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CHEBI:16015 ChEBI C26H44N7O17P3S 2-Methylbutyryl-CoA 851.1727 439161 PubChem-compound C25H42N7O18P3S (S)-3-Hydroxyisobutyryl-CoA 853.152 HMDB02310 HMDB 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 HMDB00148 HMDB 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 2.3.1.168 false 2.3.1.168 Coenzyme A + S-(2-Methylbutanoyl)-dihydrolipoamide → 2-Methylbutyryl-CoA + Dihydrolipoamide LEFT_TO_RIGHT HMDB00595 HMDB C25H40N7O17P3S Methacrylyl-CoA 835.1414 2.0 Isovaleryl-CoA P09622 UniProt HMDB01206 HMDB HCO3 BioCyc CHEBI:15380 ChEBI 5742 ChemSpider C25H40N7O19P3S Succinyl-CoA 867.1312 2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency 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(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 HMDB01423 HMDB 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 HMDB01429 HMDB 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 SMP00137 SMPDB 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 CL:0000000 CELL TYPE ONTOLOGY 3-HYDROXY-ISOBUTYRYL-COA BioCyc P31937 UniProt SMILES OC(=O)CCC(O)=O 1.0 Acetoacetic acid 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 CHEBI:17191 ChEBI HMDB00538 HMDB 784 PubChem-compound 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial 72-18-4 CAS Isobutyryl-CoA 389707 ChemSpider 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 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial 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 BiologicalState3 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 BiologicalState4 BiologicalState2 SMILES CC1=C(CCO[P@](O)(=O)OP(O)(O)=O)SC=[N+]1CC1=CN=C(C)N=C1N 14265-44-2 CAS HMDB00985 HMDB 1.0 99043-16-0 CAS 11953835 PubChem-compound C00091 KEGG Compound 840 ChemSpider 6247-73-0 CAS L-Leucine Methacrylyl-CoA Alpha-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 1.0 NADH BioCyc HMDB00962 HMDB 977 PubChem-compound 1.0 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial CHO3 Hydrogen carbonate 60.99257 30572 ChemSpider Pyrimidine Metabolism SubPathway 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 Propanoate Metabolism SubPathway Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial 1.0 3-hydroxyacyl-CoA dehydrogenase type-2 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 Citric Acid Cycle SubPathway 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 Alpha-ketoisovaleric acid 116.04734 Citric Acid Cycle SubPathway C4H8O3 (S)-3-Hydroxyisobutyric acid 104.04734 O4P Phosphate 94.95342 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 CHEBI:27754 ChEBI SMILES [O-]P([O-])([O-])=O SMILES CC(C)C(=O)C(O)=O CHEBI:30915 ChEBI P30084 UniProt Ketoleucine 4444187 ChemSpider 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial 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 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 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 5957 PubChem-compound 47 PubChem-compound 1.0 Enoyl-CoA hydratase, mitochondrial GO:0005739 GENE ONTOLOGY 1.0 1.0 2.6.1.42 false 2.6.1.42 L-Valine + Oxoglutaric acid → Alpha-ketoisovaleric acid + L-Glutamic 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 389295 ChemSpider P05091 UniProt 72-89-9 CAS 1078 ChemSpider 1.0 HMDB02166 HMDB Carbon dioxide Cell 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 HMDB01041 HMDB 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 HMDB01057 HMDB 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 HMDB01052 HMDB 816-66-0 CAS Medium-chain specific acyl-CoA dehydrogenase, mitochondrial 389035 ChemSpider 7782-44-7 CAS 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 HMDB00172 HMDB 1.0 Hydroxymethylglutaryl-CoA synthase, mitochondrial HMDB02111 HMDB HMDB01022 HMDB 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 HMDB01275 HMDB Q6NVY1 UniProt Acetyl-CoA acetyltransferase, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial Thiamine pyrophosphate HMDB01487 HMDB CHEBI:15344 ChEBI CHEBI:37373 ChEBI CHEBI:15345 ChEBI PW000005 PathWhiz Isobutyryl-CoA dehydrogenase, mitochondrial 389466 ChemSpider 1.0 389465 ChemSpider HMDB01243 HMDB HMDB01484 HMDB 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 3-METHYLBENZYLSUCCINYL-COA BioCyc P54687 UniProt (S)-b-aminoisobutyric acid SubPathwayInput 439371 PubChem-compound CHEBI:15351 ChEBI HMDB01493 HMDB HMDB01011 HMDB 3884-47-7 CAS 4-aminobutyrate aminotransferase, mitochondrial 85-61-0 CAS 1.0 C5H11NO2 L-Valine 117.07898 HMDB00254 HMDB ReactionCatalysis2091 ACTIVATION 388357 ChemSpider 6022 PubChem-compound 7722-84-1 CAS ReactionCatalysis2090 ACTIVATION C23H38N7O17P3S Acetyl-CoA 809.1258 HMDB00019 HMDB CHEBI:17460 ChEBI 937 ChemSpider HMDB00491 HMDB 6.0 PW000061 PathWhiz HMDB01341 HMDB 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 Reaction1010 false (S)-Methylmalonic acid semialdehyde + Oxygen + Water → Hydrogen peroxide + Methylmalonic acid LEFT_TO_RIGHT 440021 PubChem-compound Succinic acid HMDB00023 HMDB HMDB01113 HMDB HMDB01356 HMDB 1.0 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 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 HMDB59597 HMDB Hydroxymethylglutaryl-CoA lyase, mitochondrial Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial S-(2-Methylpropionyl)-dihydrolipoamide-E SMP00046 SMPDB 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 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 Branched-chain-amino-acid aminotransferase, cytosolic 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial Methylglutaconyl-CoA hydratase, mitochondrial Aldehyde dehydrogenase, mitochondrial SMP00057 SMPDB 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 HMDB00695 HMDB 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 HMDB00208 HMDB Reaction274 false (S)-b-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 C21H28N7O14P2 NAD 664.11694 ReactionCatalysis2073 ACTIVATION S-(2-Methylpropionyl)-dihydrolipoamide-E ReactionCatalysis2072 ACTIVATION 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 HMDB00202 HMDB HMDB00687 HMDB 388307 ChemSpider SMP00016 SMPDB 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 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 HYDROGEN-PEROXIDE BioCyc Adenosine diphosphate 2-Methyl-1-hydroxypropyl-ThPP 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 1.0 763 ChemSpider C13H25NO2S2 S-(2-Methylbutanoyl)-dihydrolipoamide 291.13266 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 1.0 1.0 HMDB00883 HMDB 1.0 C01033 KEGG Compound 1.0 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 HMDB01967 HMDB C6H13NO2 L-Leucine 131.09464 C8H17NOS2 Dihydrolipoamide 207.07515 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 2068-83-9 CAS METHYL-MALONYL-COA BioCyc C4H9NO2 (S)-b-aminoisobutyric acid 103.06333 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 P11182 UniProt NAD BioCyc SubPathwayInteraction763 SubPathway763Reaction SubPathwayReaction SubPathwayInteraction767 SubPathway767Reaction SubPathwayReaction 1.0 749 ChemSpider 71-52-3 CAS 3-Hydroxyisovaleryl-CoA C02170 KEGG Compound 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 1.2.4.4 false 1.2.4.4 Alpha-ketoisovaleric acid + Thiamine pyrophosphate → 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 HMDB00902 HMDB C6H10O3 3-Methyl-2-oxovaleric acid 130.06299 439855 PubChem-compound C4H6O4 Methylmalonic acid 118.02661 CHEBI:16908 ChEBI 1.0 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 L-Valine 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 Isobutyryl-CoA dehydrogenase, mitochondrial C03069 KEGG Compound 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 HMDB02054 HMDB 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 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 HMDB03125 HMDB CHEBI:15449 ChEBI 6306 PubChem-compound HMDB06870 HMDB 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 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 HMDB02255 HMDB 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 HMDB06868 HMDB HMDB06867 HMDB 1.0 HMDB06866 HMDB ReactionCatalysis562 ACTIVATION HMDB06865 HMDB ReactionCatalysis560 ACTIVATION ReactionCatalysis561 ACTIVATION 1.0 HMDB06869 HMDB 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 HMDB01157 HMDB 1.0 ReactionCatalysis582 ACTIVATION 516-05-2 CAS ReactionCatalysis580 ACTIVATION C00407 KEGG Compound ReactionCatalysis581 ACTIVATION 4.0 HMDB00060 HMDB 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 HMDB02217 HMDB 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 HMDB01377 HMDB 1061 PubChem-compound CHEBI:15478 ChEBI CHEBI:15477 ChEBI CHEBI:15479 ChEBI Hydroxymethylglutaryl-CoA lyase, mitochondrial HMDB01372 HMDB Isovaleryl-CoA dehydrogenase, mitochondrial 1.0 1.0 HMDB01375 HMDB 1.0 6244-91-3 CAS 1.0