CHEBI:16015
ChEBI
A1L107
UniProt
C26H44N7O17P3S
2-Methylbutyryl-CoA
851.1727
439161
PubChem-compound
Dihydrolipoyl dehydrogenase, mitochondrial
C25H42N7O18P3S
(S)-3-Hydroxyisobutyryl-CoA
853.152
439153
PubChem-compound
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
52
ChemSpider
D-METHYL-MALONYL-COA
BioCyc
50
ChemSpider
2-METHYL-BUTYRYL-COA
BioCyc
Aldehyde dehydrogenase, mitochondrial
73-32-5
CAS
C27H42N7O19P3S
3-Methylglutaconyl-CoA
893.1469
C00683
KEGG Compound
48
ChemSpider
46
ChemSpider
Dihydrolipoamide
1.0
HMDB0002166
HMDB
C25H40N7O17P3S
Methacrylyl-CoA
835.1414
Hydroxymethylglutaryl-CoA lyase, mitochondrial
Isovaleryl-CoA
HCO3
BioCyc
CHEBI:15380
ChEBI
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
CHEBI:16240
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@@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
1.8.1.4
false
1.8.1.4
Dihydrolipoamide + NAD → Hydrogen Ion + Lipoamide + NADH
LEFT_TO_RIGHT
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
P07633
UniProt
C00007
KEGG Compound
C00248
KEGG Compound
C00001
KEGG Compound
C00004
KEGG Compound
C00003
KEGG Compound
3-Methylglutaconyl-CoA
C00002
KEGG Compound
METHACRYLYL-COA
BioCyc
86
ChemSpider
C15976
KEGG Compound
C15974
KEGG Compound
388897
ChemSpider
SubPathwayInteraction108612
SubPathway108612Reaction
SubPathwayReaction
SubPathwayInteraction108613
SubPathway108613Reaction
SubPathwayReaction
SubPathwayInteraction108614
SubPathwayReaction
SubPathway108614Reaction
73173-91-8
CAS
SubPathwayInteraction108615
SubPathway108615Reaction
SubPathwayReaction
C00011
KEGG Compound
C00010
KEGG Compound
Oxygen
3-HYDROXY-3-METHYL-GLUTARYL-COA
BioCyc
H
Hydrogen Ion
1.007825
1.0
P08503
UniProt
1.2.4.4
false
1.2.4.4
3-Methyl-1-hydroxybutyl-ThPP + Lipoamide → S-(3-Methylbutanoyl)-dihydrolipoamide-E
LEFT_TO_RIGHT
4.1.3.4
false
4.1.3.4
3-Hydroxy-3-methylglutaryl-CoA → Acetyl-CoA
LEFT_TO_RIGHT
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
2-Methyl-1-hydroxypropyl-ThPP + 3-Methyl-1-hydroxybutyl-ThPP → Thiamine pyrophosphate
LEFT_TO_RIGHT
1.2.4.4
false
1.2.4.4
2-Methyl-1-hydroxypropyl-ThPP → S-(2-Methylpropionyl)-dihydrolipoamide-E
LEFT_TO_RIGHT
56-65-5
CAS
53-84-9
CAS
L-Glutamic acid
C00027
KEGG Compound
C00026
KEGG Compound
C00025
KEGG Compound
C00024
KEGG Compound
317-66-8
CAS
CHEBI:28259
ChEBI
(S)-Methylmalonic acid semialdehyde
26333171
ChemSpider
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
1.0
1.2.4.4
false
1.2.4.4
2-Methyl-1-hydroxypropyl-ThPP + Lipoamide → S-(2-Methylbutanoyl)-dihydrolipoamide + Thiamine pyrophosphate
LEFT_TO_RIGHT
4.0
1.2.4.4
false
1.2.4.4
Lipoamide → S-(2-Methylpropionyl)-dihydrolipoamide-E
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
2.6.1.42
false
2.6.1.42
L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic acid
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
1.2.4.4
false
1.2.4.4
Ketoleucine + Thiamine pyrophosphate ? 3-Methyl-1-hydroxybutyl-ThPP + Carbon dioxide
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
769
PubChem-compound
5462303
PubChem-compound
2.3.1.168
false
2.3.1.168
Coenzyme A + S-(3-Methylbutanoyl)-dihydrolipoamide-E → Dihydrolipoamide + Isovaleryl-CoA
LEFT_TO_RIGHT
793193-48-3
CAS
S-Methylmalonyl-CoA
1.3.8.4
false
1.3.8.4
Isovaleryl-CoA + electron-transfer flavoprotein → 3-Methylcrotonyl-CoA + Reduced electron-transfer flavoprotein
LEFT_TO_RIGHT
1.3.8.7
false
1.3.8.7
Isovaleryl-CoA + electron-transfer flavoprotein ↔ 3-Methylcrotonyl-CoA + Reduced electron-transfer flavoprotein
REVERSIBLE
4.2.1.17
false
4.2.1.17
3-Hydroxyisovaleryl-CoA → 3-Methylcrotonyl-CoA + Water
LEFT_TO_RIGHT
3-HYDROXY-ISOBUTYRYL-COA
BioCyc
4.0
SMILES
OC(=O)CCC(O)=O
1.0
Acetoacetic acid
HMDB0002310
HMDB
6.4.1.4
false
6.4.1.4
3-Methylcrotonyl-CoA + Adenosine triphosphate + Hydrogen carbonate → 3-Methylglutaconyl-CoA + Adenosine diphosphate
LEFT_TO_RIGHT
Propionyl-CoA carboxylase alpha chain, mitochondrial
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
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
1.3.8.1
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
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
1.1.1.31
false
1.1.1.31
(S)-3-Hydroxyisobutyric acid + NAD → (S)-Methylmalonic acid semialdehyde + Hydrogen Ion + NADH
LEFT_TO_RIGHT
Acetyl-CoA acetyltransferase, mitochondrial
11953876
PubChem-compound
C25H40N7O19P3S
R-Methylmalonyl-CoA
867.1312
3-METHYL-CROTONYL-COA
BioCyc
C04405
KEGG Compound
1.0
C00042
KEGG Compound
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
C00288
KEGG Compound
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
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
4-aminobutyrate aminotransferase, mitochondrial
604-98-8
CAS
1.0
HMDB0000148
HMDB
CHEBI:17191
ChEBI
Reaction147490
false
(S)-beta-Aminoisobutyric acid + Oxoglutaric acid ↔ (S)-Methylmalonic acid semialdehyde + L-Glutamic acid
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
4.2.1.17
false
4.2.1.17
Tiglyl-CoA + Water → 2-Methyl-3-hydroxybutyryl-CoA
LEFT_TO_RIGHT
Reaction147494
false
2-Methyl-3-hydroxybutyryl-CoA + NAD ↔ 2-Methylacetoacetyl-CoA + NADH
REVERSIBLE
784
PubChem-compound
2.3.1.16
false
2.3.1.16
2-Methylacetoacetyl-CoA + Coenzyme A → Acetyl-CoA + Propionyl-CoA
LEFT_TO_RIGHT
Reaction147496
false
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD + Water → Hydrogen carbonate + NADH + Propionyl-CoA
LEFT_TO_RIGHT
6.4.1.3
false
6.4.1.3
Adenosine triphosphate + Hydrogen carbonate + Propionyl-CoA → Adenosine diphosphate + Phosphate + S-Methylmalonyl-CoA
LEFT_TO_RIGHT
5.1.99.1
false
5.1.99.1
S-Methylmalonyl-CoA → R-Methylmalonyl-CoA
LEFT_TO_RIGHT
5.4.99.2
false
5.4.99.2
R-Methylmalonyl-CoA → Succinyl-CoA
LEFT_TO_RIGHT
2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency
HMDB0001487
HMDB
72-18-4
CAS
Isobutyryl-CoA
389707
ChemSpider
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
Branched-chain-amino-acid aminotransferase, cytosolic
2K-4CH3-PENTANOATE
BioCyc
HMDB0001493
HMDB
Q5XIT9
UniProt
HMDB0001011
HMDB
1.0
962
PubChem-compound
Acetoacetic acid
3-Methyl-1-hydroxybutyl-ThPP
2-KETO-3-METHYL-VALERATE
BioCyc
CO2
Carbon dioxide
43.98983
Methylmalonyl CoA epimerase
C03344
KEGG Compound
C03345
KEGG Compound
C00080
KEGG Compound
C25H40N7O19P3S
S-Methylmalonyl-CoA
867.1312
SMILES
O=O
2.0
2-Methyl-3-hydroxybutyryl-CoA
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
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
SMILES
CC1=C(CCO[P@](O)(=O)OP(O)(O)=O)SC=[N+]1CC1=CN=C(C)N=C1N
HMDB0001022
HMDB
14265-44-2
CAS
Enoyl-CoA hydratase, mitochondrial
1.0
99043-16-0
CAS
11953835
PubChem-compound
C00091
KEGG Compound
840
ChemSpider
Branched-chain-amino-acid aminotransferase, cytosolic
6247-73-0
CAS
P15651
UniProt
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
L-Leucine
Methacrylyl-CoA
HMDB0001275
HMDB
α-Ketoisovaleric acid
4575365
ChemSpider
1100
ChemSpider
3-Methylglutaconyl-CoA
Isovaleryl-CoA dehydrogenase, mitochondrial
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
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
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
HMDB0001041
HMDB
1.0
NADH
BioCyc
977
PubChem-compound
1.0
3-ketoacyl-CoA thiolase, mitochondrial
HMDB0001057
HMDB
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
3-hydroxyisobutyrate dehydrogenase, mitochondrial
CHO3
Hydrogen carbonate
60.99257
HMDB0001052
HMDB
Propionyl-CoA carboxylase beta chain, mitochondrial
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
30572
ChemSpider
Enoyl-CoA hydratase, mitochondrial
SMP0120660
SMPDB
1132
PubChem-compound
2.0
SMILES
OO
1.0
P70584
UniProt
22378483
ChemSpider
1.0
HMDB0000538
HMDB
52227-66-4
CAS
6816
PubChem-compound
51
PubChem-compound
P14882
UniProt
11471767
ChemSpider
SMILES
OC([O-])=O
C26H42N7O17P3S
Tiglyl-CoA
849.15704
53
PubChem-compound
C4H6O3
Acetoacetic acid
102.03169
3-Methyl-2-oxovaleric acid
ReactionCatalysis137672
ACTIVATION
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
CHEBI:48430
ChEBI
274
ChemSpider
3-Methylcrotonyl-CoA
Reduced electron-transfer flavoprotein
Hydrogen Ion
88
PubChem-compound
C5H8O3
α-Ketoisovaleric acid
116.04734
P12007
UniProt
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
Aldehyde dehydrogenase, mitochondrial
Adenosine triphosphate
1.0
70
PubChem-compound
2-KETO-ISOVALERATE
BioCyc
3-Methyl-2-oxovaleric acid
Q5XIE6
UniProt
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
CHEBI:30915
ChEBI
SMP0000046
SMPDB
Ketoleucine
4444187
ChemSpider
3-hydroxyacyl-CoA dehydrogenase type-2
HMDB0001429
HMDB
SMILES
C[C@@H](CO)C(O)=O
P14604
UniProt
GO:0005759
GENE ONTOLOGY
P97519
UniProt
5462214
PubChem-compound
SMP0000057
SMPDB
49
PubChem-compound
2-Methylbutyryl-CoA
Oxoglutaric acid
CHEBI:15741
ChEBI
SMILES
O=C=O
1110
PubChem-compound
C06002
KEGG Compound
Acetyl-CoA acetyltransferase, mitochondrial
HMDB0000595
HMDB
4.0
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
473
ChemSpider
HMDB0001206
HMDB
5957
PubChem-compound
2.3.3.10
false
2.3.3.10
Acetoacetyl-CoA + Acetyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA + Coenzyme A
LEFT_TO_RIGHT
47
PubChem-compound
1.0
Q5I0C3
UniProt
3-hydroxyacyl-CoA dehydrogenase type-2
GO:0005739
GENE ONTOLOGY
1.0
1.0
C26H42N7O17P3S
3-Methylcrotonyl-CoA
849.15704
2300823
ChemSpider
319440-43-2
CAS
CHEBI:16414
ChEBI
3-KETOBUTYRATE
BioCyc
R-Methylmalonyl-CoA
10116
TAXONOMY
2-Methyl-1-hydroxypropyl-ThPP
P35738
UniProt
5682
ChemSpider
23724625
PubChem-compound
23724626
PubChem-compound
Q3B7V7
UniProt
17216177
ChemSpider
Mitochondrial Matrix
2-(alpha-lactyl)-thpp
BioCyc
CO-A
BioCyc
SMP0000016
SMPDB
4-aminobutyrate aminotransferase, mitochondrial
389295
ChemSpider
72-89-9
CAS
1078
ChemSpider
Methylmalonyl-CoA mutase, mitochondrial
1.0
Carbon dioxide
Aldehyde dehydrogenase, mitochondrial
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
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
1.0
3-Methylcrotonyl-CoA
NADH
Hydroxymethylglutaryl-CoA lyase, mitochondrial
C02939
KEGG Compound
CHEBI:17544
ChEBI
P11960
UniProt
2-KETOGLUTARATE
BioCyc
392413
ChemSpider
61-90-5
CAS
1.0
F1LU71
UniProt
110-15-6
CAS
1.0
SMILES
C[C@@H](C=O)C(O)=O
13628334
ChemSpider
P54690
UniProt
CHEBI:15377
ChEBI
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
CHEBI:15379
ChEBI
CHEBI:15378
ChEBI
SMILES
[H+]
6.0
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
Thiamine pyrophosphate
CHEBI:15344
ChEBI
CHEBI:37373
ChEBI
CHEBI:15345
ChEBI
PW000005
PathWhiz
1.0
389466
ChemSpider
1.0
Hydroxymethylglutaryl-CoA lyase, mitochondrial
389465
ChemSpider
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
Reaction147500
false
(S)-Methylmalonic acid semialdehyde + Oxygen + Water → Hydrogen peroxide + Methylmalonic acid
LEFT_TO_RIGHT
1.2.1.3
false
1.2.1.3
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH
LEFT_TO_RIGHT
PW121916
PathWhiz
(S)-beta-Aminoisobutyric acid
SubPathwayInput
4.2.1.18
false
4.2.1.18
3-Methylglutaconyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA
LEFT_TO_RIGHT
439371
PubChem-compound
2.3.1.9
false
2.3.1.9
2 Acetyl-CoA → Acetoacetyl-CoA + Coenzyme A
LEFT_TO_RIGHT
2.0
CHEBI:15351
ChEBI
2.8.3.5
false
2.8.3.5
Acetoacetic acid + Succinyl-CoA → Acetoacetyl-CoA + Succinic acid
LEFT_TO_RIGHT
3884-47-7
CAS
85-61-0
CAS
1.0
4-aminobutyrate aminotransferase, mitochondrial
Dihydrolipoyl dehydrogenase, mitochondrial
C5H11NO2
L-Valine
117.07898
388357
ChemSpider
6022
PubChem-compound
3-hydroxyacyl-CoA dehydrogenase type-2
7722-84-1
CAS
C23H38N7O17P3S
Acetyl-CoA
809.1258
CHEBI:17460
ChEBI
937
ChemSpider
388353
ChemSpider
HMDB0003125
HMDB
440021
PubChem-compound
Succinic acid
HMDB0006870
HMDB
D4A197
UniProt
1.0
ATP
BioCyc
4249-19-8
CAS
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
S-(2-Methylpropionyl)-dihydrolipoamide-E
1.0
HMDB0002054
HMDB
C00100
KEGG Compound
Aldehyde oxidase 1
1010
ChemSpider
CHEBI:17694
ChEBI
2-oxoisovalerate dehydrogenase
Rattus norvegicus
Q6P6R2
UniProt
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
73173-92-9
CAS
Hydroxymethylglutaryl-CoA synthase, mitochondrial
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Isovaleryl-CoA dehydrogenase, mitochondrial
S-(3-Methylbutanoyl)-dihydrolipoamide-E
1.0
LEU
BioCyc
PROPIONYL-COA
BioCyc
NADH
Lipoamide
Dihydrolipoyl dehydrogenase, mitochondrial
CHEBI:18367
ChEBI
2-Methyl-1-hydroxypropyl-ThPP
1032
ChemSpider
C00123
KEGG Compound
Phosphate
C21H28N7O14P2
NAD
664.11694
S-(2-Methylpropionyl)-dihydrolipoamide-E
HMDB0000902
HMDB
Succinyl-CoA
SubPathwayInput
388543
ChemSpider
388307
ChemSpider
22833590
PubChem-compound
C00141
KEGG Compound
TRANS-3-METHYL-GLUTACONYL-COA
BioCyc
940-69-2
CAS
1.0
B2GV06
UniProt
ReactionCatalysis137730
ACTIVATION
ReactionCatalysis137731
ACTIVATION
ReactionCatalysis137732
ACTIVATION
C6H10O3
Ketoleucine
130.06299
ReactionCatalysis137733
ACTIVATION
ReactionCatalysis137738
ACTIVATION
ReactionCatalysis137739
ACTIVATION
ReactionCatalysis137734
ACTIVATION
ReactionCatalysis137735
ACTIVATION
ReactionCatalysis137736
ACTIVATION
ReactionCatalysis137737
ACTIVATION
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
1.0
HMDB0000019
HMDB
HYDROGEN-PEROXIDE
BioCyc
Adenosine diphosphate
HMDB0000491
HMDB
2-Methyl-1-hydroxypropyl-ThPP
HMDB0001341
HMDB
ReactionCatalysis137741
ACTIVATION
ReactionCatalysis137742
ACTIVATION
ReactionCatalysis137743
ACTIVATION
ReactionCatalysis137744
ACTIVATION
1.0
ReactionCatalysis137740
ACTIVATION
ReactionCatalysis137749
ACTIVATION
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
ReactionCatalysis137745
ACTIVATION
C12H23NO2S2
S-(2-Methylpropionyl)-dihydrolipoamide-E
277.11703
ReactionCatalysis137746
ACTIVATION
ReactionCatalysis137747
ACTIVATION
ReactionCatalysis137748
ACTIVATION
HMDB0001113
HMDB
1.0
HMDB0000023
HMDB
Methylmalonyl CoA mutase
763
ChemSpider
C13H25NO2S2
S-(2-Methylbutanoyl)-dihydrolipoamide
291.13266
HMDB0001356
HMDB
C26H44N7O18P3S
3-Hydroxyisovaleryl-CoA
867.16766
CHEBI:9532
ChEBI
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
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
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
C8H15NOS2
Lipoamide
205.05951
663
PubChem-compound
ReactionCatalysis137711
ACTIVATION
Oxoglutaric acid
ReactionCatalysis137716
ACTIVATION
ReactionCatalysis137717
ACTIVATION
ReactionCatalysis137718
ACTIVATION
1.0
ReactionCatalysis137719
ACTIVATION
ReactionCatalysis137712
ACTIVATION
7732-18-5
CAS
ReactionCatalysis137713
ACTIVATION
ReactionCatalysis137714
ACTIVATION
ReactionCatalysis137715
ACTIVATION
NAD
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
4.0
1.0
C00183
KEGG Compound
1.0
56-86-0
CAS
HMDB0002217
HMDB
P17764
UniProt
P50554
UniProt
1.0
ReactionCatalysis137720
ACTIVATION
1.0
ReactionCatalysis137721
ACTIVATION
ReactionCatalysis137722
ACTIVATION
1.0
ReactionCatalysis137727
ACTIVATION
ReactionCatalysis137728
ACTIVATION
ReactionCatalysis137729
ACTIVATION
C01033
KEGG Compound
ReactionCatalysis137723
ACTIVATION
1.0
ReactionCatalysis137724
ACTIVATION
ReactionCatalysis137725
ACTIVATION
ReactionCatalysis137726
ACTIVATION
HMDB0001377
HMDB
C03460
KEGG Compound
SMILES
CC(=O)CC(O)=O
3-HYDROXY-ISOBUTYRATE
BioCyc
CHEBI:35932
ChEBI
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
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
Dihydrolipoyl dehydrogenase E3
C03465
KEGG Compound
C03231
KEGG Compound
C6H13NO2
L-Isoleucine
131.09464
Hydrogen peroxide
SMILES
N[C@@H](CCC(O)=O)C(O)=O
4.0
Methylglutaconyl-CoA hydratase, mitochondrial
58-64-0
CAS
1.0
HMDB0001157
HMDB
2068-83-9
CAS
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
METHYL-MALONYL-COA
BioCyc
C4H9NO2
(S)-beta-Aminoisobutyric acid
103.06333
HMDB0000060
HMDB
4.0
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
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
HMDB0059597
HMDB
1.0
3-ketoacyl-CoA thiolase, mitochondrial
749
ChemSpider
3-Hydroxyisovaleryl-CoA
71-52-3
CAS
HMDB0006869
HMDB
HMDB0006865
HMDB
HMDB0006866
HMDB
HMDB0006867
HMDB
C02170
KEGG Compound
HMDB0006868
HMDB
2-Methylacetoacetyl-CoA
487
PubChem-compound
ReactionCatalysis137331
ACTIVATION
CHEBI:15603
ChEBI
Q02253
UniProt
SMILES
O
1.0
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
P13437
UniProt
C03284
KEGG Compound
CHEBI:27821
ChEBI
1.0
TIGLYL-COA
BioCyc
CHEBI:15846
ChEBI
ADP
BioCyc
Pyrimidine Metabolism
SubPathway
3-hydroxyisobutyrate dehydrogenase, mitochondrial
Ketoleucine
1553-55-5
CAS
SMILES
CC(C)CC(=O)C(O)=O
1038
PubChem-compound
2.0
GO:0005829
GENE ONTOLOGY
759-05-7
CAS
Tiglyl-CoA
4.0
Lipoamide
C5H6O5
Oxoglutaric acid
146.02153
C6H10O3
3-Methyl-2-oxovaleric acid
130.06299
439855
PubChem-compound
Hydroxymethylglutaryl-CoA lyase, mitochondrial
C4H6O4
Methylmalonic acid
118.02661
CHEBI:16908
ChEBI
1.0
Citric Acid Cycle
SubPathway
Enoyl-CoA hydratase, 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
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
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
Methylmalonyl CoA epimerase
Hydroxymethylglutaryl-CoA lyase, mitochondrial
electron-transfer flavoprotein
1.0
HMDB0000208
HMDB
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
Propionyl-CoA carboxylase alpha chain, mitochondrial
Propionyl-CoA carboxylase beta chain, mitochondrial
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
DIHYDROLIPOAMIDE
BioCyc
1460-34-0
CAS
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
Lipoamide
1.0
1.0
6050
ChemSpider
2.0
GLT
BioCyc
439869
PubChem-compound
Methylmalonyl CoA mutase
Hydrogen carbonate
Aldehyde oxidase 1
Methylglutaconyl-CoA hydratase, mitochondrial
ReactionCatalysis137752
ACTIVATION
10128175
ChemSpider
ReactionCatalysis137753
ACTIVATION
ReactionCatalysis137754
ACTIVATION
ReactionCatalysis137755
ACTIVATION
ReactionCatalysis137750
ACTIVATION
ReactionCatalysis137751
ACTIVATION
1.0
1.0
ReactionCatalysis137756
ACTIVATION
ReactionCatalysis137757
ACTIVATION
ReactionCatalysis137758
ACTIVATION
Hydrogen Ion
ReactionCatalysis137759
ACTIVATION
1.0
3-ketoacyl-CoA thiolase, mitochondrial
SMILES
CC[C@H](C)[C@H](N)C(O)=O
Propanoate Metabolism
SubPathway
1.0
C4H6O4
Succinic acid
118.02661
3036931
PubChem-compound
ReactionCatalysis137760
ACTIVATION
ReactionCatalysis137761
ACTIVATION
(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
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
439434
PubChem-compound
1.0
21252287
PubChem-compound
ILE
BioCyc
C21H36N7O16P3S
Coenzyme A
767.11523
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
Adenosine diphosphate
D3ZKG1
UniProt
CHEBI:33094
ChEBI
ACETOACETYL-COA
BioCyc
CHEBI:15422
ChEBI
Coenzyme A
1.0
Acetyl-CoA acetyltransferase, mitochondrial
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
C12H19N4O7P2S
Thiamine pyrophosphate
425.04498
15621-60-0
CAS
439218
PubChem-compound
1.0
Hydroxymethylglutaryl-CoA synthase, mitochondrial
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
1.0
CHEBI:16526
ChEBI
P22791
UniProt
CHEBI:16761
ChEBI
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
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
CHEBI:15487
ChEBI
CHEBI:15486
ChEBI
Dihydrolipoyl dehydrogenase E3
CHEBI:15488
ChEBI
PW000149
PathWhiz
3-Hydroxy-3-methylglutaryl-CoA
SMILES
NC(=O)CCCCC1CCSS1
5893
PubChem-compound
C00630
KEGG Compound
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
1420-36-6
CAS
1.0
Q9QYD4
UniProt
516-05-2
CAS
C00407
KEGG Compound
2.0
1.0
VAL
BioCyc
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
Q9R240
UniProt
2-oxoisovalerate dehydrogenase
CHEBI:15467
ChEBI
1.0
Methylcrotonoyl-CoA carboxylase subunit alpha, 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
2.0
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
1.0
HMDB0001967
HMDB
4.0
1.0
1.0
Q91ZD7
UniProt
HMDB0000883
HMDB
1.0
6244-91-3
CAS
1.0