Toggle navigation
SMPDB
Browse SMPDB
Pathways
Table of Primary Pathways
Compounds
Proteins
Search
TextQuery Search Instructions
SMP-MAP Advanced Search
ChemQuery Structure Search
Molecular Weight Search
Sequence Search
About
About SMPDB
Release Notes
Citing SMPDB
Statistics
Other Databases
Pathway Legend
Pathway Category Definitions
PathWhiz
Downloads
Contact Us
Quantitative metabolomics services for biomarker discovery and validation.
Specializing in ready to use metabolomics kits.
Your source for quantitative metabolomics technologies and bioinformatics.
Loading Pathway...
Error: Pathway image not found.
2-oxoisovalerate
dehydrogenase
subunit beta,
mitochondrial
2-oxoisovalerate
dehydrogenase
subunit alpha,
mitochondrial
Dihydrolipoyl
dehydrogenase,
mitochondrial
Hydroxymethylglutaryl-
CoA lyase,
mitochondrial
2-oxoisovalerate
dehydrogenase
subunit beta,
mitochondrial
2-oxoisovalerate
dehydrogenase
subunit beta,
mitochondrial
Branched-chain-
amino-acid
aminotransferase,
cytosolic
Branched-chain-
amino-acid
aminotransferase,
cytosolic
Branched-chain-
amino-acid
aminotransferase,
cytosolic
2-oxoisovalerate
dehydrogenase
Lipoamide
acyltransferase
component of
branched-chain
alpha-keto acid
dehydrogenase
complex,
mitochondrial
Isovaleryl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific acyl-CoA
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
Methylcrotonoyl-
CoA carboxylase
subunit alpha,
mitochondrial
Methylcrotonoyl-
CoA carboxylase
beta chain,
mitochondrial
Dihydrolipoyl
dehydrogenase,
mitochondrial
Lipoamide
acyltransferase
component of
branched-chain
alpha-keto acid
dehydrogenase
complex,
mitochondrial
2-oxoisovalerate
dehydrogenase
2-oxoisovalerate
dehydrogenase
Lipoamide
acyltransferase
component of
branched-chain
alpha-keto acid
dehydrogenase
complex,
mitochondrial
Short-chain
specific acyl-CoA
dehydrogenase,
mitochondrial
Short/branched
chain specific
acyl-CoA
dehydrogenase,
mitochondrial
Isobutyryl-CoA
dehydrogenase,
mitochondrial
Medium-chain
specific acyl-CoA
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
3-
hydroxyisobutyryl-
CoA hydrolase,
mitochondrial
3-
hydroxyisobutyrate
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
4-aminobutyrate
aminotransferase,
mitochondrial
Medium-chain
specific acyl-CoA
dehydrogenase,
mitochondrial
Short-chain
specific acyl-CoA
dehydrogenase,
mitochondrial
Short/branched
chain specific
acyl-CoA
dehydrogenase,
mitochondrial
Enoyl-CoA
hydratase,
mitochondrial
3-hydroxyacyl-CoA
dehydrogenase
type-2
3-ketoacyl-CoA
thiolase,
mitochondrial
Methylmalonate-
semialdehyde
dehydrogenase
[acylating],
mitochondrial
Propionyl-CoA
carboxylase alpha
chain,
mitochondrial
Propionyl-CoA
carboxylase beta
chain,
mitochondrial
Methylmalonyl-CoA
epimerase,
mitochondrial
Methylmalonyl-CoA
mutase,
mitochondrial
Aldehyde oxidase
Aldehyde
dehydrogenase,
mitochondrial
Methylglutaconyl-
CoA hydratase,
mitochondrial
Hydroxymethylglutaryl-
CoA synthase,
mitochondrial
Acetyl-CoA
acetyltransferase,
mitochondrial
Succinyl-CoA:3-
ketoacid coenzyme
A transferase 1,
mitochondrial
2-oxoisovalerate
dehydrogenase
subunit alpha,
mitochondrial
2-oxoisovalerate
dehydrogenase
subunit alpha,
mitochondrial
(S)-beta-
Aminoisobutyric
acid
Acetyl-CoA
Propionyl-CoA
Acetyl-CoA
Succinyl-CoA
3-Methyl-1-
hydroxybutyl-
ThPP
Lipoamide
S-(3-
Methylbutanoyl)-
dihydrolipoamide-
E
Dihydrolipoamide
NAD
NADH
H
+
3-Hydroxy-3-
methylglutaryl-
CoA
Acetoacetic acid
2-Methyl-1-
hydroxypropyl-
ThPP
S-(2-
Methylpropionyl)-
dihydrolipoamide-
E
Lipoamide
2-Methyl-1-
hydroxypropyl-
ThPP
S-(2-
Methylbutanoyl)-
dihydrolipoamide
Thiamine
pyrophosphate
L-Valine
Oxoglutaric acid
α-Ketoisovaleric
acid
L-Glutamic acid
L-Leucine
Oxoglutaric acid
Ketoleucine
L-Glutamic acid
L-Isoleucine
Oxoglutaric acid
3-Methyl-2-
oxovaleric acid
L-Glutamic acid
Thiamine
pyrophosphate
CO
2
CoA
Isovaleryl-CoA
3-
Methylcrotonyl-
CoA
3-
Hydroxyisovaleryl-
CoA
H
2
O
ATP
HCO
3
-
3-
Methylglutaconyl-
CoA
ADP
Dihydrolipoamide
NAD
NADH
H
+
CoA
Isobutyryl-CoA
CO
2
CO
2
CoA
2- Methylbutyryl-
CoA
Methacrylyl-CoA
H
2
O
(S)-3-
Hydroxyisobutyryl-
CoA
H
2
O
(S)-3-
Hydroxyisobutyric
acid
CoA
NAD
(S)-Methylmalonic
acid semialdehyde
NADH
H
+
Oxoglutaric acid
L-Glutamic acid
Tiglyl-CoA
H
2
O
2-Methyl-3-
hydroxybutyryl-
CoA
NAD
2-
Methylacetoacetyl-
CoA
NADH
CoA
H
2
O
NAD
CoA