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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 SMP00384 SMPDB 73-32-5 CAS HMDB00148 HMDB C27H42N7O19P3S 3-Methylglutaconyl-CoA 893.1469 C00683 KEGG Compound 48 ChemSpider 46 ChemSpider P45954 UniProt Dihydrolipoamide 1.0 false Isobutyryl-CoA + electron-transfer flavoprotein → Methacrylyl-CoA + Reduced electron-transfer flavoprotein LEFT_TO_RIGHT false 3-Methyl-2-oxovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide LEFT_TO_RIGHT false 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 Methylmalonate Semialdehyde Dehydrogenase Deficiency HMDB01206 HMDB 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 false 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 false 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 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