6022
PubChem-compound
439161
PubChem-compound
CHEBI:16134
ChEBI
1.0
ReactionCatalysis1203
ACTIVATION
S-Methylmalonyl-CoA
Methionine Metabolism
SubPathway
ReactionCatalysis1205
ACTIVATION
ReactionCatalysis1204
ACTIVATION
ReactionCatalysis1207
ACTIVATION
439153
PubChem-compound
57
ChemSpider
Transport118
false
2-Ketobutyric acid (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrion
LEFT_TO_RIGHT
Transport117
false
L-Threonine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytosol
LEFT_TO_RIGHT
H3N
Ammonia
17.026548
P12694
UniProt
1.0
D-METHYL-MALONYL-COA
BioCyc
58838673
PubChem-compound
2-Ketobutyric acid
PW000056
PathWhiz
HMDB0000538
HMDB
C00683
KEGG Compound
ATP
BioCyc
6.4.1.3
false
6.4.1.3
Adenosine triphosphate + Hydrogen carbonate + Propionyl-CoA → Adenosine diphosphate + Hydrogen + S-Methylmalonyl-CoA
LEFT_TO_RIGHT
Reaction1482
false
L-Threonine → 2-Ketobutyric acid + Ammonia
LEFT_TO_RIGHT
2.3.1.168
false
2.3.1.168
2-Ketobutyric acid + Coenzyme A + NAD → NADH + Propionyl-CoA
LEFT_TO_RIGHT
6816
PubChem-compound
2-Ketobutyric acid
SubPathwayOutput
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
SMILES
OC(O)=O
CHEBI:15846
ChEBI
ADP
BioCyc
58
PubChem-compound
5.4.99.2
false
5.4.99.2
R-Methylmalonyl-CoA → Succinyl-CoA
LEFT_TO_RIGHT
PW000166
PathWhiz
6288
PubChem-compound
branched-chain alpha-keto dehydrogenase complex
P09622
UniProt
HCO3
BioCyc
THR
BioCyc
CHEBI:15380
ChEBI
5742
ChemSpider
Methylmalonyl-CoA mutase, mitochondrial
C25H40N7O19P3S
Succinyl-CoA
867.1312
1.0
GO:0005829
GENE ONTOLOGY
ALPHA-GLUCOSE-16-BISPHOSPHATE
BioCyc
1.0
1.0
CHEBI:16908
ChEBI
Citric Acid Cycle
SubPathway
Ammonia
1.0
C00109
KEGG Compound
C00100
KEGG Compound
P22033
UniProt
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
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
1.0
Adenosine triphosphate
Reaction1263
false
S-Methylmalonyl-CoA → R-Methylmalonyl-CoA
LEFT_TO_RIGHT
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
439164
PubChem-compound
600-18-0
CAS
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Mitochondrion
Propionyl-CoA carboxylase
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(O)C(=O)NCCC(=O)NCCSC(=O)CCC(O)=O
C24H40N7O17P3S
Propionyl-CoA
823.1414
CHEBI:16857
ChEBI
C01213
KEGG Compound
Cytosol
388310
ChemSpider
217
ChemSpider
58-68-4
CAS
72-19-5
CAS
HMDB0001423
HMDB
Propionyl-CoA
73173-92-9
CAS
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
C00008
KEGG Compound
PROPIONYL-COA
BioCyc
C00004
KEGG Compound
2-OXOBUTANOATE
BioCyc
C00003
KEGG Compound
C00002
KEGG Compound
6051
ChemSpider
L-serine dehydratase/L-threonine deaminase
7664-41-7
CAS
SMP0000057
SMPDB
H2
Hydrogen
2.01565
Succinyl-CoA
SubPathwayInput
C21H27N7O14P2
NAD
663.10913
73173-91-8
CAS
C00010
KEGG Compound
1.0
HMDB0000902
HMDB
222
PubChem-compound
SMILES
C[C@@H](O)[C@H](N)C(O)=O
C00014
KEGG Compound
Extracellular Space
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
HMDB0000595
HMDB
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
P21953
UniProt
C21H29N7O14P2
NADH
665.12476
Succinyl-CoA
5957
PubChem-compound
1.0
1.0
388307
ChemSpider
22833590
PubChem-compound
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
56-65-5
CAS
53-84-9
CAS
Hydrogen
L-Threonine
317-66-8
CAS
1.0
L-Threonine
GO:0005739
GENE ONTOLOGY
1.0
GO:0005615
GENE ONTOLOGY
C10H15N5O10P2
Adenosine diphosphate
427.02942
Methylmalonyl-CoA mutase, mitochondrial
HMDB0000005
HMDB
SMILES
CCC(=O)C(O)=O
Propionyl-CoA carboxylase alpha chain, mitochondrial
Propionyl-CoA carboxylase beta chain, mitochondrial
1.0
Propionyl-CoA carboxylase alpha chain, mitochondrial
1.0
SubPathwayInteraction316
SubPathway316Reaction
SubPathwayReaction
769
PubChem-compound
SubPathwayInteraction317
SubPathwayReaction
SubPathway317Reaction
S-Methylmalonyl-CoA
SubPathwayInteraction315
SubPathwayReaction
SubPathway315Reaction
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
R-Methylmalonyl-CoA
CHEBI:18276
ChEBI
5682
ChemSpider
CHEBI:30831
ChEBI
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
CHEBI:1146900
ChEBI
1.0
C00282
KEGG Compound
17216177
ChemSpider
Adenosine diphosphate
HMDB0002310
HMDB
21252287
PubChem-compound
HMDB0001341
HMDB
CO-A
BioCyc
C25H40N7O19P3S
R-Methylmalonyl-CoA
867.1312
C00288
KEGG Compound
L-serine dehydratase/L-threonine deaminase
SMILES
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
C21H36N7O16P3S
Coenzyme A
767.11523
762
ChemSpider
604-98-8
CAS
CHEBI:15422
ChEBI
Coenzyme A
6557
ChemSpider
Homo sapiens
Hydrogen carbonate
2.0
388299
ChemSpider
Threonine and 2-Oxobutanoate Degradation
NAD
HMDB0001487
HMDB
AMMONIA
BioCyc
SMILES
N
CHEBI:15539
ChEBI
HMDB0001362
HMDB
Homocysteine Degradation
SubPathway
C10H16N5O13P3
Adenosine triphosphate
506.99576
Dihydrolipoyl dehydrogenase, mitochondrial
Propionyl-CoA carboxylase beta chain, mitochondrial
C00188
KEGG Compound
1.0
CHEBI:16761
ChEBI
HMDB0000167
HMDB
1.0
P20132
UniProt
1.0
NADH
SMP0000033
SMPDB
9606
TAXONOMY
C4H9NO3
L-Threonine
119.05824
P05166
UniProt
1333-74-0
CAS
P05165
UniProt
CHEBI:17544
ChEBI
2.0
PW000027
PathWhiz
5800
ChemSpider
5893
PubChem-compound
C25H40N7O19P3S
S-Methylmalonyl-CoA
867.1312
HMDB0000051
HMDB
13628334
ChemSpider
HMDB0001022
HMDB
58-64-0
CAS
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
L-serine dehydratase/L-threonine deaminase
C00091
KEGG Compound
Methylmalonyl-CoA mutase, mitochondrial
METHYL-MALONYL-COA
BioCyc
C4H6O3
2-Ketobutyric acid
102.03169
SMP0000455
SMPDB
SMP0000452
SMPDB
HMDB0001275
HMDB
1.0
PW000005
PathWhiz
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
R-Methylmalonyl-CoA
1.0
Dihydrolipoyl dehydrogenase, mitochondrial
NADH
BioCyc
3-METHYLBENZYLSUCCINYL-COA
BioCyc
HMDB0002255
HMDB
P11182
UniProt
NAD
BioCyc
749
ChemSpider
71-52-3
CAS
1.0
85-61-0
CAS
1.0
CH2O3
Hydrogen carbonate
62.000393
SMILES
[H][H]
1.0