: SubPathway118989Reaction
name: SubPathwayReaction
participant: Acetyl-CoA

ChemicalStructure/PW C007691
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)[C@@H]1CCCC=N1

ChemicalStructure/PW C000904
type: ChemicalStructure
structure format: SMILES

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

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

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

Extracellular Space
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005615

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

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

Allysine
type: SmallMoleculeReference
xref: Reference/ChemSpider 202
xref: Reference/ChEBI CHEBI 17027
xref: Reference/PubChem-compound 207
xref: Reference/CAS 1962-83-0
xref: Reference/KEGG Compound C01475
xref: Reference/BioCyc ALLYSINE
xref: Reference/HMDB HMDB0001263
chemical formula: C6H11NO3
molecular weight: 145.0739
structure: ChemicalStructure/PW C000976

Reference/KEGG Compound C01475
type: UnificationXref
id: C01475
db: KEGG Compound

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

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

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

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

Reference/UniProt Q8N5Z0
type: UnificationXref
id: Q8N5Z0
db: UniProt

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

1.2.4.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000150 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001032 Mitochondrion/Stoichiometry/1.0
right: Carbon dioxide
right: Glutaryl-CoA
spontaneous: false
EC number: 1.2.4.2
left: Coenzyme A
left: Oxoadipic acid
name: Coenzyme A + Oxoadipic acid → Carbon dioxide + Glutaryl-CoA
conversion direction: LEFT_TO_RIGHT

Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q16836
organism: Homo sapiens

Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q8N5Z0
organism: Homo sapiens

Reference/CAS 3184-35-8
type: UnificationXref
id: 3184-35-8
db: CAS

(S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate
type: SmallMolecule
cellular location: Peroxisome
entity reference: (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate

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

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial

Reference/CAS 2871-66-1
type: UnificationXref
id: 2871-66-1
db: CAS

ChemicalStructure/PW C001142
type: ChemicalStructure
structure format: SMILES

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

Water
type: SmallMolecule
entity reference: Water

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

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

Reference/SMPDB SMP0125700
type: UnificationXref
id: SMP0125700
db: SMPDB

Compound/PW C000189 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Saccharopine
stoichiometric coefficient: 1.0

Acetyl-CoA acetyltransferase, mitochondrial
type: Complex
component stoichiometry: Protein/36 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: Acetyl-CoA acetyltransferase, mitochondrial

Alpha-aminoadipic semialdehyde synthase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q9UDR5
organism: Homo sapiens

Reference/KEGG Compound C00047
type: UnificationXref
id: C00047
db: KEGG Compound

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

ChemicalStructure/PW C001032
type: ChemicalStructure
structure format: SMILES

Reference/KEGG Compound C03561
type: UnificationXref
id: C03561
db: KEGG Compound

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

ChemicalStructure/PW C000189
type: ChemicalStructure
structure format: SMILES

Reference/CAS 542-32-5
type: UnificationXref
id: 542-32-5
db: CAS

Reference/ChEBI CHEBI 18019
type: UnificationXref
id: CHEBI:18019
db: ChEBI

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

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

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

Reference/HMDB HMDB0012130
type: UnificationXref
id: HMDB0012130
db: HMDB

Reaction818
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000189 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000143 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000118 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000146 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Mitochondrion/Stoichiometry/1.0
right: Saccharopine
right: Water
right: NADP
spontaneous: false
left: L-Lysine
left: Oxoglutaric acid
left: NADPH
name: L-Lysine + NADPH + Oxoglutaric acid → NADP + Saccharopine + Water
conversion direction: LEFT_TO_RIGHT

Reaction819
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000976 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000189 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
right: L-Glutamic acid
right: Allysine
right: NADH
spontaneous: false
left: NAD
left: Saccharopine
left: Water
name: NAD + Saccharopine + Water → Allysine + L-Glutamic acid + NADH
conversion direction: LEFT_TO_RIGHT

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

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

Reference/HMDB HMDB0000279
type: UnificationXref
id: HMDB0000279
db: HMDB

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

ChemicalStructure/PW C000150
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CCCC(=O)C(O)=O

Reference/UniProt Q92947
type: UnificationXref
id: Q92947
db: UniProt

ChemicalStructure/PW C000390
type: ChemicalStructure
structure format: SMILES
structure data: NC(CCCC(O)=O)C(O)=O

Compound/PW C000118 Extracellular Space/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Lysine
stoichiometric coefficient: 1.0

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

Reaction825
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001065 Peroxisome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C007691 Peroxisome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000567 Peroxisome/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001783 Peroxisome/Stoichiometry/1.0
right: (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate
right: Hydrogen peroxide
spontaneous: false
left: Oxygen
left: L-Pipecolic acid
name: L-Pipecolic acid + Oxygen → (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate + Hydrogen peroxide
conversion direction: LEFT_TO_RIGHT

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

Reaction824
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000976/Stoichiometry/1.0
participant stoichiometry: Compound/PW C007691/Stoichiometry/1.0
right: Allysine
spontaneous: true
left: Water
left: (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate
name: (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate + Water ↔ Allysine
conversion direction: REVERSIBLE

Reaction821
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000150 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000390 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Mitochondrion/Stoichiometry/1.0
right: L-Glutamic acid
right: Oxoadipic acid
spontaneous: false
left: Oxoglutaric acid
left: Aminoadipic acid
name: Aminoadipic acid + Oxoglutaric acid ↔ L-Glutamic acid + Oxoadipic acid
conversion direction: REVERSIBLE

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

Reaction820
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000976 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000390 Mitochondrion/Stoichiometry/1.0
right: Aminoadipic acid
right: NADH
spontaneous: false
left: NAD
left: Water
left: Allysine
name: Allysine + NAD + Water → Aminoadipic acid + NADH
conversion direction: LEFT_TO_RIGHT

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

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

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

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

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

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

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

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

Alpha-aminoadipic semialdehyde synthase, mitochondrial
type: Complex
component stoichiometry: Protein/797 Mitochondrion/Stoichiometry/2.0
cellular location: Mitochondrion
component: Alpha-aminoadipic semialdehyde synthase, mitochondrial

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

Reference/HMDB HMDB0001263
type: UnificationXref
id: HMDB0001263
db: HMDB

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

Hyperlysinemia I, Familial
type: Pathway
pathway order: PathwayTransport/2516560
pathway order: PathwayTransport/2516562
pathway order: PathwayTransport/2516561
pathway order: PathwayEnzymeReaction/2516555
pathway order: PathwayEnzymeReaction/2516554
pathway order: PathwayEnzymeReaction/2516553
pathway order: PathwayEnzymeReaction/2516552
pathway order: PathwayEnzymeReaction/2516551
pathway order: PathwayEnzymeReaction/2516550
pathway order: PathwayEnzymeReaction/2516549
pathway order: PathwayEnzymeReaction/2516559
pathway order: PathwayEnzymeReaction/2516558
pathway order: PathwayEnzymeReaction/2516557
pathway order: PathwaySubPathway/118990
pathway order: PathwayEnzymeReaction/2516556
pathway order: PathwaySubPathway/118989
pathway component: ReactionCatalysis177
pathway component: ReactionCatalysis178
pathway component: ReactionCatalysis176
pathway component: ReactionCatalysis371
pathway component: 4.2.1.17
pathway component: Transport29
pathway component: Reaction818
pathway component: Reaction819
pathway component: ReactionCatalysis2233
pathway component: ReactionCatalysis368
pathway component: Citric Acid Cycle
pathway component: ReactionCatalysis366
pathway component: ReactionCatalysis367
pathway component: Reaction825
pathway component: ReactionCatalysis365
pathway component: Reaction824
pathway component: Reaction821
pathway component: TransportCatalysis4
pathway component: Transport30
pathway component: TransportCatalysis5
pathway component: TransportCatalysis1558
pathway component: 1.3.8.6
pathway component: 1.1.1.35
pathway component: Biotin Metabolism
pathway component: 1.2.4.2
pathway component: Transport10393
pathway component: Reaction820
pathway component: ReactionCatalysis2143
pathway component: 2.3.1.9
xref: Reference/SMPDB SMP0125700
xref: Reference/PathWhiz PW127266
organism: Homo sapiens

L-Lysine
type: SmallMoleculeReference
xref: Reference/BioCyc LYS
xref: Reference/ChEBI CHEBI 18019
xref: Reference/ChemSpider 5747
xref: Reference/CAS 56-87-1
xref: Reference/HMDB HMDB0000182
xref: Reference/PubChem-compound 5962
xref: Reference/KEGG Compound C00047
chemical formula: C6H14N2O2
molecular weight: 146.10553
structure: ChemicalStructure/PW C000118

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

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

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

Compound/PW C000150 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Oxoadipic acid
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0002009
type: UnificationXref
id: HMDB0002009
db: HMDB

Reference/ChemSpider 144715
type: UnificationXref
id: 144715
db: ChemSpider

Reference/HMDB HMDB0000182
type: UnificationXref
id: HMDB0000182
db: HMDB

Reference/CAS 3105-95-1
type: UnificationXref
id: 3105-95-1
db: CAS

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

L-Pipecolic acid
type: SmallMoleculeReference
xref: Reference/CAS 3105-95-1
xref: Reference/KEGG Compound C00408
xref: Reference/HMDB HMDB0000716
xref: Reference/ChEBI CHEBI 30913
xref: Reference/ChemSpider 388365
xref: Reference/PubChem-compound 439227
xref: Reference/BioCyc L-PIPECOLATE
chemical formula: C6H11NO2
molecular weight: 129.07898
structure: ChemicalStructure/PW C000567

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

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

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

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

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

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

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

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 NADH
type: UnificationXref
id: NADH
db: BioCyc

Reference/HMDB HMDB0001166
type: UnificationXref
id: HMDB0001166
db: HMDB

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

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

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

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

L-Lysine
type: SmallMolecule
cellular location: Extracellular Space
entity reference: L-Lysine

Reference/UniProt P49419
type: UnificationXref
id: P49419
db: UniProt

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

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

Compound/PW C007691 Peroxisome/Stoichiometry/1.0
type: Stoichiometry
physical entity: (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate
stoichiometric coefficient: 1.0

Protein/370 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
stoichiometric coefficient: 2.0

ChemicalStructure/PW C000567
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)[C@@H]1CCCCN1

Allysine
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Allysine

Compound/PW C007691/Stoichiometry/1.0
type: Stoichiometry
physical entity: (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate
stoichiometric coefficient: 1.0

Reference/BioCyc 2K-ADIPATE
type: UnificationXref
id: 2K-ADIPATE
db: BioCyc

Aminoadipic acid
type: SmallMoleculeReference
xref: Reference/ChemSpider 456
xref: Reference/HMDB HMDB0000510
xref: Reference/BioCyc CPD-468
xref: Reference/PubChem-compound 469
xref: Reference/KEGG Compound C00956
xref: Reference/CAS 542-32-5
xref: Reference/ChEBI CHEBI 37024
chemical formula: C6H11NO4
molecular weight: 161.0688
structure: ChemicalStructure/PW C000390

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

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

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

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

Reference/ChEBI CHEBI 16927
type: UnificationXref
id: CHEBI:16927
db: ChEBI

Alpha-aminoadipic semialdehyde dehydrogenase
type: Complex
component stoichiometry: Protein/287 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: Alpha-aminoadipic semialdehyde dehydrogenase

Oxoadipic acid
type: SmallMoleculeReference
xref: Reference/CAS 3184-35-8
xref: Reference/ChEBI CHEBI 15753
xref: Reference/HMDB HMDB0000225
xref: Reference/PubChem-compound 71
xref: Reference/KEGG Compound C00322
xref: Reference/BioCyc 2K-ADIPATE
xref: Reference/ChemSpider 70
chemical formula: C6H8O5
molecular weight: 160.03717
structure: ChemicalStructure/PW C000150

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

TransportCatalysis4
type: Catalysis
controller: Low affinity cationic amino acid transporter 2
controlled: Transport29
control type: ACTIVATION

Reference/PubChem-compound 5962
type: UnificationXref
id: 5962
db: PubChem-compound

TransportCatalysis5
type: Catalysis
controller: Mitochondrial ornithine transporter 2
controlled: Transport30
control type: ACTIVATION

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

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

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

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

Reference/CAS 102680-35-3
type: UnificationXref
id: 102680-35-3
db: CAS

ReactionCatalysis177
type: Catalysis
controller: Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
controlled: 1.1.1.35
control type: ACTIVATION

Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial

ReactionCatalysis178
type: Catalysis
controller: Acetyl-CoA acetyltransferase, mitochondrial
controlled: 2.3.1.9
control type: ACTIVATION

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

ReactionCatalysis176
type: Catalysis
controller: Enoyl-CoA hydratase, mitochondrial
controlled: 4.2.1.17
control type: ACTIVATION

Reference/PubChem-compound 5280381
type: UnificationXref
id: 5280381
db: PubChem-compound

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

Peroxisomal sarcosine oxidase
type: Protein
cellular location: Peroxisome
entity reference: Peroxisomal sarcosine oxidase

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

Crotonoyl-CoA
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00877
xref: Reference/HMDB HMDB0002009
xref: Reference/PubChem-compound 5280381
xref: Reference/CAS 102680-35-3
xref: Reference/BioCyc CPD-1083
xref: Reference/ChEBI CHEBI 15473
xref: Reference/ChemSpider 4444072
chemical formula: C25H40N7O17P3S
molecular weight: 835.1414
structure: ChemicalStructure/PW C001345

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

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

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

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

Citric Acid Cycle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway118989
pathway component: SubPathwayInteraction118989
xref: Reference/PathWhiz PW000005
xref: Reference/SMPDB SMP0000057
name: SubPathway

Biotin Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway118990
pathway component: SubPathwayInteraction118990
xref: Reference/PathWhiz PW000013
xref: Reference/SMPDB SMP0000066
name: SubPathway

Reference/PubChem-compound 71
type: UnificationXref
id: 71
db: PubChem-compound

NADPH
type: SmallMoleculeReference
xref: Reference/CAS 53-57-6
xref: Reference/HMDB HMDB0000221
xref: Reference/PubChem-compound 22833512
xref: Reference/BioCyc NADPH
xref: Reference/ChemSpider 17215925
xref: Reference/KEGG Compound C00005
xref: Reference/ChEBI CHEBI 16474
chemical formula: C21H30N7O17P3
molecular weight: 745.0911
structure: ChemicalStructure/PW C000146

Compound/PW C000390 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Aminoadipic acid
stoichiometric coefficient: 1.0

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

NADP
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00006
xref: Reference/CAS 53-59-8
xref: Reference/BioCyc NAD(P)
xref: Reference/HMDB HMDB0000217
xref: Reference/ChEBI CHEBI 18009
xref: Reference/PubChem-compound 5886
xref: Reference/ChemSpider 5675
chemical formula: C21H28N7O17P3
molecular weight: 743.07544
structure: ChemicalStructure/PW C000143

Compound/PW C001032 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glutaryl-CoA
stoichiometric coefficient: 1.0

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

Reference/PubChem-compound 207
type: UnificationXref
id: 207
db: PubChem-compound

Reference/ChEBI CHEBI 15524
type: UnificationXref
id: CHEBI:15524
db: ChEBI

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

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

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

Glutaryl-CoA dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Glutaryl-CoA dehydrogenase, mitochondrial

Alpha-aminoadipic semialdehyde synthase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Alpha-aminoadipic semialdehyde synthase, mitochondrial

Reference/ChemSpider 4444072
type: UnificationXref
id: 4444072
db: ChemSpider

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

Reference/ChemSpider 456
type: UnificationXref
id: 456
db: ChemSpider

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

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

ChemicalStructure/PW C001345
type: ChemicalStructure
structure format: SMILES

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

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

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

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

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

Reference/ChEBI CHEBI 30913
type: UnificationXref
id: CHEBI:30913
db: ChEBI

Reference/UniProt P30084
type: UnificationXref
id: P30084
db: UniProt

Mitochondrial ornithine transporter 2
type: ProteinReference
xref: Reference/UniProt Q9BXI2

Reference/BioCyc CPD-468
type: UnificationXref
id: CPD-468
db: BioCyc

Compound/PW C000976 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Allysine
stoichiometric coefficient: 1.0

Reference/BioCyc CPD-1083
type: UnificationXref
id: CPD-1083
db: BioCyc

ChemicalStructure/PW C000143
type: ChemicalStructure
structure format: SMILES

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

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

Reference/ChemSpider 202
type: UnificationXref
id: 202
db: ChemSpider

ChemicalStructure/PW C000146
type: ChemicalStructure
structure format: SMILES

Reference/HMDB HMDB0000225
type: UnificationXref
id: HMDB0000225
db: HMDB

Reference/UniProt P24752
type: UnificationXref
id: P24752
db: UniProt

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

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

Protein/287 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Alpha-aminoadipic semialdehyde dehydrogenase
stoichiometric coefficient: 4.0

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

Reference/PubChem-compound 469
type: UnificationXref
id: 469
db: PubChem-compound

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

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

Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/373 Mitochondrion/Stoichiometry/2.0
cellular location: Mitochondrion
component: Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial

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

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

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

Reference/SMPDB SMP0000066
type: UnificationXref
id: SMP0000066
db: SMPDB

Reference/ChEBI CHEBI 15753
type: UnificationXref
id: CHEBI:15753
db: ChEBI

Compound/PW C000976 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Allysine
stoichiometric coefficient: 1.0

ChemicalStructure/PW C000118
type: ChemicalStructure
structure format: SMILES
structure data: NCCCC[C@H](N)C(O)=O

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

Compound/PW C000567 Peroxisome/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Pipecolic acid
stoichiometric coefficient: 1.0

TransportCatalysis1558
type: Catalysis
controller: Unknown
controlled: Transport10393
control type: ACTIVATION

Low affinity cationic amino acid transporter 2
type: ProteinReference
xref: Reference/UniProt P52569

Enoyl-CoA hydratase, mitochondrial
type: Complex
component stoichiometry: Protein/380 Mitochondrion/Stoichiometry/6.0
cellular location: Mitochondrion
component: Enoyl-CoA hydratase, mitochondrial

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

Glutaryl-CoA
type: SmallMoleculeReference
xref: Reference/BioCyc GLUTARYL-COA
xref: Reference/CAS 103192-48-9
xref: Reference/ChEBI CHEBI 15524
xref: Reference/PubChem-compound 439252
xref: Reference/HMDB HMDB0001339
xref: Reference/KEGG Compound C00527
xref: Reference/ChemSpider 388388
chemical formula: C26H42N7O19P3S
molecular weight: 881.1469
structure: ChemicalStructure/PW C001032

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

Reference/GENE ONTOLOGY GO 0005615
type: UnificationXref
id: GO:0005615
db: GENE ONTOLOGY

Reference/GENE ONTOLOGY GO 0005737
type: UnificationXref
id: GO:0005737
db: GENE ONTOLOGY

Reference/HMDB HMDB0001339
type: UnificationXref
id: HMDB0001339
db: HMDB

Saccharopine
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00449
xref: Reference/HMDB HMDB0000279
xref: Reference/ChemSpider 141086
xref: Reference/CAS 997-68-2
xref: Reference/ChEBI CHEBI 16927
xref: Reference/PubChem-compound 160556
xref: Reference/BioCyc SACCHAROPINE
chemical formula: C11H20N2O6
molecular weight: 276.13214
structure: ChemicalStructure/PW C000189

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

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

Reference/UniProt Q9UDR5
type: UnificationXref
id: Q9UDR5
db: UniProt

Reference/KEGG Compound C00956
type: UnificationXref
id: C00956
db: KEGG Compound

Transport29
type: Transport
participant stoichiometry: Compound/PW C000118 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000118 Extracellular Space/Stoichiometry/1.0
right: L-Lysine
spontaneous: false
left: L-Lysine
name: L-Lysine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm
conversion direction: LEFT_TO_RIGHT

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

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

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

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

Reference/BioCyc L-PIPECOLATE
type: UnificationXref
id: L-PIPECOLATE
db: BioCyc

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

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

Compound/PW C000976/Stoichiometry/1.0
type: Stoichiometry
physical entity: Allysine
stoichiometric coefficient: 1.0

Compound/PW C000118 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Lysine
stoichiometric coefficient: 1.0

Reference/UniProt Q9P0Z9
type: UnificationXref
id: Q9P0Z9
db: UniProt

Reference/ChemSpider 141086
type: UnificationXref
id: 141086
db: ChemSpider

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

ChemicalStructure/PW C000976
type: ChemicalStructure
structure format: SMILES
structure data: NC(CCCC=O)C(O)=O

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

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

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

Reference/ChEBI CHEBI 15452
type: UnificationXref
id: CHEBI:15452
db: ChEBI

Reference/ChemSpider 389056
type: UnificationXref
id: 389056
db: ChemSpider

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

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

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

Cytoplasm
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005737

Reference/CAS 997-68-2
type: UnificationXref
id: 997-68-2
db: CAS

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

ReactionCatalysis2143
type: Catalysis
controller: Oxoglutarate dehydrogenase complex
controlled: 1.2.4.2
control type: ACTIVATION

Reference/UniProt Q16836
type: UnificationXref
id: Q16836
db: UniProt

Reference/CAS 3038-89-9
type: UnificationXref
id: 3038-89-9
db: CAS

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

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

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

Homo sapiens
type: BioSource
xref: Reference/TAXONOMY 9606

Reference/CAS 1962-83-0
type: UnificationXref
id: 1962-83-0
db: CAS

Reference/PubChem-compound 160556
type: UnificationXref
id: 160556
db: PubChem-compound

ChemicalStructure/PW C000940
type: ChemicalStructure
structure format: SMILES

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

Oxygen
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001377
xref: Reference/ChemSpider 952
xref: Reference/KEGG Compound C00007
xref: Reference/ChEBI CHEBI 15379
xref: Reference/PubChem-compound 977
xref: Reference/BioCyc CPD-6641
xref: Reference/CAS 7782-44-7
chemical formula: O2
molecular weight: 31.98983
structure: ChemicalStructure/PW C001065

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

Protein/197 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Glutaryl-CoA dehydrogenase, mitochondrial
stoichiometric coefficient: 4.0

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

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

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

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

Reference/HMDB HMDB0000716
type: UnificationXref
id: HMDB0000716
db: HMDB

Transport30
type: Transport
participant stoichiometry: Compound/PW C000118 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000118 Mitochondrion/Stoichiometry/1.0
right: L-Lysine
spontaneous: false
left: L-Lysine
name: L-Lysine (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrion
conversion direction: LEFT_TO_RIGHT

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

Protein/797 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Alpha-aminoadipic semialdehyde synthase, mitochondrial
stoichiometric coefficient: 2.0

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

Reference/ChEBI CHEBI 49015
type: UnificationXref
id: CHEBI:49015
db: ChEBI

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

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

Reference/KEGG Compound C00877
type: UnificationXref
id: C00877
db: KEGG Compound

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

Glutaryl-CoA dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/197 Mitochondrion/Stoichiometry/4.0
cellular location: Mitochondrion
component: Glutaryl-CoA dehydrogenase, mitochondrial

4.2.1.17
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001345 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000904 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/1.0
right: Water
right: Crotonoyl-CoA
spontaneous: false
EC number: 4.2.1.17
left: 3-Hydroxybutyryl-CoA
name: 3-Hydroxybutyryl-CoA ↔ Crotonoyl-CoA + Water
conversion direction: REVERSIBLE

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

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

3-Hydroxybutyryl-CoA
type: SmallMoleculeReference
xref: Reference/CAS 2871-66-1
xref: Reference/KEGG Compound C03561
xref: Reference/PubChem-compound 440045
xref: Reference/HMDB HMDB0001166
xref: Reference/ChEBI CHEBI 15452
xref: Reference/BioCyc 2-METHYL-3-HYDROXY-BUTYRYL-COA
xref: Reference/ChemSpider 389056
chemical formula: C25H42N7O18P3S
molecular weight: 853.152
structure: ChemicalStructure/PW C000904

Compound/PW C000146 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADPH
stoichiometric coefficient: 1.0

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

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

Unknown
type: Protein
entity reference: Unknown

Reference/PathWhiz PW000013
type: UnificationXref
id: PW000013
db: PathWhiz

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

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

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

Reference/KEGG Compound C00408
type: UnificationXref
id: C00408
db: KEGG Compound

Reference/KEGG Compound C00527
type: UnificationXref
id: C00527
db: KEGG Compound

Protein/36 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Acetyl-CoA acetyltransferase, mitochondrial
stoichiometric coefficient: 4.0

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

Reference/ChEBI CHEBI 37024
type: UnificationXref
id: CHEBI:37024
db: ChEBI

1.1.1.35
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C040034 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001142 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000904 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
right: Acetoacetyl-CoA
right: Hydrogen Ion
right: NADH
spontaneous: false
EC number: 1.1.1.35
left: NAD
left: 3-Hydroxybutyryl-CoA
name: 3-Hydroxybutyryl-CoA + NAD → Acetoacetyl-CoA + Hydrogen Ion + NADH
conversion direction: LEFT_TO_RIGHT

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

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

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

Glutaryl-CoA dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q92947
organism: Homo sapiens

Compound/PW C000904 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Hydroxybutyryl-CoA
stoichiometric coefficient: 1.0

L-Pipecolic acid
type: SmallMolecule
cellular location: Peroxisome
entity reference: L-Pipecolic acid

Reference/PubChem-compound 439227
type: UnificationXref
id: 439227
db: PubChem-compound

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

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

L-Lysine
type: SmallMolecule
cellular location: Mitochondrion
entity reference: L-Lysine
name: SubPathwayInput

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

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

ReactionCatalysis371
type: Catalysis
controller: Peroxisomal sarcosine oxidase
controlled: Reaction825
control type: ACTIVATION

Compound/PW C000118 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Lysine
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000510
type: UnificationXref
id: HMDB0000510
db: HMDB

Compound/PW C001345 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Crotonoyl-CoA
stoichiometric coefficient: 1.0

Reference/PubChem-compound 439252
type: UnificationXref
id: 439252
db: PubChem-compound

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

ReactionCatalysis2233
type: Catalysis
controller: Glutaryl-CoA dehydrogenase, mitochondrial
controlled: 1.3.8.6
control type: ACTIVATION

ReactionCatalysis368
type: Catalysis
controller: Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
controlled: Reaction821
control type: ACTIVATION

ReactionCatalysis366
type: Catalysis
controller: Alpha-aminoadipic semialdehyde synthase, mitochondrial
controlled: Reaction819
control type: ACTIVATION

ReactionCatalysis367
type: Catalysis
controller: Alpha-aminoadipic semialdehyde dehydrogenase
controlled: Reaction820
control type: ACTIVATION

ReactionCatalysis365
type: Catalysis
controller: Alpha-aminoadipic semialdehyde synthase, mitochondrial
controlled: Reaction818
control type: ACTIVATION

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

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

Reference/ChemSpider 388388
type: UnificationXref
id: 388388
db: ChemSpider

Reference/ChEBI CHEBI 15473
type: UnificationXref
id: CHEBI:15473
db: ChEBI

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

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

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