CHEBI:16015
ChEBI
C26H44N7O17P3S
2-Methylbutyryl-CoA
851.1727
439161
PubChem-compound
C25H42N7O18P3S
(S)-3-Hydroxyisobutyryl-CoA
853.152
439153
PubChem-compound
52
ChemSpider
D-METHYL-MALONYL-COA
BioCyc
50
ChemSpider
2-METHYL-BUTYRYL-COA
BioCyc
73-32-5
CAS
ReactionCatalysis100730
ACTIVATION
5.4.99.2
false
5.4.99.2
R-Methylmalonyl-CoA → Succinyl-CoA
LEFT_TO_RIGHT
C27H42N7O19P3S
3-Methylglutaconyl-CoA
893.1469
C00683
KEGG Compound
P45952
UniProt
48
ChemSpider
46
ChemSpider
Dihydrolipoamide
1.0
HMDB0002166
HMDB
C25H40N7O17P3S
Methacrylyl-CoA
835.1414
Isovaleryl-CoA
HCO3
BioCyc
CHEBI:15380
ChEBI
Pyruvate carboxylase, mitochondrial
5742
ChemSpider
C25H40N7O19P3S
Succinyl-CoA
867.1312
Acetyl-CoA
SubPathwayInput
PW000160
PathWhiz
ISOBUTYRYL-COA
BioCyc
154-87-0
CAS
L-Glutamic acid
CHEBI:17577
ChEBI
Thioredoxin reductase 2, mitochondrial
CHEBI:16240
ChEBI
4.0
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
Reaction2230
false
Methylmalonic acid → R-Methylmalonyl-CoA
LEFT_TO_RIGHT
69
ChemSpider
439164
PubChem-compound
1.0
CHEBI:28692
ChEBI
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
3-Hydroxy-3-methylglutaryl-CoA
Dihydrolipoamide
SMILES
CC(=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=C1N=CN=C2N
6106
PubChem-compound
CHEBI:5086
ChEBI
C00233
KEGG Compound
SUC
BioCyc
6712-02-3
CAS
1.0
C26H44N7O17P3S
Isovaleryl-CoA
851.1727
94
ChemSpider
C00009
KEGG Compound
C00008
KEGG Compound
C00007
KEGG Compound
C00248
KEGG Compound
C00001
KEGG Compound
2.0
P54869
UniProt
C00004
KEGG Compound
C00003
KEGG Compound
3-Methylglutaconyl-CoA
C00002
KEGG Compound
METHACRYLYL-COA
BioCyc
86
ChemSpider
C15976
KEGG Compound
C15974
KEGG Compound
Dihydrolipoyl dehydrogenase E3
388897
ChemSpider
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
73173-91-8
CAS
C00011
KEGG Compound
C00010
KEGG Compound
Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
Oxygen
4.0
3-HYDROXY-3-METHYL-GLUTARYL-COA
BioCyc
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
H
Hydrogen Ion
1.007825
Pyruvate carboxylase, mitochondrial
1.0
56-65-5
CAS
53-84-9
CAS
L-Glutamic acid
C00027
KEGG Compound
C00026
KEGG Compound
C00025
KEGG Compound
C00024
KEGG Compound
Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
317-66-8
CAS
CHEBI:28259
ChEBI
(S)-Methylmalonic acid semialdehyde
26333171
ChemSpider
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
1.0
Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
Propionyl-CoA carboxylase beta chain, mitochondrial
Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
3-Methylglutaconic Aciduria Type I
769
PubChem-compound
5462303
PubChem-compound
793193-48-3
CAS
S-Methylmalonyl-CoA
3-HYDROXY-ISOBUTYRYL-COA
BioCyc
SMILES
OC(=O)CCC(O)=O
1.0
Acetoacetic acid
ReactionCatalysis100579
ACTIVATION
HMDB0002310
HMDB
ReactionCatalysis100578
ACTIVATION
11953876
PubChem-compound
C25H40N7O19P3S
R-Methylmalonyl-CoA
867.1312
3-METHYL-CROTONYL-COA
BioCyc
C04405
KEGG Compound
1.0
C00042
KEGG Compound
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
643
ChemSpider
604-98-8
CAS
1.0
HMDB0000148
HMDB
2.0
CHEBI:17191
ChEBI
4.0
784
PubChem-compound
HMDB0001487
HMDB
72-18-4
CAS
Q07417
UniProt
Isobutyryl-CoA
Enoyl-CoA hydratase, mitochondrial
ReactionCatalysis100590
ACTIVATION
389707
ChemSpider
Enoyl-CoA hydratase, mitochondrial
HMDB0001243
HMDB
HMDB0001484
HMDB
C10H16N5O13P3
Adenosine triphosphate
506.99576
1.0
C26H42N7O18P3S
2-Methylacetoacetyl-CoA
865.152
CHEBI:28291
ChEBI
C05998
KEGG Compound
C4H6O3
(S)-Methylmalonic acid semialdehyde
102.03169
C04424
KEGG Compound
NAD
1.0
C00068
KEGG Compound
2K-4CH3-PENTANOATE
BioCyc
Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
HMDB0001493
HMDB
HMDB0001011
HMDB
1.0
962
PubChem-compound
1.1.1.31
false
1.1.1.31
(S)-3-Hydroxyisobutyric acid + NAD → (S)-Methylmalonic acid semialdehyde + Hydrogen Ion + NADH
LEFT_TO_RIGHT
Q8BH95
UniProt
Reaction110705
false
(S)-beta-Aminoisobutyric acid + Oxoglutaric acid ↔ (S)-Methylmalonic acid semialdehyde + L-Glutamic acid
REVERSIBLE
4.2.1.17
false
4.2.1.17
Methacrylyl-CoA + Water → (S)-3-Hydroxyisobutyryl-CoA
LEFT_TO_RIGHT
3.1.2.4
false
3.1.2.4
(S)-3-Hydroxyisobutyryl-CoA + Water → (S)-3-Hydroxyisobutyric acid + Coenzyme A
LEFT_TO_RIGHT
Acetoacetic acid
4.2.1.17
false
4.2.1.17
Tiglyl-CoA + Water → 2-Methyl-3-hydroxybutyryl-CoA
LEFT_TO_RIGHT
Reaction110709
false
2-Methyl-3-hydroxybutyryl-CoA + NAD ↔ 2-Methylacetoacetyl-CoA + NADH
REVERSIBLE
1.3.8.7
false
1.3.8.7
2-Methylbutyryl-CoA + electron-transfer flavoprotein ↔ Reduced electron-transfer flavoprotein + Tiglyl-CoA
REVERSIBLE
1.3.8.1
false
1.3.8.1
2-Methylbutyryl-CoA + electron-transfer flavoprotein → Reduced electron-transfer flavoprotein + Tiglyl-CoA
LEFT_TO_RIGHT
4.0
3-Methyl-1-hydroxybutyl-ThPP
2-KETO-3-METHYL-VALERATE
BioCyc
CO2
Carbon dioxide
43.98983
1.0
C03344
KEGG Compound
C03345
KEGG Compound
1.0
C00080
KEGG Compound
C25H40N7O19P3S
S-Methylmalonyl-CoA
867.1312
SMILES
O=O
2.0
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
2-Methyl-3-hydroxybutyryl-CoA
HMDB0000172
HMDB
SMILES
C[C@](O)(CC(O)=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
HMDB0002111
HMDB
1.3.8.1
false
1.3.8.1
Isobutyryl-CoA + electron-transfer flavoprotein → Methacrylyl-CoA + Reduced electron-transfer flavoprotein
LEFT_TO_RIGHT
1.3.8.7
false
1.3.8.7
Isobutyryl-CoA + electron-transfer flavoprotein ↔ Methacrylyl-CoA + Reduced electron-transfer flavoprotein
REVERSIBLE
SMILES
CC1=C(CCO[P@](O)(=O)OP(O)(O)=O)SC=[N+]1CC1=CN=C(C)N=C1N
HMDB0001022
HMDB
2.8.3.5
false
2.8.3.5
Acetoacetic acid + Succinyl-CoA → Acetoacetyl-CoA + Succinic acid
LEFT_TO_RIGHT
4.2.1.18
false
4.2.1.18
3-Methylglutaconyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA
LEFT_TO_RIGHT
14265-44-2
CAS
2.3.1.9
false
2.3.1.9
2 Acetyl-CoA → Acetoacetyl-CoA + Coenzyme A
LEFT_TO_RIGHT
1.0
99043-16-0
CAS
11953835
PubChem-compound
Q9ESL0
UniProt
C00091
KEGG Compound
840
ChemSpider
6247-73-0
CAS
Methylmalonyl-CoA mutase, mitochondrial
L-Leucine
P35487
UniProt
Methacrylyl-CoA
6.4.1.3
false
6.4.1.3
Adenosine triphosphate + Hydrogen carbonate + Propionyl-CoA → Adenosine diphosphate + Phosphate + S-Methylmalonyl-CoA
LEFT_TO_RIGHT
Reaction110712
false
(S)-Methylmalonic acid semialdehyde + Oxygen + Water → Hydrogen peroxide + Methylmalonic acid
LEFT_TO_RIGHT
2.3.1.16
false
2.3.1.16
2-Methylacetoacetyl-CoA + Coenzyme A → Acetyl-CoA + Propionyl-CoA
LEFT_TO_RIGHT
HMDB0001275
HMDB
α-Ketoisovaleric acid
4575365
ChemSpider
1100
ChemSpider
3-Methylglutaconyl-CoA
Methylmalonic acid
H2O2
Hydrogen peroxide
34.005478
SMILES
C[C@H](O)[C@H](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=C1N=CN=C2N
1.0
HMDB0001041
HMDB
1.0
NADH
BioCyc
977
PubChem-compound
4.0
1.0
HMDB0001057
HMDB
CHO3
Hydrogen carbonate
60.99257
HMDB0001052
HMDB
30572
ChemSpider
1132
PubChem-compound
ReactionCatalysis101026
ACTIVATION
ReactionCatalysis101025
ACTIVATION
ReactionCatalysis101024
ACTIVATION
ReactionCatalysis101023
ACTIVATION
ReactionCatalysis101022
ACTIVATION
ReactionCatalysis101021
ACTIVATION
ReactionCatalysis101020
ACTIVATION
SMILES
OO
1.0
ReactionCatalysis101029
ACTIVATION
22378483
ChemSpider
ReactionCatalysis101028
ACTIVATION
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
ReactionCatalysis101027
ACTIVATION
1.0
HMDB0000538
HMDB
52227-66-4
CAS
ReactionCatalysis101015
ACTIVATION
ReactionCatalysis101014
ACTIVATION
ReactionCatalysis101013
ACTIVATION
ReactionCatalysis101012
ACTIVATION
ReactionCatalysis101011
ACTIVATION
6816
PubChem-compound
ReactionCatalysis101010
ACTIVATION
51
PubChem-compound
11471767
ChemSpider
SMILES
OC([O-])=O
C26H42N7O17P3S
Tiglyl-CoA
849.15704
53
PubChem-compound
ReactionCatalysis101019
ACTIVATION
C4H6O3
Acetoacetic acid
102.03169
ReactionCatalysis101018
ACTIVATION
ReactionCatalysis101017
ACTIVATION
ReactionCatalysis101016
ACTIVATION
3-Methyl-2-oxovaleric acid
Dihydrolipoyl dehydrogenase E3
ReactionCatalysis101040
ACTIVATION
Q9D2G2
UniProt
3-hydroxyacyl-CoA dehydrogenase type-2
ReactionCatalysis101048
ACTIVATION
ReactionCatalysis101047
ACTIVATION
CHEBI:48430
ChEBI
ReactionCatalysis101046
ACTIVATION
ReactionCatalysis101045
ACTIVATION
ReactionCatalysis101044
ACTIVATION
ReactionCatalysis101043
ACTIVATION
ReactionCatalysis101042
ACTIVATION
274
ChemSpider
3-Methylcrotonyl-CoA
ReactionCatalysis101041
ACTIVATION
Reduced electron-transfer flavoprotein
Hydrogen Ion
88
PubChem-compound
C5H8O3
α-Ketoisovaleric acid
116.04734
ReactionCatalysis101049
ACTIVATION
C4H8O3
(S)-3-Hydroxyisobutyric acid
104.04734
C05118
KEGG Compound
O4P
Phosphate
94.95342
C05119
KEGG Compound
SMILES
C[C@H](C(O)=O)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12
ReactionCatalysis101037
ACTIVATION
ReactionCatalysis101036
ACTIVATION
ReactionCatalysis101035
ACTIVATION
Adenosine triphosphate
1.0
ReactionCatalysis101034
ACTIVATION
ReactionCatalysis101033
ACTIVATION
ReactionCatalysis101032
ACTIVATION
70
PubChem-compound
ReactionCatalysis101031
ACTIVATION
ReactionCatalysis101030
ACTIVATION
2-KETO-ISOVALERATE
BioCyc
3-Methyl-2-oxovaleric acid
2.0
ReactionCatalysis101039
ACTIVATION
ReactionCatalysis101038
ACTIVATION
Carbon dioxide
1.0
CPD-6641
BioCyc
58-68-4
CAS
HMDB0001423
HMDB
CHEBI:27754
ChEBI
SMILES
[O-]P([O-])([O-])=O
SMILES
CC(C)C(=O)C(O)=O
Hydroxymethylglutaryl-CoA lyase, mitochondrial
CHEBI:30915
ChEBI
SMP0000046
SMPDB
Ketoleucine
4444187
ChemSpider
HMDB0001429
HMDB
SMILES
C[C@@H](CO)C(O)=O
GO:0005759
GENE ONTOLOGY
5462214
PubChem-compound
SMP0000057
SMPDB
49
PubChem-compound
2-Methylbutyryl-CoA
Oxoglutaric acid
CHEBI:15741
ChEBI
SMP0120442
SMPDB
SMILES
O=C=O
1110
PubChem-compound
ReactionCatalysis101004
ACTIVATION
C06002
KEGG Compound
ReactionCatalysis101003
ACTIVATION
HMDB0000595
HMDB
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
C06000
KEGG Compound
SMILES
C[C@@H](C(O)=O)C(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12
C25H40N7O18P3S
Acetoacetyl-CoA
851.13635
C06001
KEGG Compound
C21H29N7O14P2
NADH
665.12476
Succinyl-CoA:3-ketoacid coenzyme A transferase 2B, mitochondrial
473
ChemSpider
HMDB0001206
HMDB
5957
PubChem-compound
2-oxoisovalerate dehydrogenase
ReactionCatalysis101009
ACTIVATION
ReactionCatalysis101008
ACTIVATION
47
PubChem-compound
ReactionCatalysis101007
ACTIVATION
ReactionCatalysis101006
ACTIVATION
ReactionCatalysis101005
ACTIVATION
1.0
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
GO:0005739
GENE ONTOLOGY
1.0
1.0
C26H42N7O17P3S
3-Methylcrotonyl-CoA
849.15704
ReactionCatalysis100147
ACTIVATION
2300823
ChemSpider
319440-43-2
CAS
CHEBI:16414
ChEBI
3-KETOBUTYRATE
BioCyc
R-Methylmalonyl-CoA
2-Methyl-1-hydroxypropyl-ThPP
5682
ChemSpider
23724625
PubChem-compound
23724626
PubChem-compound
Propionyl-CoA carboxylase beta chain, mitochondrial
17216177
ChemSpider
Mitochondrial Matrix
2-(alpha-lactyl)-thpp
BioCyc
CO-A
BioCyc
SMP0000016
SMPDB
389295
ChemSpider
72-89-9
CAS
1078
ChemSpider
1.0
Propionyl-CoA carboxylase beta chain, mitochondrial
Carbon dioxide
SubPathwayInteraction57710
SubPathway57710Reaction
SubPathwayReaction
SubPathwayInteraction57711
SubPathwayReaction
SubPathway57711Reaction
SubPathwayInteraction57712
SubPathway57712Reaction
SubPathwayReaction
1.0
SMILES
CC(CO)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12
6557
ChemSpider
SMILES
CCC(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=C1N=CN=C2N
Hydrogen carbonate
440326
PubChem-compound
440566
PubChem-compound
440565
PubChem-compound
6712-01-2
CAS
SubPathwayInteraction57709
SubPathway57709Reaction
SubPathwayReaction
1.0
CHEBI:15539
ChEBI
C5H9NO4
L-Glutamic acid
147.05316
33032
PubChem-compound
Thiamine pyrophosphate
SMILES
C\C(CC(O)=O)=C/C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12
816-66-0
CAS
389035
ChemSpider
7782-44-7
CAS
HMDB0000962
HMDB
1.0
3-Methylcrotonyl-CoA
NADH
C02939
KEGG Compound
CHEBI:17544
ChEBI
2-KETOGLUTARATE
BioCyc
392413
ChemSpider
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
61-90-5
CAS
1.0
110-15-6
CAS
1.0
SMILES
C[C@@H](C=O)C(O)=O
1.2.1.3
false
1.2.1.3
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH
LEFT_TO_RIGHT
13628334
ChemSpider
5.1.99.1
false
5.1.99.1
S-Methylmalonyl-CoA → R-Methylmalonyl-CoA
LEFT_TO_RIGHT
2.0
CHEBI:15377
ChEBI
CHEBI:15379
ChEBI
CHEBI:15378
ChEBI
SMILES
[H+]
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
SMILES
CC(C(C)=O)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12
S-(3-Methylbutanoyl)-dihydrolipoamide-E
1.0
HMDB0000985
HMDB
Reaction110278
false
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD + Water → Hydrogen carbonate + NADH + Propionyl-CoA
LEFT_TO_RIGHT
Acetyl-CoA acetyltransferase, mitochondrial
Thiamine pyrophosphate
CHEBI:15344
ChEBI
CHEBI:37373
ChEBI
CHEBI:15345
ChEBI
PW000005
PathWhiz
389466
ChemSpider
1.0
389465
ChemSpider
6.0
SMILES
CC(C)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
Thiamine pyrophosphate
3-METHYLBENZYLSUCCINYL-COA
BioCyc
Hydroxymethylglutaryl-CoA synthase, mitochondrial
(S)-beta-Aminoisobutyric acid
SubPathwayInput
439371
PubChem-compound
CHEBI:15351
ChEBI
1.0
3884-47-7
CAS
85-61-0
CAS
1.0
C5H11NO2
L-Valine
117.07898
388357
ChemSpider
6022
PubChem-compound
7722-84-1
CAS
C23H38N7O17P3S
Acetyl-CoA
809.1258
CHEBI:17460
ChEBI
937
ChemSpider
2-oxoisovalerate dehydrogenase
388353
ChemSpider
HMDB0003125
HMDB
440021
PubChem-compound
Thioredoxin reductase 2, mitochondrial
Succinic acid
Q9D051
UniProt
HMDB0006870
HMDB
1.0
ATP
BioCyc
4249-19-8
CAS
3-hydroxyacyl-CoA dehydrogenase type-2
Acetoacetyl-CoA
C25H42N7O17P3S
Isobutyryl-CoA
837.15704
6008-91-9
CAS
6287
PubChem-compound
C00579
KEGG Compound
2-METHYL-ACETO-ACETYL-COA
BioCyc
C00332
KEGG Compound
952
ChemSpider
4.1.3.4
false
4.1.3.4
3-Hydroxy-3-methylglutaryl-CoA → Acetyl-CoA
LEFT_TO_RIGHT
P38060
UniProt
4.1.3.4
false
4.1.3.4
3-Hydroxy-3-methylglutaryl-CoA → Acetoacetic acid
LEFT_TO_RIGHT
1.2.4.4
false
1.2.4.4
3-Methyl-1-hydroxybutyl-ThPP + Lipoamide → S-(3-Methylbutanoyl)-dihydrolipoamide-E
LEFT_TO_RIGHT
1.2.4.4
false
1.2.4.4
2-Methyl-1-hydroxypropyl-ThPP + Lipoamide → S-(2-Methylbutanoyl)-dihydrolipoamide + Thiamine pyrophosphate
LEFT_TO_RIGHT
1.2.4.4
false
1.2.4.4
Lipoamide → S-(2-Methylpropionyl)-dihydrolipoamide-E
LEFT_TO_RIGHT
S-(2-Methylpropionyl)-dihydrolipoamide-E
1.2.4.4
false
1.2.4.4
2-Methyl-1-hydroxypropyl-ThPP + 3-Methyl-1-hydroxybutyl-ThPP → Thiamine pyrophosphate
LEFT_TO_RIGHT
4-aminobutyrate aminotransferase, mitochondrial
1.2.4.4
false
1.2.4.4
2-Methyl-1-hydroxypropyl-ThPP → S-(2-Methylpropionyl)-dihydrolipoamide-E
LEFT_TO_RIGHT
2.6.1.42
false
2.6.1.42
L-Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + L-Glutamic acid
LEFT_TO_RIGHT
2.6.1.42
false
2.6.1.42
L-Valine + Oxoglutaric acid → L-Glutamic acid + α-Ketoisovaleric acid
LEFT_TO_RIGHT
1.0
HMDB0002054
HMDB
2.6.1.42
false
2.6.1.42
L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic acid
LEFT_TO_RIGHT
C00100
KEGG Compound
1010
ChemSpider
CHEBI:17694
ChEBI
1.3.8.4
false
1.3.8.4
Isovaleryl-CoA + electron-transfer flavoprotein → 3-Methylcrotonyl-CoA + Reduced electron-transfer flavoprotein
LEFT_TO_RIGHT
Q99MN9
UniProt
1.3.8.7
false
1.3.8.7
Isovaleryl-CoA + electron-transfer flavoprotein ↔ 3-Methylcrotonyl-CoA + Reduced electron-transfer flavoprotein
REVERSIBLE
1.2.4.4
false
1.2.4.4
Ketoleucine + Thiamine pyrophosphate ? 3-Methyl-1-hydroxybutyl-ThPP + Carbon dioxide
2.3.1.168
false
2.3.1.168
Coenzyme A + S-(3-Methylbutanoyl)-dihydrolipoamide-E → Dihydrolipoamide + Isovaleryl-CoA
LEFT_TO_RIGHT
2.3.1.168
false
2.3.1.168
Coenzyme A + S-(2-Methylpropionyl)-dihydrolipoamide-E → Dihydrolipoamide + Isobutyryl-CoA
LEFT_TO_RIGHT
1.2.4.4
false
1.2.4.4
Thiamine pyrophosphate + α-Ketoisovaleric acid → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
LEFT_TO_RIGHT
4.2.1.17
false
4.2.1.17
3-Hydroxyisovaleryl-CoA → 3-Methylcrotonyl-CoA + Water
LEFT_TO_RIGHT
Mus musculus
6.4.1.4
false
6.4.1.4
3-Methylcrotonyl-CoA + Adenosine triphosphate + Hydrogen carbonate → 3-Methylglutaconyl-CoA + Adenosine diphosphate
LEFT_TO_RIGHT
1.2.4.4
false
1.2.4.4
3-Methyl-2-oxovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
LEFT_TO_RIGHT
2.3.1.168
false
2.3.1.168
Coenzyme A + S-(2-Methylbutanoyl)-dihydrolipoamide → 2-Methylbutyryl-CoA + Dihydrolipoamide
LEFT_TO_RIGHT
1.0
C24H40N7O17P3S
Propionyl-CoA
823.1414
1.0
C01213
KEGG Compound
Cytosol
388310
ChemSpider
C00356
KEGG Compound
5880
ChemSpider
1.0
Propionyl-CoA
SubPathwayInput
3-hydroxyacyl-CoA dehydrogenase type-2
73173-92-9
CAS
S-(3-Methylbutanoyl)-dihydrolipoamide-E
1.0
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
LEU
BioCyc
PROPIONYL-COA
BioCyc
NADH
Lipoamide
CHEBI:18367
ChEBI
2-Methyl-1-hydroxypropyl-ThPP
1032
ChemSpider
C00123
KEGG Compound
Phosphate
3-ketoacyl-CoA thiolase, mitochondrial
Q571I9
UniProt
4-aminobutyrate aminotransferase, mitochondrial
C21H28N7O14P2
NAD
664.11694
S-(2-Methylpropionyl)-dihydrolipoamide-E
HMDB0000902
HMDB
Succinyl-CoA
SubPathwayInput
388543
ChemSpider
388307
ChemSpider
Thioredoxin reductase 2, mitochondrial
22833590
PubChem-compound
C00141
KEGG Compound
TRANS-3-METHYL-GLUTACONYL-COA
BioCyc
940-69-2
CAS
1.0
1.8.1.4
false
1.8.1.4
Dihydrolipoamide + NAD → Hydrogen Ion + Lipoamide + NADH
LEFT_TO_RIGHT
C6H10O3
Ketoleucine
130.06299
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
328-50-7
CAS
HMDB0000254
HMDB
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
PW121693
PathWhiz
1.0
HMDB0000019
HMDB
Unknown
HYDROGEN-PEROXIDE
BioCyc
Adenosine diphosphate
HMDB0000491
HMDB
2-Methyl-1-hydroxypropyl-ThPP
HMDB0001341
HMDB
1.0
2.0
C17H29N4O8P2S
3-Methyl-1-hydroxybutyl-ThPP
511.11813
C00164
KEGG Compound
C16H27N4O8P2S
2-Methyl-1-hydroxypropyl-ThPP
497.10248
C13H25NO2S2
S-(3-Methylbutanoyl)-dihydrolipoamide-E
291.13266
C12H23NO2S2
S-(2-Methylpropionyl)-dihydrolipoamide-E
277.11703
Q9JLT4
UniProt
HMDB0001113
HMDB
1.0
HMDB0000023
HMDB
763
ChemSpider
C13H25NO2S2
S-(2-Methylbutanoyl)-dihydrolipoamide
291.13266
HMDB0001356
HMDB
C26H44N7O18P3S
3-Hydroxyisovaleryl-CoA
867.16766
CHEBI:9532
ChEBI
SMILES
CC(C)=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
C8H15NOS2
Lipoamide
205.05951
663
PubChem-compound
Oxoglutaric acid
1.0
7732-18-5
CAS
4.0
NAD
1.0
C00183
KEGG Compound
1.0
56-86-0
CAS
HMDB0002217
HMDB
1.0
1.0
Alpha-aminoadipic semialdehyde dehydrogenase
1.0
C01033
KEGG Compound
Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
1.0
2.3.3.10
false
2.3.3.10
Acetoacetyl-CoA + Acetyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA + Coenzyme A
LEFT_TO_RIGHT
HMDB0001377
HMDB
C03460
KEGG Compound
Hydroxymethylglutaryl-CoA lyase, mitochondrial
SMILES
CC(=O)CC(O)=O
3-HYDROXY-ISOBUTYRATE
BioCyc
CHEBI:35932
ChEBI
Enoyl-CoA hydratase, mitochondrial
SMILES
C\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
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
C8H17NOS2
Dihydrolipoamide
207.07515
C6H13NO2
L-Leucine
131.09464
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
HMDB0001372
HMDB
HMDB0001375
HMDB
1.0
ISOVALERYL-COA
BioCyc
S-(2-Methylbutanoyl)-dihydrolipoamide
5800
ChemSpider
C03465
KEGG Compound
C03231
KEGG Compound
C6H13NO2
L-Isoleucine
131.09464
Hydrogen peroxide
SMILES
N[C@@H](CCC(O)=O)C(O)=O
58-64-0
CAS
1.0
4.0
HMDB0001157
HMDB
2068-83-9
CAS
METHYL-MALONYL-COA
BioCyc
C4H9NO2
(S)-beta-Aminoisobutyric acid
103.06333
HMDB0000060
HMDB
96
PubChem-compound
863
PubChem-compound
444493
PubChem-compound
LIPOAMIDE
BioCyc
1.0
ACETYL-COA
BioCyc
(S)-3-Hydroxyisobutyryl-CoA
H2O
Water
18.010565
HMDB0002255
HMDB
NAD
BioCyc
Hydroxymethylglutaryl-CoA lyase, mitochondrial
HMDB0059597
HMDB
1.0
749
ChemSpider
3-Hydroxyisovaleryl-CoA
71-52-3
CAS
HMDB0006869
HMDB
HMDB0006865
HMDB
HMDB0006866
HMDB
HMDB0006867
HMDB
C02170
KEGG Compound
HMDB0006868
HMDB
Q9DBL1
UniProt
2-Methylacetoacetyl-CoA
487
PubChem-compound
CHEBI:15603
ChEBI
SMILES
O
1.0
Hydroxymethylglutaryl-CoA lyase, mitochondrial
C03284
KEGG Compound
CHEBI:27821
ChEBI
1.0
TIGLYL-COA
BioCyc
CHEBI:15846
ChEBI
ADP
BioCyc
Pyrimidine Metabolism
SubPathway
Ketoleucine
10090
TAXONOMY
1553-55-5
CAS
SMILES
CC(C)CC(=O)C(O)=O
1038
PubChem-compound
GO:0005829
GENE ONTOLOGY
759-05-7
CAS
Tiglyl-CoA
Lipoamide
C5H6O5
Oxoglutaric acid
146.02153
C6H10O3
3-Methyl-2-oxovaleric acid
130.06299
439855
PubChem-compound
C4H6O4
Methylmalonic acid
118.02661
CHEBI:16908
ChEBI
1.0
Citric Acid Cycle
SubPathway
Hydroxymethylglutaryl-CoA synthase, mitochondrial
1.0
SMILES
CC(C)C(=O)SCCC(S)CCCCC(N)=O
SMILES
CC(C)CC(O)C1=[N+](CC2=C(N)N=C(C)N=C2)C(C)=C(CCOP(O)(=O)OP(O)(O)=O)S1
SMILES
CC(C)C(O)C1=[N+](CC2=C(N)N=C(C)N=C2)C(C)=C(CCOP(O)(=O)OP(O)(O)=O)S1
SMILES
CC(C)CC(=O)SCCC(S)CCCCC(N)=O
Q8BWT1
UniProt
HMDB0000202
HMDB
L-Valine
HMDB0000687
HMDB
SMILES
CCC(C)C(=O)SCCC(S)CCCCC(N)=O
SMILES
CC(C)(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
Mitochondrion
388909
ChemSpider
L-Isoleucine
electron-transfer flavoprotein
1.0
HMDB0000208
HMDB
Pyruvate carboxylase, mitochondrial
C03069
KEGG Compound
HMDB0000695
HMDB
SMILES
CCC(C)C(=O)C(O)=O
Water
SMILES
CC(C(O)=O)C(O)=O
5280564
PubChem-compound
SMILES
OC(=O)CCC(=O)C(O)=O
C26H44N7O18P3S
2-Methyl-3-hydroxybutyryl-CoA
867.16766
124-38-9
CAS
DIHYDROLIPOAMIDE
BioCyc
1460-34-0
CAS
Lipoamide
1.0
1.0
6050
ChemSpider
GLT
BioCyc
439869
PubChem-compound
Q9DBF1
UniProt
Hydrogen carbonate
10128175
ChemSpider
P61922
UniProt
1.0
1.0
Hydrogen Ion
1.0
SMILES
CC[C@H](C)[C@H](N)C(O)=O
Propanoate Metabolism
SubPathway
1.0
C4H6O4
Succinic acid
118.02661
Hydroxymethylglutaryl-CoA lyase, mitochondrial
3036931
PubChem-compound
(S)-3-Hydroxyisobutyric acid
6067
ChemSpider
3-Methyl-1-hydroxybutyl-ThPP
440873
PubChem-compound
1.0
Adenosine triphosphate
C10H15N5O10P2
Adenosine diphosphate
427.02942
SMILES
C[C@@H](CN)C(O)=O
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
CHEBI:48522
ChEBI
1.0
CHEBI:15449
ChEBI
6306
PubChem-compound
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
CPD-8587
BioCyc
CHEBI:16530
ChEBI
2-METHYL-3-HYDROXY-BUTYRYL-COA
BioCyc
Reduced electron-transfer flavoprotein
CHEBI:1146900
ChEBI
3-ketoacyl-CoA thiolase, mitochondrial
439434
PubChem-compound
1.0
21252287
PubChem-compound
Alpha-aminoadipic semialdehyde dehydrogenase
Aldehyde dehydrogenase family 16 member A1
ILE
BioCyc
C21H36N7O16P3S
Coenzyme A
767.11523
Adenosine diphosphate
O08756
UniProt
CHEBI:33094
ChEBI
ACETOACETYL-COA
BioCyc
CHEBI:15422
ChEBI
Coenzyme A
1.0
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
SMILES
CC(C)[C@H](N)C(O)=O
1.0
C27H44N7O20P3S
3-Hydroxy-3-methylglutaryl-CoA
911.1575
O2
Oxygen
31.98983
439214
PubChem-compound
ReactionCatalysis100912
ACTIVATION
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
C12H19N4O7P2S
Thiamine pyrophosphate
425.04498
15621-60-0
CAS
439218
PubChem-compound
1.0
1.0
Unknown
1.0
CHEBI:16526
ChEBI
CHEBI:16761
ChEBI
Q05920
UniProt
SMILES
CC(C)C(=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
10128135
ChemSpider
CHEBI:15487
ChEBI
CHEBI:15486
ChEBI
CHEBI:15488
ChEBI
PW000149
PathWhiz
3-Hydroxy-3-methylglutaryl-CoA
SMILES
NC(=O)CCCCC1CCSS1
5893
PubChem-compound
C00630
KEGG Compound
Unknown
Aldehyde dehydrogenase family 16 member A1
1420-36-6
CAS
1.0
516-05-2
CAS
C00407
KEGG Compound
1.0
VAL
BioCyc
3-ketoacyl-CoA thiolase, mitochondrial
1.0
1.0
280
PubChem-compound
SMILES
OC(=N)CCCCC(S)CCS
SMILES
CC(C)C[C@H](N)C(O)=O
CHEBI:27462
ChEBI
CHEBI:15467
ChEBI
Succinyl-CoA:3-ketoacid coenzyme A transferase 2B, mitochondrial
388491
ChemSpider
CHEBI:30860
ChEBI
SMILES
CCC(=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
541-50-4
CAS
S-(2-Methylbutanoyl)-dihydrolipoamide
1.0
1.0
CHEBI:15476
ChEBI
1061
PubChem-compound
CHEBI:15478
ChEBI
CHEBI:15477
ChEBI
CHEBI:15479
ChEBI
Aldehyde dehydrogenase, mitochondrial
HMDB0001967
HMDB
1.0
1.0
HMDB0000883
HMDB
1.0
6244-91-3
CAS
1.0