CHEBI:16015
ChEBI
SubPathwayInteraction108519
SubPathway108519Reaction
SubPathwayReaction
7722-84-1
CAS
Oxoglutarate dehydrogenase complex
C23H38N7O17P3S
Acetyl-CoA
809.1258
937
ChemSpider
Dihydrolipoyl dehydrogenase, mitochondrial
388353
ChemSpider
HMDB0003125
HMDB
439153
PubChem-compound
SubPathwayInteraction108520
SubPathwayReaction
SubPathway108520Reaction
50
ChemSpider
C00449
KEGG Compound
388365
ChemSpider
C00322
KEGG Compound
Acetoacetyl-CoA
SACCHAROPINE
BioCyc
5747
ChemSpider
Acetyl-CoA
SubPathwayInput
CHEBI:16474
ChEBI
C00332
KEGG Compound
952
ChemSpider
L-Lysine
440045
PubChem-compound
Alpha-aminoadipic semialdehyde synthase, mitochondrial
Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
70
ChemSpider
L-Glutamic acid
1.0
CHEBI:16240
ChEBI
1010
ChemSpider
SMILES
CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)[C@@H](O)C(=O)NCCC(=O)NCCS
Rattus norvegicus
C6H9NO2
(S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate
127.06333
Q6P6R2
UniProt
CHEBI:17027
ChEBI
CHEBI:5086
ChEBI
165067
PubChem-compound
1.0
56-87-1
CAS
Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
3-Hydroxybutyryl-CoA
C00007
KEGG Compound
C00006
KEGG Compound
CHEBI:18009
ChEBI
C00001
KEGG Compound
C00005
KEGG Compound
C00004
KEGG Compound
C00003
KEGG Compound
103192-48-9
CAS
Pipecolic acid and sarcosine oxidase
SMILES
OC(=O)C1CCCC=N1
SMILES
C[C@@H](O)CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N
C21H28N7O14P2
NAD
664.11694
Hydrogen peroxide
C00011
KEGG Compound
C00010
KEGG Compound
HMDB0000902
HMDB
Extracellular Space
NAD(P)
BioCyc
H
Hydrogen Ion
1.007825
C6H11NO3
Allysine
145.0739
C01475
KEGG Compound
53-84-9
CAS
C00027
KEGG Compound
C00026
KEGG Compound
C00025
KEGG Compound
C00024
KEGG Compound
3184-35-8
CAS
(S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate
1.0
328-50-7
CAS
SMILES
NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O
2871-66-1
CAS
SMILES
CC(=O)CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N
Peroxisome
1.0
HYDROGEN-PEROXIDE
BioCyc
Glutaryl-CoA dehydrogenase, mitochondrial
1.0
Acetyl-CoA acetyltransferase, mitochondrial
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
C00047
KEGG Compound
763
ChemSpider
SMILES
CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)C(O)C(=O)NCCC(=O)NCCSC(=O)CCCC(O)=O
C03561
KEGG Compound
HMDB0000148
HMDB
SMILES
N[C@@H](CCCCN[C@@H](CCC(O)=O)C(O)=O)C(O)=O
TransportCatalysis1052
ACTIVATION
542-32-5
CAS
TransportCatalysis1053
ACTIVATION
CHEBI:18019
ChEBI
784
PubChem-compound
Oxoglutaric acid
Alpha-aminoadipic semialdehyde dehydrogenase
7732-18-5
CAS
HMDB0012130
HMDB
Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
NAD
HMDB0001487
HMDB
HMDB0000279
HMDB
56-86-0
CAS
P17764
UniProt
SMILES
OC(=O)CCCC(=O)C(O)=O
SMILES
NC(CCCC(O)=O)C(O)=O
1.0
HMDB0001484
HMDB
1.0
Reaction824
true
(S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate + Water ↔ Allysine
REVERSIBLE
HMDB0001377
HMDB
Q5XI35
UniProt
962
PubChem-compound
GLUTARYL-COA
BioCyc
CO2
Carbon dioxide
43.98983
Allysine
C00080
KEGG Compound
SMILES
O=O
2.0
HMDB0002111
HMDB
HMDB0001263
HMDB
SMILES
N[C@@H](CCC(O)=O)C(O)=O
C6H14N2O2
L-Lysine
146.10553
Enoyl-CoA hydratase, mitochondrial
1.0
NADP
1.0
HMDB0002009
HMDB
P15651
UniProt
144715
ChemSpider
HMDB0000182
HMDB
3105-95-1
CAS
444493
PubChem-compound
C6H11NO2
L-Pipecolic acid
129.07898
Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
1.0
Crotonoyl-CoA
Mitochondrial ornithine transporter 1
Q5I0K1
UniProt
22833512
PubChem-compound
ACETYL-COA
BioCyc
H2O
Water
18.010565
H2O2
Hydrogen peroxide
34.005478
Glutaryl-CoA
1.0
NADH
BioCyc
HMDB0001166
HMDB
NAD
BioCyc
NADPH
977
PubChem-compound
HMDB0059597
HMDB
L-Lysine
30572
ChemSpider
Alpha-aminoadipic semialdehyde synthase, mitochondrial
17215925
ChemSpider
Enoyl-CoA hydratase, mitochondrial
1.0
Allysine
SMILES
OC(=O)[C@@H]1CCCCN1
2K-ADIPATE
BioCyc
Alpha-aminoadipic semialdehyde synthase, mitochondrial
C6H11NO4
Aminoadipic acid
161.0688
2.0
SMILES
O
SMILES
OO
CHEBI:16927
ChEBI
C6H8O5
Oxoadipic acid
160.03717
1.0
5962
PubChem-compound
6816
PubChem-compound
51
PubChem-compound
Water
CHEBI:15846
ChEBI
2.3.1.9
false
2.3.1.9
2 Acetyl-CoA ↔ Acetoacetyl-CoA + Coenzyme A
REVERSIBLE
4.2.1.17
false
4.2.1.17
3-Hydroxybutyryl-CoA ↔ Crotonoyl-CoA + Water
REVERSIBLE
102680-35-3
CAS
ReactionCatalysis137435
ACTIVATION
ReactionCatalysis137436
ACTIVATION
53-59-8
CAS
5280381
PubChem-compound
1038
PubChem-compound
ReactionCatalysis137437
ACTIVATION
ReactionCatalysis137438
ACTIVATION
ReactionCatalysis137439
ACTIVATION
LYS
BioCyc
C25H40N7O17P3S
Crotonoyl-CoA
835.1414
4.0
274
ChemSpider
C5H6O5
Oxoglutaric acid
146.02153
Hydrogen Ion
Reduced electron-transfer flavoprotein
CHEBI:16908
ChEBI
Citric Acid Cycle
SubPathway
Enoyl-CoA hydratase, mitochondrial
ReactionCatalysis137440
ACTIVATION
ReactionCatalysis137441
ACTIVATION
4.0
SMP0120870
SMPDB
ReactionCatalysis137329
ACTIVATION
Biotin Metabolism
SubPathway
71
PubChem-compound
C21H30N7O17P3
NADPH
745.0911
1.0
PW122131
PathWhiz
Mitochondrion
C21H29N7O17P3
NADP
744.08325
1.0
NADPH
BioCyc
207
PubChem-compound
CHEBI:15524
ChEBI
electron-transfer flavoprotein
HMDB0000208
HMDB
CPD-6641
BioCyc
4444072
ChemSpider
58-68-4
CAS
456
ChemSpider
Water
Aldehyde dehydrogenase, mitochondrial
HMDB0001423
HMDB
SMILES
C\C=C\C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12
SMILES
OC(=O)CCC(=O)C(O)=O
HMDB0000217
HMDB
CHEBI:30915
ChEBI
124-38-9
CAS
CHEBI:30913
ChEBI
CPD-468
BioCyc
1.0
P14604
UniProt
CPD-1083
BioCyc
SMILES
NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O
HMDB0000221
HMDB
1.0
202
ChemSpider
SMILES
NC(=O)C1=CN(C=CC1)[C@H]1O[C@@H](COP(O)(=O)OP(O)(=O)OC[C@@H]2O[C@@H]([C@@H](OP(O)(O)=O)[C@H]2O)N2C=NC3=C(N)N=CN=C23)[C@H](O)[C@@H]1O
HMDB0000225
HMDB
GLT
BioCyc
SMP0000057
SMPDB
53-57-6
CAS
Q64602
UniProt
469
PubChem-compound
SMILES
O=C=O
Oxoadipic acid
Acetyl-CoA acetyltransferase, mitochondrial
Q4KLP0
UniProt
C25H40N7O18P3S
Acetoacetyl-CoA
851.13635
(S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate
C21H29N7O14P2
NADH
665.12476
1.0
HMDB0001206
HMDB
1.1.1.35
false
1.1.1.35
3-Hydroxybutyryl-CoA + NAD → Acetoacetyl-CoA + Hydrogen Ion + NADH
LEFT_TO_RIGHT
SMP0000066
SMPDB
2-Aminoadipic 2-Oxoadipic Aciduria
Aldehyde dehydrogenase, mitochondrial
CHEBI:15753
ChEBI
1.0
Mitochondrial ornithine transporter 1
SMILES
NCCCC[C@H](N)C(O)=O
ALLYSINE
BioCyc
1.0
1.0
C26H42N7O19P3S
Glutaryl-CoA
881.1469
GO:0005739
GENE ONTOLOGY
GO:0005615
GENE ONTOLOGY
GO:0005737
GENE ONTOLOGY
HMDB0001339
HMDB
C11H20N2O6
Saccharopine
276.13214
1.0
P11884
UniProt
Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
C00956
KEGG Compound
Cationic amino acid transporter 2
SMILES
NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O
electron-transfer flavoprotein
10116
TAXONOMY
Transport8008
false
L-Lysine (→) Transport: Rattus norvegicus, Extracellular Space to Rattus norvegicus, Cell, Cytoplasm
LEFT_TO_RIGHT
Transport8009
false
L-Lysine (→) Transport: Rattus norvegicus, Cell, Cytoplasm to Rattus norvegicus, Cell, Mitochondrion
LEFT_TO_RIGHT
5682
ChemSpider
2-METHYL-3-HYDROXY-BUTYRYL-COA
BioCyc
L-PIPECOLATE
BioCyc
Reduced electron-transfer flavoprotein
CHEBI:1146900
ChEBI
1.0
141086
ChemSpider
CO-A
BioCyc
SMILES
NC(CCCC=O)C(O)=O
5675
ChemSpider
72-89-9
CAS
1.0
CHEBI:15452
ChEBI
389056
ChemSpider
Oxygen
Carbon dioxide
C21H36N7O16P3S
Coenzyme A
767.11523
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
1.2.4.2
false
1.2.4.2
Coenzyme A + Oxoadipic acid → Carbon dioxide + Glutaryl-CoA
LEFT_TO_RIGHT
Cytoplasm
1.3.8.6
false
1.3.8.6
Glutaryl-CoA + electron-transfer flavoprotein → Carbon dioxide + Crotonoyl-CoA + Reduced electron-transfer flavoprotein
LEFT_TO_RIGHT
997-68-2
CAS
Reaction147211
false
L-Pipecolic acid + Oxygen → (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate + Hydrogen peroxide
LEFT_TO_RIGHT
Reaction147210
false
Aminoadipic acid + Oxoglutaric acid ↔ L-Glutamic acid + Oxoadipic acid
REVERSIBLE
3038-89-9
CAS
ACETOACETYL-COA
BioCyc
Coenzyme A
6557
ChemSpider
1962-83-0
CAS
Acetyl-CoA acetyltransferase, mitochondrial
160556
PubChem-compound
SMILES
CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N
388299
ChemSpider
Reaction147209
false
Allysine + NAD + Water → Aminoadipic acid + NADH
LEFT_TO_RIGHT
Reaction147208
false
NAD + Saccharopine + Water → Allysine + L-Glutamic acid + NADH
LEFT_TO_RIGHT
O2
Oxygen
31.98983
Reaction147207
false
L-Lysine + NADPH + Oxoglutaric acid → NADP + Saccharopine + Water
LEFT_TO_RIGHT
439214
PubChem-compound
C5H9NO4
L-Glutamic acid
147.05316
33032
PubChem-compound
B5D5P0
UniProt
CHEBI:16526
ChEBI
HMDB0000716
HMDB
7782-44-7
CAS
1.0
GO:0005777
GENE ONTOLOGY
CHEBI:49015
ChEBI
NADH
C00877
KEGG Compound
2-KETOGLUTARATE
BioCyc
ReactionCatalysis137255
ACTIVATION
ReactionCatalysis137256
ACTIVATION
392413
ChemSpider
Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial
2.0
5893
PubChem-compound
C25H42N7O18P3S
3-Hydroxybutyryl-CoA
853.152
Q9WVK7
UniProt
1.0
A2VCW9
UniProt
1.0
1420-36-6
CAS
CHEBI:15377
ChEBI
PW000013
PathWhiz
Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
CHEBI:15379
ChEBI
CHEBI:15378
ChEBI
SMILES
[H+]
C00408
KEGG Compound
C00527
KEGG Compound
6.0
5886
PubChem-compound
Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
CHEBI:37024
ChEBI
280
PubChem-compound
1.0
L-Pipecolic acid
439227
PubChem-compound
Cationic amino acid transporter 2
2.0
Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial
Saccharopine
L-Lysine
SubPathwayInput
CHEBI:15345
ChEBI
PW000005
PathWhiz
1.0
HMDB0000510
HMDB
1.0
439252
PubChem-compound
Aminoadipic acid
2.0
Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial
Pipecolic acid and sarcosine oxidase
HMDB0001967
HMDB
4.0
CHEBI:15351
ChEBI
388388
ChemSpider
CHEBI:15473
ChEBI
1.0
1.0
85-61-0
CAS
Dihydrolipoyl dehydrogenase, mitochondrial