CHEBI:16015
ChEBI
C26H44N7O17P3S
2-Methylbutyryl-CoA
851.1727
439161
PubChem-compound
C25H42N7O18P3S
(S)-3-Hydroxyisobutyryl-CoA
853.152
1.0
3-hydroxyisobutyrate dehydrogenase, mitochondrial
439153
PubChem-compound
D-METHYL-MALONYL-COA
BioCyc
52
ChemSpider
Enoyl-CoA hydratase, mitochondrial
50
ChemSpider
2-METHYL-BUTYRYL-COA
BioCyc
73-32-5
CAS
C27H42N7O19P3S
3-Methylglutaconyl-CoA
893.1469
C00683
KEGG Compound
48
ChemSpider
46
ChemSpider
P45954
UniProt
Dihydrolipoamide
1.0
1.3.8.1
false
1.3.8.1
Isobutyryl-CoA + electron-transfer flavoprotein → Methacrylyl-CoA + Reduced electron-transfer flavoprotein
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
HMDB0002166
HMDB
2.3.1.168
false
2.3.1.168
Coenzyme A + S-(2-Methylbutanoyl)-dihydrolipoamide → 2-Methylbutyryl-CoA + Dihydrolipoamide
LEFT_TO_RIGHT
C25H40N7O17P3S
Methacrylyl-CoA
835.1414
2.0
Isovaleryl-CoA
P09622
UniProt
HCO3
BioCyc
CHEBI:15380
ChEBI
5742
ChemSpider
C25H40N7O19P3S
Succinyl-CoA
867.1312
P35914
UniProt
Acetyl-CoA
SubPathwayInput
PW000160
PathWhiz
ISOBUTYRYL-COA
BioCyc
154-87-0
CAS
Isovaleryl-CoA dehydrogenase, mitochondrial
ReactionCatalysis601
ACTIVATION
ReactionCatalysis602
ACTIVATION
L-Glutamic acid
CHEBI:17577
ChEBI
CHEBI:16240
ChEBI
2.3.1.9
false
2.3.1.9
2 Acetyl-CoA → Acetoacetyl-CoA + Coenzyme A
LEFT_TO_RIGHT
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
2.8.3.5
false
2.8.3.5
Acetoacetic acid + Succinyl-CoA → Acetoacetyl-CoA + Succinic acid
LEFT_TO_RIGHT
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
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
6712-02-3
CAS
1.0
ReactionCatalysis629
ACTIVATION
ReactionCatalysis627
ACTIVATION
ReactionCatalysis628
ACTIVATION
C26H44N7O17P3S
Isovaleryl-CoA
851.1727
94
ChemSpider
ReactionCatalysis624
ACTIVATION
C00009
KEGG Compound
C00008
KEGG Compound
C00007
KEGG Compound
C00248
KEGG Compound
Q9UKU7
UniProt
C00001
KEGG Compound
P54868
UniProt
C00004
KEGG Compound
C00003
KEGG Compound
3-Methylglutaconyl-CoA
C00002
KEGG Compound
METHACRYLYL-COA
BioCyc
86
ChemSpider
ReactionCatalysis615
ACTIVATION
ReactionCatalysis612
ACTIVATION
C15976
KEGG Compound
ReactionCatalysis613
ACTIVATION
C15974
KEGG Compound
388897
ChemSpider
73173-91-8
CAS
C00011
KEGG Compound
C00010
KEGG Compound
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
Oxygen
P21953
UniProt
3-HYDROXY-3-METHYL-GLUTARYL-COA
BioCyc
Aldehyde oxidase
4.0
3-hydroxyacyl-CoA dehydrogenase type-2
H
Hydrogen Ion
1.007825
1.0
56-65-5
CAS
53-84-9
CAS
3-hydroxyisobutyrate dehydrogenase, mitochondrial
L-Glutamic acid
C00027
KEGG Compound
C00026
KEGG Compound
C00025
KEGG Compound
C00024
KEGG Compound
317-66-8
CAS
CHEBI:28259
ChEBI
Dihydrolipoyl dehydrogenase E3
(S)-Methylmalonic acid semialdehyde
3-hydroxyacyl-CoA dehydrogenase type-2
26333171
ChemSpider
1.0
5462303
PubChem-compound
769
PubChem-compound
S-Methylmalonyl-CoA
793193-48-3
CAS
3-HYDROXY-ISOBUTYRYL-COA
BioCyc
P31937
UniProt
SMILES
OC(=O)CCC(O)=O
1.0
Acetoacetic acid
HMDB0002310
HMDB
11953876
PubChem-compound
C25H40N7O19P3S
R-Methylmalonyl-CoA
867.1312
1.0
C04405
KEGG Compound
3-METHYL-CROTONYL-COA
BioCyc
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
CHEBI:17191
ChEBI
784
PubChem-compound
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
HMDB0001487
HMDB
72-18-4
CAS
Isobutyryl-CoA
389707
ChemSpider
HMDB0001243
HMDB
HMDB0001484
HMDB
C10H16N5O13P3
Adenosine triphosphate
506.99576
C26H42N7O18P3S
2-Methylacetoacetyl-CoA
865.152
1.0
CHEBI:28291
ChEBI
C4H6O3
(S)-Methylmalonic acid semialdehyde
102.03169
C05998
KEGG Compound
C04424
KEGG Compound
NAD
1.0
C00068
KEGG Compound
2K-4CH3-PENTANOATE
BioCyc
HMDB0001493
HMDB
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
HMDB0001011
HMDB
962
PubChem-compound
1.0
Acetoacetic acid
3-Methyl-1-hydroxybutyl-ThPP
2-KETO-3-METHYL-VALERATE
BioCyc
CO2
Carbon dioxide
43.98983
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
Q9HCC0
UniProt
HMDB0000172
HMDB
HMDB0002111
HMDB
SMILES
C[C@](O)(CC(O)=O)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
SMILES
CC1=C(CCOP(O)(=O)OP(O)(O)=O)SC=[N+]1CC1=CN=C(C)N=C1N
HMDB0001022
HMDB
14265-44-2
CAS
1.0
99043-16-0
CAS
11953835
PubChem-compound
C00091
KEGG Compound
840
ChemSpider
6247-73-0
CAS
L-Leucine
Methacrylyl-CoA
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
1.0
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
HMDB0001057
HMDB
HMDB0001052
HMDB
CH2O3
Hydrogen carbonate
62.000393
30572
ChemSpider
1132
PubChem-compound
Aldehyde oxidase
SMILES
OO
P12694
UniProt
1.0
1.2.4.4
false
1.2.4.4
2-Methyl-1-hydroxypropyl-ThPP + 3-Methyl-1-hydroxybutyl-ThPP → Thiamine pyrophosphate
LEFT_TO_RIGHT
22378483
ChemSpider
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
1.0
3-hydroxyacyl-CoA dehydrogenase type-2
HMDB0000538
HMDB
52227-66-4
CAS
2.0
6816
PubChem-compound
51
PubChem-compound
11471767
ChemSpider
SMILES
OC(O)=O
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
C26H42N7O17P3S
Tiglyl-CoA
849.15704
53
PubChem-compound
C4H6O3
Acetoacetic acid
102.03169
3-Methyl-2-oxovaleric acid
CHEBI:48430
ChEBI
Q99714
UniProt
274
ChemSpider
3-Methylcrotonyl-CoA
88
PubChem-compound
Reduced electron-transfer flavoprotein
Hydrogen Ion
C5H8O3
α-Ketoisovaleric acid
116.04734
C4H8O3
(S)-3-Hydroxyisobutyric acid
104.04734
H3O4P
Phosphate
97.9769
C05118
KEGG Compound
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
Adenosine triphosphate
1.0
70
PubChem-compound
2-KETO-ISOVALERATE
BioCyc
3-Methyl-2-oxovaleric acid
1.3.8.4
false
1.3.8.4
Isovaleryl-CoA + electron-transfer flavoprotein → 3-Methylcrotonyl-CoA + Reduced electron-transfer flavoprotein
LEFT_TO_RIGHT
2.3.1.168
false
2.3.1.168
Coenzyme A + S-(3-Methylbutanoyl)-dihydrolipoamide-E → Dihydrolipoamide + Isovaleryl-CoA
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
2.6.1.42
false
2.6.1.42
L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic acid
LEFT_TO_RIGHT
Carbon dioxide
1.0
CPD-6641
BioCyc
58-68-4
CAS
HMDB0001423
HMDB
CHEBI:27754
ChEBI
SMILES
OP(O)(O)=O
SMILES
CC(C)C(=O)C(O)=O
CHEBI:30915
ChEBI
SMP0000046
SMPDB
P30084
UniProt
Ketoleucine
4444187
ChemSpider
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
HMDB0001429
HMDB
SMILES
C[C@@H](CO)C(O)=O
4.0
GO:0005759
GENE ONTOLOGY
6.4.1.4
false
6.4.1.4
3-Methylcrotonyl-CoA + Adenosine triphosphate + Hydrogen carbonate → 3-Methylglutaconyl-CoA + Adenosine diphosphate
LEFT_TO_RIGHT
5462214
PubChem-compound
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
SMP0000057
SMPDB
4.2.1.18
false
4.2.1.18
3-Methylglutaconyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA
LEFT_TO_RIGHT
49
PubChem-compound
2-Methylbutyryl-CoA
Oxoglutaric acid
CHEBI:15741
ChEBI
SMILES
O=C=O
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
1110
PubChem-compound
C06002
KEGG Compound
HMDB0000595
HMDB
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, 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
473
ChemSpider
HMDB0001206
HMDB
5957
PubChem-compound
47
PubChem-compound
1.0
Reaction987
false
2-Methyl-1-hydroxypropyl-ThPP → S-(2-Methylpropionyl)-dihydrolipoamide-E
LEFT_TO_RIGHT
Enoyl-CoA hydratase, mitochondrial
GO:0005739
GENE ONTOLOGY
1.0
3-Methylglutaconic Aciduria Type III
1.0
2.6.1.42
false
2.6.1.42
L-Valine + Oxoglutaric acid → L-Glutamic acid + α-Ketoisovaleric acid
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
C26H42N7O17P3S
3-Methylcrotonyl-CoA
849.15704
319440-43-2
CAS
2300823
ChemSpider
ReactionCatalysis672
ACTIVATION
ReactionCatalysis671
ACTIVATION
CHEBI:16414
ChEBI
Hydroxymethylglutaryl-CoA lyase, mitochondrial
3-KETOBUTYRATE
BioCyc
R-Methylmalonyl-CoA
2-Methyl-1-hydroxypropyl-ThPP
4.0
5682
ChemSpider
23724625
PubChem-compound
23724626
PubChem-compound
17216177
ChemSpider
Mitochondrial Matrix
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
ReactionCatalysis666
ACTIVATION
2-(alpha-lactyl)-thpp
BioCyc
CO-A
BioCyc
SMP0000016
SMPDB
389295
ChemSpider
P05091
UniProt
72-89-9
CAS
1078
ChemSpider
1.0
Carbon dioxide
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
4-aminobutyrate aminotransferase, mitochondrial
6557
ChemSpider
Hydrogen carbonate
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
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
440326
PubChem-compound
440566
PubChem-compound
440565
PubChem-compound
6712-01-2
CAS
1.0
CHEBI:15539
ChEBI
ReactionCatalysis689
ACTIVATION
ReactionCatalysis687
ACTIVATION
C5H9NO4
L-Glutamic acid
147.05316
ReactionCatalysis688
ACTIVATION
33032
PubChem-compound
ReactionCatalysis685
ACTIVATION
ReactionCatalysis686
ACTIVATION
Thiamine pyrophosphate
ReactionCatalysis684
ACTIVATION
Dihydrolipoyl dehydrogenase, mitochondrial
ReactionCatalysis682
ACTIVATION
Propionyl-CoA carboxylase beta chain, mitochondrial
ReactionCatalysis680
ACTIVATION
P11310
UniProt
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
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
389035
ChemSpider
7782-44-7
CAS
HMDB0000962
HMDB
1.0
3-Methylcrotonyl-CoA
NADH
ReactionCatalysis679
ACTIVATION
9606
TAXONOMY
CHEBI:17544
ChEBI
C02939
KEGG Compound
2-KETOGLUTARATE
BioCyc
392413
ChemSpider
61-90-5
CAS
1.0
1.0
Hydroxymethylglutaryl-CoA synthase, mitochondrial
110-15-6
CAS
1.0
SMILES
C[C@@H](C=O)C(O)=O
13628334
ChemSpider
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
Q6NVY1
UniProt
HMDB0000985
HMDB
Acetyl-CoA acetyltransferase, mitochondrial
Dihydrolipoyl dehydrogenase, mitochondrial
Thiamine pyrophosphate
CHEBI:15344
ChEBI
CHEBI:37373
ChEBI
CHEBI:15345
ChEBI
PW000005
PathWhiz
Isobutyryl-CoA dehydrogenase, mitochondrial
389466
ChemSpider
1.0
389465
ChemSpider
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
Thiamine pyrophosphate
2.0
3-METHYLBENZYLSUCCINYL-COA
BioCyc
P54687
UniProt
(S)-beta-Aminoisobutyric acid
SubPathwayInput
439371
PubChem-compound
CHEBI:15351
ChEBI
3884-47-7
CAS
4-aminobutyrate aminotransferase, mitochondrial
85-61-0
CAS
1.0
C5H11NO2
L-Valine
117.07898
PW000067
PathWhiz
ReactionCatalysis2091
ACTIVATION
388357
ChemSpider
6022
PubChem-compound
7722-84-1
CAS
ReactionCatalysis2090
ACTIVATION
C23H38N7O17P3S
Acetyl-CoA
809.1258
CHEBI:17460
ChEBI
937
ChemSpider
6.0
388353
ChemSpider
Reaction1011
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
HMDB0003125
HMDB
Reaction1010
false
(S)-Methylmalonic acid semialdehyde + Oxygen + Water → Hydrogen peroxide + Methylmalonic acid
LEFT_TO_RIGHT
440021
PubChem-compound
Succinic acid
HMDB0006870
HMDB
1.0
Hydroxymethylglutaryl-CoA synthase, mitochondrial
ATP
BioCyc
4249-19-8
CAS
Acetoacetyl-CoA
C25H42N7O17P3S
Isobutyryl-CoA
837.15704
Hydroxymethylglutaryl-CoA lyase, mitochondrial
6008-91-9
CAS
3.1.2.4
false
3.1.2.4
(S)-3-Hydroxyisobutyryl-CoA + Water → (S)-3-Hydroxyisobutyric acid + Coenzyme A
LEFT_TO_RIGHT
1.3.8.7
false
1.3.8.7
Isobutyryl-CoA + electron-transfer flavoprotein ↔ Methacrylyl-CoA + Reduced electron-transfer flavoprotein
REVERSIBLE
Reaction1486
false
R-Methylmalonyl-CoA → Succinyl-CoA
LEFT_TO_RIGHT
4.2.1.17
false
4.2.1.17
Methacrylyl-CoA + Water → (S)-3-Hydroxyisobutyryl-CoA
LEFT_TO_RIGHT
6287
PubChem-compound
C00579
KEGG Compound
2-METHYL-ACETO-ACETYL-COA
BioCyc
C00332
KEGG Compound
2.3.1.168
false
2.3.1.168
Coenzyme A + S-(2-Methylpropionyl)-dihydrolipoamide-E → Dihydrolipoamide + Isobutyryl-CoA
LEFT_TO_RIGHT
952
ChemSpider
1.2.4.4
false
1.2.4.4
Lipoamide → S-(2-Methylpropionyl)-dihydrolipoamide-E
LEFT_TO_RIGHT
Aldehyde dehydrogenase, mitochondrial
Hydroxymethylglutaryl-CoA lyase, mitochondrial
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
S-(2-Methylpropionyl)-dihydrolipoamide-E
2.3.1.16
false
2.3.1.16
2-Methylacetoacetyl-CoA + Coenzyme A → Acetyl-CoA + Propionyl-CoA
LEFT_TO_RIGHT
4.2.1.17
false
4.2.1.17
Tiglyl-CoA + Water → 2-Methyl-3-hydroxybutyryl-CoA
LEFT_TO_RIGHT
Reaction1038
false
2-Methyl-3-hydroxybutyryl-CoA + NAD ↔ 2-Methylacetoacetyl-CoA + NADH
REVERSIBLE
2-oxoisovalerate dehydrogenase
1.0
HMDB0002054
HMDB
1.3.8.1
false
1.3.8.1
2-Methylbutyryl-CoA + electron-transfer flavoprotein → Reduced electron-transfer flavoprotein + Tiglyl-CoA
LEFT_TO_RIGHT
1.3.8.7
false
1.3.8.7
2-Methylbutyryl-CoA + electron-transfer flavoprotein ↔ Reduced electron-transfer flavoprotein + Tiglyl-CoA
REVERSIBLE
C00100
KEGG Compound
1010
ChemSpider
CHEBI:17694
ChEBI
Reaction1263
false
S-Methylmalonyl-CoA → R-Methylmalonyl-CoA
LEFT_TO_RIGHT
Branched-chain-amino-acid aminotransferase, cytosolic
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Methylglutaconyl-CoA hydratase, mitochondrial
Aldehyde dehydrogenase, mitochondrial
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Q13825
UniProt
1.2.4.4
false
1.2.4.4
2-Methyl-1-hydroxypropyl-ThPP + Lipoamide → S-(2-Methylbutanoyl)-dihydrolipoamide + Thiamine pyrophosphate
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
1.0
C24H40N7O17P3S
Propionyl-CoA
823.1414
1.0
C01213
KEGG Compound
Cytosol
P16219
UniProt
388310
ChemSpider
C00356
KEGG Compound
5880
ChemSpider
Propionyl-CoA
SubPathwayInput
1.0
73173-92-9
CAS
Reaction274
false
(S)-beta-Aminoisobutyric acid + Oxoglutaric acid ↔ (S)-Methylmalonic acid semialdehyde + L-Glutamic acid
REVERSIBLE
Branched-chain-amino-acid aminotransferase, cytosolic
S-(3-Methylbutanoyl)-dihydrolipoamide-E
2.0
3-ketoacyl-CoA thiolase, mitochondrial
1.0
LEU
BioCyc
PROPIONYL-COA
BioCyc
NADH
Lipoamide
CHEBI:18367
ChEBI
Hydroxymethylglutaryl-CoA synthase, mitochondrial
2-Methyl-1-hydroxypropyl-ThPP
1032
ChemSpider
C00123
KEGG Compound
Acetyl-CoA acetyltransferase, mitochondrial
P42765
UniProt
1.2.1.3
false
1.2.1.3
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH
LEFT_TO_RIGHT
Phosphate
ReactionCatalysis2063
ACTIVATION
ReactionCatalysis2065
ACTIVATION
ReactionCatalysis2068
ACTIVATION
ReactionCatalysis2067
ACTIVATION
C21H27N7O14P2
NAD
663.10913
ReactionCatalysis2073
ACTIVATION
S-(2-Methylpropionyl)-dihydrolipoamide-E
ReactionCatalysis2072
ACTIVATION
HMDB0000902
HMDB
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
P55809
UniProt
3-ketoacyl-CoA thiolase, mitochondrial
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
Succinyl-CoA
SubPathwayInput
Dihydrolipoyl dehydrogenase E3
4-aminobutyrate aminotransferase, mitochondrial
ReactionCatalysis2074
ACTIVATION
ReactionCatalysis2079
ACTIVATION
ReactionCatalysis2078
ACTIVATION
ReactionCatalysis2080
ACTIVATION
388543
ChemSpider
388307
ChemSpider
4.0
2.0
22833590
PubChem-compound
C00141
KEGG Compound
TRANS-3-METHYL-GLUTACONYL-COA
BioCyc
940-69-2
CAS
Propionyl-CoA carboxylase alpha chain, mitochondrial
Propionyl-CoA carboxylase beta chain, mitochondrial
1.0
2-oxoisovalerate dehydrogenase
ReactionCatalysis2087
ACTIVATION
ReactionCatalysis2089
ACTIVATION
C6H10O3
Ketoleucine
130.06299
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
HMDB0000254
HMDB
SMILES
CC(=O)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
1.0
HMDB0000019
HMDB
HYDROGEN-PEROXIDE
BioCyc
Adenosine diphosphate
HMDB0000491
HMDB
2-Methyl-1-hydroxypropyl-ThPP
HMDB0001341
HMDB
1.0
Acetyl-CoA acetyltransferase, mitochondrial
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
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
HMDB0001113
HMDB
HMDB0000023
HMDB
1.0
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
NAD
1.0
C00183
KEGG Compound
1.0
56-86-0
CAS
HMDB0002217
HMDB
1.0
1.0
1.0
C01033
KEGG Compound
1.0
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
C6H13NO2
L-Leucine
131.09464
C8H17NOS2
Dihydrolipoamide
207.07515
HMDB0001372
HMDB
HMDB0001375
HMDB
1.0
ISOVALERYL-COA
BioCyc
Aldehyde dehydrogenase, mitochondrial
S-(2-Methylbutanoyl)-dihydrolipoamide
5800
ChemSpider
SubPathwayInteraction759
SubPathwayReaction
SubPathway759Reaction
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
Q06278
UniProt
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
SubPathwayInteraction772
SubPathway772Reaction
SubPathwayReaction
444493
PubChem-compound
LIPOAMIDE
BioCyc
P26440
UniProt
1.0
ACETYL-COA
BioCyc
(S)-3-Hydroxyisobutyryl-CoA
H2O
Water
18.010565
Dihydrolipoyl dehydrogenase, mitochondrial
HMDB0002255
HMDB
P11182
UniProt
NAD
BioCyc
SubPathwayInteraction763
SubPathway763Reaction
SubPathwayReaction
SubPathwayInteraction767
SubPathway767Reaction
SubPathwayReaction
HMDB0059597
HMDB
1.0
749
ChemSpider
71-52-3
CAS
3-Hydroxyisovaleryl-CoA
HMDB0006869
HMDB
HMDB0006865
HMDB
HMDB0006866
HMDB
HMDB0006867
HMDB
C02170
KEGG Compound
HMDB0006868
HMDB
2-Methylacetoacetyl-CoA
487
PubChem-compound
CHEBI:15603
ChEBI
Q96RQ3
UniProt
Enoyl-CoA hydratase, mitochondrial
2.0
Q02252
UniProt
SMILES
O
1.0
C03284
KEGG Compound
CHEBI:27821
ChEBI
1.0
TIGLYL-COA
BioCyc
CHEBI:15846
ChEBI
ADP
BioCyc
1.0
Pyrimidine Metabolism
SubPathway
1.2.4.4
false
1.2.4.4
Thiamine pyrophosphate + α-Ketoisovaleric acid → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
LEFT_TO_RIGHT
Ketoleucine
1.2.4.4
false
1.2.4.4
Ketoleucine + Thiamine pyrophosphate ? 3-Methyl-1-hydroxybutyl-ThPP + Carbon dioxide
1553-55-5
CAS
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
SMILES
CC(C)CC(=O)C(O)=O
1038
PubChem-compound
GO:0005829
GENE ONTOLOGY
759-05-7
CAS
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
1.0
Tiglyl-CoA
C5H6O5
Oxoglutaric acid
146.02153
Lipoamide
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
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
4.0
Mitochondrion
388909
ChemSpider
4.0
L-Isoleucine
electron-transfer flavoprotein
1.0
HMDB0000208
HMDB
Isobutyryl-CoA dehydrogenase, 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
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
DIHYDROLIPOAMIDE
BioCyc
4.0
1460-34-0
CAS
ReactionCatalysis61
ACTIVATION
Lipoamide
1.0
1.0
6050
ChemSpider
P24752
UniProt
GLT
BioCyc
439869
PubChem-compound
Hydrogen carbonate
10128175
ChemSpider
1.0
1.0
Hydrogen Ion
Methylglutaconyl-CoA hydratase, mitochondrial
1.0
SMILES
CC[C@H](C)[C@H](N)C(O)=O
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
Propanoate Metabolism
SubPathway
1.0
C4H6O4
Succinic acid
118.02661
3036931
PubChem-compound
(S)-3-Hydroxyisobutyric acid
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
1.1.1.31
false
1.1.1.31
(S)-3-Hydroxyisobutyric acid + NAD → (S)-Methylmalonic acid semialdehyde + Hydrogen Ion + NADH
LEFT_TO_RIGHT
6067
ChemSpider
3-Methyl-1-hydroxybutyl-ThPP
440873
PubChem-compound
1.0
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
Adenosine triphosphate
C10H15N5O10P2
Adenosine diphosphate
427.02942
SMILES
C[C@@H](CN)C(O)=O
CHEBI:48522
ChEBI
ReactionCatalysis553
ACTIVATION
Propionyl-CoA carboxylase alpha chain, mitochondrial
ReactionCatalysis554
ACTIVATION
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
CPD-8587
BioCyc
electron-transfer flavoprotein
CHEBI:16530
ChEBI
2-METHYL-3-HYDROXY-BUTYRYL-COA
BioCyc
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
Reduced electron-transfer flavoprotein
CHEBI:1146900
ChEBI
439434
PubChem-compound
1.0
ReactionCatalysis548
ACTIVATION
1.8.1.4
false
1.8.1.4
Dihydrolipoamide + NAD → Hydrogen Ion + Lipoamide + NADH
LEFT_TO_RIGHT
21252287
PubChem-compound
ILE
BioCyc
C21H36N7O16P3S
Coenzyme A
767.11523
Adenosine diphosphate
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
CHEBI:33094
ChEBI
ACETOACETYL-COA
BioCyc
CHEBI:15422
ChEBI
Coenzyme A
1.0
P80404
UniProt
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
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
SMP0000140
SMPDB
439218
PubChem-compound
1.0
ReactionCatalysis562
ACTIVATION
ReactionCatalysis560
ACTIVATION
ReactionCatalysis561
ACTIVATION
1.0
CHEBI:16526
ChEBI
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
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
ReactionCatalysis559
ACTIVATION
ReactionCatalysis557
ACTIVATION
10128135
ChemSpider
ReactionCatalysis558
ACTIVATION
CHEBI:15487
ChEBI
P05166
UniProt
CHEBI:15486
ChEBI
P05165
UniProt
CHEBI:15488
ChEBI
PW000149
PathWhiz
ReactionCatalysis590
ACTIVATION
3-Hydroxy-3-methylglutaryl-CoA
SMILES
NC(=O)CCCCC1CCSS1
5893
PubChem-compound
C00630
KEGG Compound
1420-36-6
CAS
Hydroxymethylglutaryl-CoA lyase, mitochondrial
1.0
ReactionCatalysis582
ACTIVATION
516-05-2
CAS
ReactionCatalysis580
ACTIVATION
C00407
KEGG Compound
ReactionCatalysis581
ACTIVATION
4.0
1.0
VAL
BioCyc
1.0
1.0
280
PubChem-compound
SMILES
CC(C)C[C@H](N)C(O)=O
SMILES
OC(=N)CCCCC(S)CCS
CHEBI:27462
ChEBI
2.3.3.10
false
2.3.3.10
Acetoacetyl-CoA + Acetyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA + Coenzyme A
LEFT_TO_RIGHT
CHEBI:15467
ChEBI
3-ketoacyl-CoA thiolase, 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
1.0
S-(2-Methylbutanoyl)-dihydrolipoamide
CHEBI:15476
ChEBI
1.0
1061
PubChem-compound
CHEBI:15478
ChEBI
CHEBI:15477
ChEBI
CHEBI:15479
ChEBI
HMDB0001967
HMDB
Hydroxymethylglutaryl-CoA lyase, mitochondrial
Isovaleryl-CoA dehydrogenase, mitochondrial
1.0
1.0
HMDB0000883
HMDB
1.0
6244-91-3
CAS
1.0