6022
PubChem-compound
C23H38N7O17P3S
Acetyl-CoA
809.1258
439161
PubChem-compound
Mitochondrial pyruvate carrier 1
937
ChemSpider
C00311
KEGG Compound
Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
CHEBI:30797
ChEBI
439153
PubChem-compound
1.0
110-17-8
CAS
HMDB0000094
HMDB
50
ChemSpider
1.0
PW000054
PathWhiz
Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial
OXALACETIC_ACID
BioCyc
ATP
BioCyc
PW000051
PathWhiz
1.0
4.0
HMDB0001072
HMDB
HMDB0001197
HMDB
PW000046
PathWhiz
PW000165
PathWhiz
CHEBI:16238
ChEBI
P09622
UniProt
HCO3
BioCyc
CHEBI:15380
ChEBI
5742
ChemSpider
C25H40N7O19P3S
Succinyl-CoA
867.1312
PW000163
PathWhiz
Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
PW000162
PathWhiz
1.0
1.0
5281915
PubChem-compound
97-67-6
CAS
PW000155
PathWhiz
C27H35N9O15P2
FADH
787.1728
Carbon dioxide
945
ChemSpider
PW000152
PathWhiz
PW000030
PathWhiz
1010
ChemSpider
C10H15N5O11P2
Guanosine diphosphate
443.02432
1.0
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
CHEBI:30769
ChEBI
Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
CHEBI:30887
ChEBI
1.0
PW000029
PathWhiz
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)CCC(O)=O
SubPathwayInteraction356
SubPathway356Reaction
SubPathwayReaction
SubPathwayInteraction357
SubPathwayReaction
SubPathway357Reaction
SubPathwayInteraction354
SubPathwayReaction
SubPathway354Reaction
SubPathwayInteraction355
SubPathway355Reaction
SubPathwayReaction
C10H16N5O14P3
Guanosine triphosphate
522.99066
SubPathwayInteraction358
SubPathwayReaction
SubPathway358Reaction
1.0
SubPathwayInteraction359
SubPathway359Reaction
SubPathwayReaction
Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial
1161
ChemSpider
Cytosol
SUC
BioCyc
O43837
UniProt
Reaction390
false
L-Malic acid + NAD ↔ Hydrogen Ion + NADH + Oxalacetic acid
REVERSIBLE
1.0
SubPathwayInteraction352
SubPathwayReaction
SubPathway352Reaction
SubPathwayInteraction353
SubPathway353Reaction
SubPathwayReaction
C00009
KEGG Compound
C00008
KEGG Compound
Succinate dehydrogenase
C00001
KEGG Compound
1.0
559059
ChemSpider
C00122
KEGG Compound
SMILES
CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=C(N2)C(=O)NC(=O)N1
4.0
NADH
SMILES
NC1=NC2=C(N=CN2[C@@H]2O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)C(=O)N1
Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
C00004
KEGG Compound
CHEBI:18367
ChEBI
C00003
KEGG Compound
1032
ChemSpider
1910-41-4
CAS
1031
ChemSpider
C00002
KEGG Compound
60.0
Coenzyme A
Mitochondrial Inner Membrane
C11378
KEGG Compound
SubPathwayInteraction378
SubPathway378Reaction
SubPathwayReaction
C59H90O4
Coenzyme Q10
862.6839
SubPathwayInteraction376
SubPathwayReaction
SubPathway376Reaction
SubPathwayInteraction377
SubPathwayReaction
SubPathway377Reaction
C27H33N9O15P2
FAD
785.1571
Pyruvic acid
SubPathwayOutput
SubPathwayInput
C21H27N7O14P2
NAD
663.10913
1.0
C00011
KEGG Compound
C00010
KEGG Compound
HMDB0000902
HMDB
Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
C00016
KEGG Compound
FAD
1.0
SubPathwayInteraction370
SubPathway370Reaction
SubPathwayReaction
SubPathwayInteraction371
SubPathwayReaction
SubPathway371Reaction
SubPathwayInteraction374
SubPathway374Reaction
SubPathwayReaction
SubPathwayInteraction375
SubPathway375Reaction
SubPathwayReaction
FADH
SubPathwayInput
SubPathwayInteraction372
SubPathwayReaction
SubPathway372Reaction
SubPathwayInteraction373
SubPathway373Reaction
SubPathwayReaction
SubPathwayInteraction367
SubPathway367Reaction
SubPathwayReaction
H
Hydrogen Ion
1.007825
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
SubPathwayInteraction368
SubPathway368Reaction
SubPathwayReaction
SubPathwayInteraction365
SubPathwayReaction
SubPathway365Reaction
C00149
KEGG Compound
SubPathwayInteraction366
SubPathwayReaction
SubPathway366Reaction
388307
ChemSpider
SubPathwayInteraction369
SubPathwayReaction
SubPathway369Reaction
Phosphate
C00022
KEGG Compound
C01352
KEGG Compound
Pyruvate carboxylase, mitochondrial
56-65-5
CAS
53-84-9
CAS
C00026
KEGG Compound
C00024
KEGG Compound
Fumaric acid
SubPathwayOutput
O14521
UniProt
C00390
KEGG Compound
SubPathwayInteraction360
SubPathway360Reaction
SubPathwayReaction
SubPathwayInteraction363
SubPathwayReaction
SubPathway363Reaction
SubPathwayInteraction364
SubPathway364Reaction
SubPathwayReaction
SubPathwayInteraction361
SubPathway361Reaction
SubPathwayReaction
SubPathwayInteraction362
SubPathway362Reaction
SubPathwayReaction
1.0
769
PubChem-compound
56275-39-9
CAS
Pyruvate carboxylase, mitochondrial
C00158
KEGG Compound
C00036
KEGG Compound
Transport1
false
Pyruvic acid (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrial Matrix
LEFT_TO_RIGHT
C00035
KEGG Compound
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
HMDB0000134
HMDB
HMDB0000254
HMDB
SMILES
OC(=O)CCC(O)=O
P50213
UniProt
1.0
SMILES
OC(=O)C\C(=C\C(O)=O)C(O)=O
HMDB0001341
HMDB
P07954
UniProt
1.0
C00044
KEGG Compound
C00042
KEGG Compound
4445197
ChemSpider
303-98-0
CAS
C00288
KEGG Compound
SMILES
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
SMILES
OC(=O)CC(O)(CC(O)=O)C(O)=O
604-98-8
CAS
P51553
UniProt
cis-Aconitic acid
Succinyl-CoA
10197150
ChemSpider
FUM
BioCyc
SMILES
OC(=O)CC(=O)C(O)=O
7732-18-5
CAS
Aconitate hydratase, mitochondrial
CHEBI:18012
ChEBI
HMDB0001487
HMDB
HMDB0000156
HMDB
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
Q99798
UniProt
HMDB0001248
HMDB
Pyruvic acid
1.0
Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial
P21912
UniProt
Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
24.0
C10H16N5O13P3
Adenosine triphosphate
506.99576
SMILES
COC1=C(O)C(C)=C(C\C=C(/C)CCC=C(C)C)C(O)=C1OC
305
ChemSpider
NAD
SMILES
CC(=O)C(O)=O
Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
6.4.1.1
false
6.4.1.1
Adenosine triphosphate + Hydrogen carbonate + Pyruvic acid → Adenosine diphosphate + Oxalacetic acid + Phosphate
LEFT_TO_RIGHT
962
PubChem-compound
1.0
5800
ChemSpider
CO2
Carbon dioxide
43.98983
C00080
KEGG Compound
Water
Glutamate Metabolism
SubPathway
Ketone Body Metabolism
SubPathway
Q9Y5U8
UniProt
HMDB0002111
HMDB
HMDB0001022
HMDB
Adenosine diphosphate
146-14-5
CAS
14265-44-2
CAS
PYRUVATE
BioCyc
QH2
BioCyc
1.0
58-64-0
CAS
GTP
BioCyc
585-84-2
CAS
C00091
KEGG Compound
1.0
P11177
UniProt
HMDB0001273
HMDB
Isocitric acid
444493
PubChem-compound
Succinic acid
SubPathwayInput
Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
ACETYL-COA
BioCyc
H2O
Water
18.010565
1.0
FADH2
BioCyc
Succinic acid
HMDB0000193
HMDB
Hydrogen carbonate
HMDB0000072
HMDB
NADH
BioCyc
NAD
BioCyc
HMDB0059597
HMDB
Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
749
ChemSpider
71-52-3
CAS
970
PubChem-compound
CIS-ACONITATE
BioCyc
SMILES
OC(=O)\C=C\C(O)=O
Acetyl-CoA
SubPathwayOutput
SubPathwayInput
Q99643
UniProt
O75390
UniProt
146-91-8
CAS
Citrate synthase, mitochondrial
C4H6O5
L-Malic acid
134.02153
CH2O3
Hydrogen carbonate
62.000393
446013
PubChem-compound
C4H4O5
Oxalacetic acid
132.00587
L-Malic acid
Succinyl-CoA ligase
Adenosine triphosphate
1.0
Valine, Leucine, and Isoleucine Degradation
SubPathway
2.0
Oxidation of Branched-Chain Fatty Acids
SubPathway
21883788
PubChem-compound
Citric acid
SMILES
O
CIT
BioCyc
Guanosine triphosphate
Lysine Degradation
SubPathway
Succinate dehydrogenase cytochrome b560 subunit, mitochondrial
P11498
UniProt
HMDB0000538
HMDB
Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial
(C5H8)nC14H20O4
QH2
TransportCatalysis6
ACTIVATION
Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial
1.0
328-42-7
CAS
6816
PubChem-compound
51
PubChem-compound
Glycolysis
SubPathway
2.0
SMILES
O[C@@H](CC(O)=O)C(O)=O
SMILES
OC(O)=O
1.0
CHEBI:15846
ChEBI
ADP
BioCyc
C3H4O3
Pyruvic acid
88.016045
CHEBI:46245
ChEBI
1.0
C6H8O7
Isocitric acid
192.02701
1038
PubChem-compound
Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial
Oxoglutarate dehydrogenase complex
Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial
GO:0005829
GENE ONTOLOGY
Alanine Metabolism
SubPathway
Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial
Fatty Acid Metabolism
SubPathway
274
ChemSpider
C5H6O5
Oxoglutaric acid
146.02153
393487
ChemSpider
Citric acid
Urea Cycle
SubPathway
Citric Acid Cycle
CHEBI:16908
ChEBI
Pyruvate dehydrogenase complex
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
H3O4P
Phosphate
97.9769
6830
PubChem-compound
77-92-9
CAS
558863
ChemSpider
Aspartate Metabolism
SubPathway
Tryptophan Metabolism
SubPathway
1.0
Pyruvate Metabolism
SubPathway
Mitochondrion
P40925
UniProt
643757
PubChem-compound
Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial
HMDB0000208
HMDB
58-68-4
CAS
SMP0000040
SMPDB
HMDB0001423
HMDB
Water
SMILES
OP(O)(O)=O
HMDB0001304
HMDB
SMILES
OC(=O)CCC(=O)C(O)=O
Q96HY7
UniProt
CHEBI:30915
ChEBI
124-38-9
CAS
Guanosine diphosphate
Mitochondrial Electron Transport Chain
SubPathway
ReactionCatalysis69
ACTIVATION
4.2.1.3
false
4.2.1.3
Citric acid ↔ Water + cis-Aconitic acid
REVERSIBLE
1.0
ReactionCatalysis4
ACTIVATION
HMDB0001429
HMDB
ReactionCatalysis63
ACTIVATION
ReactionCatalysis64
ACTIVATION
ReactionCatalysis62
ACTIVATION
ReactionCatalysis67
ACTIVATION
ReactionCatalysis68
ACTIVATION
SMP0000051
SMPDB
HMDB0000223
HMDB
GO:0005759
GENE ONTOLOGY
GDP-4-DEHYDRO-6-DEOXY-D-MANNOSE
BioCyc
4.2.1.3
false
4.2.1.3
Water + cis-Aconitic acid ↔ Isocitric acid
REVERSIBLE
Q96I99
UniProt
1.0
Malate-Aspartate Shuttle
SubPathway
SMP0000059
SMPDB
1.0
Gluconeogenesis
SubPathway
SMP0000057
SMPDB
C4H4O4
Fumaric acid
116.010956
Tyrosine Metabolism
SubPathway
Glucose-Alanine Cycle
SubPathway
SMP0000055
SMPDB
P31040
UniProt
Aconitate hydratase, mitochondrial
CHEBI:15741
ChEBI
Fumarate hydratase, mitochondrial
Malate dehydrogenase, cytoplasmic
SMILES
O=C=O
ReactionCatalysis75
ACTIVATION
ReactionCatalysis78
ACTIVATION
Mitochondrial pyruvate carrier 1
4444052
ChemSpider
ReactionCatalysis79
ACTIVATION
1110
PubChem-compound
Hydrogen Ion
1.0
HMDB0001201
HMDB
HMDB0000595
HMDB
SMP0000060
SMPDB
C21H29N7O14P2
NADH
665.12476
ReactionCatalysis70
ACTIVATION
5280344
PubChem-compound
HMDB0001206
HMDB
5957
PubChem-compound
GO:0005743
GENE ONTOLOGY
SMP0000067
SMPDB
Propanoate Metabolism
SubPathway
C4H6O4
Succinic acid
118.02661
SMP0000063
SMPDB
C6H6O6
cis-Aconitic acid
174.01643
320-77-4
CAS
threo-d(s)-iso-citrate
BioCyc
Isocitric acid
CHEBI:15996
ChEBI
SMP0000072
SMPDB
HMDB0000243
HMDB
GO:0005739
GENE ONTOLOGY
Arginine and Proline Metabolism
SubPathway
SMP0000071
SMPDB
60.0
SMILES
OC(C(CC(O)=O)C(O)=O)C(O)=O
C10H15N5O10P2
Adenosine diphosphate
427.02942
1.0
1.0
Malate dehydrogenase, cytoplasmic
C6H8O7
Citric acid
192.02701
Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial
SMP0000128
SMPDB
SMP0000129
SMPDB
SMP0000006
SMPDB
SMP0000127
SMPDB
SMILES
CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2
Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
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
CPD-8587
BioCyc
19782904
PubChem-compound
1.0
SMILES
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
5682
ChemSpider
CHEBI:1146900
ChEBI
1.2.4.2
false
1.2.4.2
Coenzyme A + NAD + Oxoglutaric acid → Carbon dioxide + Hydrogen Ion + NADH + Succinyl-CoA
LEFT_TO_RIGHT
2.3.3.1
false
2.3.3.1
Acetyl-CoA + Oxalacetic acid + Water → Citric acid + Coenzyme A
LEFT_TO_RIGHT
Oxalacetic acid
SubPathwayOutput
SubPathwayInput
1.0
Mitochondrial Matrix
1.3.5.1
false
1.3.5.1
Coenzyme Q10 + FAD + Succinic acid ↔ FADH + Fumaric acid + QH2
REVERSIBLE
Reaction593
false
Guanosine diphosphate + Phosphate + Succinyl-CoA ↔ Coenzyme A + Guanosine triphosphate + Succinic acid
REVERSIBLE
1.2.4.1
false
1.2.4.1
Coenzyme A + NAD + Pyruvic acid → Acetyl-CoA + Carbon dioxide + NADH
LEFT_TO_RIGHT
Fumarate hydratase, mitochondrial
CO-A
BioCyc
CHEBI:17877
ChEBI
SMP0000016
SMPDB
222656
PubChem-compound
Fumaric acid
72-89-9
CAS
2.0
1078
ChemSpider
1.0
C21H36N7O16P3S
Coenzyme A
767.11523
Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
CHEBI:15422
ChEBI
Citrate synthase, mitochondrial
6557
ChemSpider
1.0
643975
PubChem-compound
Homo sapiens
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
127-17-3
CAS
Coenzyme Q10
SMP0000020
SMPDB
P36957
UniProt
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
Dihydrolipoyl dehydrogenase, mitochondrial
CHEBI:16526
ChEBI
8977
PubChem-compound
1.0
CHEBI:16761
ChEBI
4.2.1.2
false
4.2.1.2
L-Malic acid ↔ Fumaric acid + Water
REVERSIBLE
SMP0000037
SMPDB
SMP0000032
SMPDB
SMP0000030
SMPDB
9606
TAXONOMY
PW000023
PathWhiz
P08559
UniProt
CHEBI:17544
ChEBI
PW000142
PathWhiz
PW000149
PathWhiz
PW000028
PathWhiz
2-KETOGLUTARATE
BioCyc
392413
ChemSpider
1.0
PW000146
PathWhiz
Oxoglutaric acid
SubPathwayOutput
SubPathwayInput
8630
ChemSpider
5893
PubChem-compound
P10515
UniProt
Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
110-15-6
CAS
Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial
Citrate synthase, mitochondrial
CHEBI:32816
ChEBI
1.0
QH2
CHEBI:15377
ChEBI
Fumarate hydratase, mitochondrial
CHEBI:15378
ChEBI
SMILES
[H+]
PW000010
PathWhiz
Isocitrate dehydrogenase
6569
ChemSpider
SMILES
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
60.0
CHEBI:17552
ChEBI
Pyruvate carboxylase, mitochondrial
280
PubChem-compound
CHEBI:30744
ChEBI
Dihydrolipoyl dehydrogenase, mitochondrial
CHEBI:32805
ChEBI
PW000002
PathWhiz
PW000001
PathWhiz
1.0
PW000005
PathWhiz
C00417
KEGG Compound
PW000003
PathWhiz
1.0
SMILES
NC1=NC2=C(N=CN2[C@@H]2O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)C(=O)N1
1198
PubChem-compound
1.0
1.0
FAD
BioCyc
193317
ChemSpider
3-METHYLBENZYLSUCCINYL-COA
BioCyc
1.1.1.41
false
1.1.1.41
Isocitric acid + NAD → Carbon dioxide + Hydrogen Ion + NADH + Oxoglutaric acid
LEFT_TO_RIGHT
86-01-1
CAS
1060
PubChem-compound
1061
PubChem-compound
P53597
UniProt
HMDB0001967
HMDB
1.0
CHEBI:15351
ChEBI
SMP0000355
SMPDB
85-61-0
CAS
UBIQUINONE-10
BioCyc