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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 C21H28N7O14P2 NAD 664.11694 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 CHO3 Hydrogen carbonate 60.99257 SMILES [H][H] 1.0 L-serine dehydratase/L- threonine deaminase branched-chain alpha-keto dehydrogenase complex Propionyl-CoA carboxylase Methylmalonyl- CoA epimerase, mitochondrial Methylmalonyl- CoA mutase, mitochondrial Succinyl-CoA 2-Ketobutyric acid L-Threonine L-Threonine 2-Ketobutyric acid NH 3 Coenzyme A NAD Propionyl-CoA NADH Hydrogen carbonate ATP S-Methylmalonyl-CoA ADP H + R-Methylmalonyl-CoA Pyridoxal 5'-phosphate Thiamine pyrophosphate FAD Thiamine pyrophosphate Biotin Adenosylcobalamin Citric Acid Cycle Homocysteine Degradation Methionine Metabolism Extracellular space Intracellular Space Mitochondria Inner mitochondrial membrane Outer mitochondrial membrane
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 C21H28N7O14P2 NAD 664.11694 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 CHO3 Hydrogen carbonate 60.99257 SMILES [H][H] 1.0 Mitochondria SDS BCKDHA PCCA PCCB MCEE MUT Succinyl-CoA 2-Ketobutyric acid L-Threonine L-Threonine 2-Ketobutyric acid Ammonia Coenzyme A NAD Propionyl-CoA NADH Hydrogen carbonate Adenosine triphosphate S- Methylmalonyl- CoA Adenosine diphosphate Hydrogen R- Methylmalonyl- CoA Citric Acid Cycle Homocysteine Degradation Methionine Metabolism