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C5H11NO2 L-Valine 117.07898 CHEBI:16015 ChEBI 1005 ChemSpider 4-aminobutyrate aminotransferase, mitochondrial 6022 PubChem-compound SMP00027 SMPDB C23H38N7O17P3S Acetyl-CoA 809.1258 439161 PubChem-compound 937 ChemSpider Acyl-CoA synthetase short-chain family member 3, mitochondrial HMDB00250 HMDB HMDB02310 HMDB HMDB01341 HMDB 388353 ChemSpider Reaction1011 false (S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD + Water → Hydrogen carbonate + NADH + Propionyl-CoA LEFT_TO_RIGHT Reaction1254 false Malonyl-CoA semialdehyde ↔ 3-Hydroxypropionyl-CoA REVERSIBLE 439153 PubChem-compound D-METHYL-MALONYL-COA BioCyc ReactionCatalysis703 ACTIVATION Enoyl-CoA hydratase, mitochondrial 50 ChemSpider false 3-Hydroxypropionyl-CoA ↔ Acrylyl-CoA + Water REVERSIBLE false 3-Hydroxypropionyl-CoA + Water ↔ Coenzyme A + Hydroxypropionic acid REVERSIBLE Propinol adenylate CHEBI:16027 ChEBI PW000054 PathWhiz HMDB00148 HMDB HMDB06806 HMDB C00683 KEGG Compound ATP BioCyc Acyl-CoA synthetase short-chain family member 3, mitochondrial PW000051 PathWhiz false Adenosine triphosphate + Hydrogen carbonate + Propionyl-CoA → Adenosine diphosphate + Hydrogen + S-Methylmalonyl-CoA LEFT_TO_RIGHT Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Acetoacetyl-CoA 559142 ChemSpider false Malonyl-CoA → Acetyl-CoA + Carbon dioxide LEFT_TO_RIGHT SMP00032 SMPDB 6287 PubChem-compound 1.0 HMDB00595 HMDB ReactionCatalysis972 ACTIVATION PANTETHEINE-P BioCyc ReactionCatalysis970 ACTIVATION HMDB00237 HMDB HMDB01206 HMDB HCO3 BioCyc CHEBI:15380 ChEBI 5742 ChemSpider NADH C25H40N7O19P3S Succinyl-CoA 867.1312 1.0 Acetyl-CoA C00332 KEGG Compound false Propinol adenylate ↔ Adenosine monophosphate + Propionic acid REVERSIBLE ALPHA-GLUCOSE-16-BISPHOSPHATE BioCyc ReactionCatalysis969 ACTIVATION 388468 ChemSpider 5858 ChemSpider ReactionCatalysis961 ACTIVATION PW000032 PathWhiz HMDB02307 HMDB C00100 KEGG Compound 5776-58-9 CAS C00222 KEGG Compound 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 Adenosine monophosphate + Propionyl-CoA + Pyrophosphate ↔ Adenosine triphosphate + Pantetheine + Propinol adenylate REVERSIBLE false S-Methylmalonyl-CoA → R-Methylmalonyl-CoA LEFT_TO_RIGHT Propionyl-CoA carboxylase alpha chain, mitochondrial false Acrylyl-CoA + electron-transfer flavoprotein ↔ Propionyl-CoA + Reduced electron-transfer flavoprotein REVERSIBLE 439164 PubChem-compound CHEBI:30768 ChEBI PPI BioCyc Enoyl-CoA hydratase, mitochondrial SMP00057 SMPDB Malonyl-CoA semialdehyde 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 L-Valine HMDB01423 HMDB C24H40N7O17P3S Propionyl-CoA 823.1414 1.0 C01213 KEGG Compound Cytosol 388310 ChemSpider 503-66-2 CAS CHEBI:5086 ChEBI 1.0 1.0 Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Reaction2266 false Malonyl-CoA ↔ Malonyl-CoA semialdehyde REVERSIBLE 7664-38-2 CAS 73173-92-9 CAS ReactionCatalysis627 ACTIVATION HMDB00208 HMDB ReactionCatalysis989 ACTIVATION HMDB11111 HMDB C00009 KEGG Compound C00008 KEGG Compound Q9H6R3 UniProt Methylmalonyl-CoA epimerase, mitochondrial C00001 KEGG Compound 1.0 PROPIONYL-COA BioCyc CHEBI:18361 ChEBI NADH C00004 KEGG Compound C00003 KEGG Compound C00002 KEGG Compound Acetyl-CoA acetyltransferase, mitochondrial Reaction1282 false (S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH LEFT_TO_RIGHT Reaction1281 false L-Valine → (S)-Methylmalonic acid semialdehyde LEFT_TO_RIGHT Malonic semialdehyde ReactionCatalysis978 ACTIVATION Reaction1048 false Acetyl-CoA + Adenosine triphosphate + Hydrogen carbonate → Adenosine diphosphate + Malonyl-CoA + Phosphoric acid LEFT_TO_RIGHT ReactionCatalysis976 ACTIVATION 389623 ChemSpider Coenzyme A C21H28N7O14P2 NAD 664.11694 73173-91-8 CAS C00011 KEGG Compound C00010 KEGG Compound C00013 KEGG Compound 4-aminobutyrate aminotransferase, mitochondrial 4-aminobutyrate aminotransferase, mitochondrial HMDB00202 HMDB 388307 ChemSpider 22833590 PubChem-compound 56-65-5 CAS 53-84-9 CAS C00020 KEGG Compound L-Glutamic acid Malonyl-CoA decarboxylase, mitochondrial C00026 KEGG Compound C00025 KEGG Compound 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)CC(O)=O C00024 KEGG Compound CHEBI:26078 ChEBI 317-66-8 CAS Pyruvate Metabolism SubPathway Citric Acid Cycle SubPathway 1.0 Intestinal Microflora SubPathway Methylmalonyl-CoA epimerase, mitochondrial Propionyl-CoA carboxylase alpha chain, mitochondrial Valine, Leucine and Isoleucine Degradation SubPathway Propionyl-CoA carboxylase beta chain, mitochondrial Pantothenate and CoA Biosynthesis SubPathway Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial Acrylyl-CoA 5462303 PubChem-compound 769 PubChem-compound CL:0000000 CELL TYPE ONTOLOGY CHEBI:18276 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 328-50-7 CAS 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 AMP BioCyc 1.0 1.0 C00282 KEGG Compound C3H6O3 Hydroxypropionic acid 90.03169 3-Hydroxypropionyl-CoA SMILES [O-]P([O-])(=O)OP([O-])([O-])=O 1.0 Hydroxypropionic acid Acetyl-CoA acetyltransferase, mitochondrial C01013 KEGG Compound C25H40N7O19P3S R-Methylmalonyl-CoA 867.1312 C11H22N2O4S Pantetheine 278.13004 C00163 KEGG Compound Propionyl-CoA carboxylase beta chain, mitochondrial 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 (S)-Methylmalonic acid semialdehyde 762 ChemSpider 604-98-8 CAS 1.0 HMDB00538 HMDB 389700 ChemSpider Succinyl-CoA SubPathwayOutput 389702 ChemSpider C13H18N5O8P Propinol adenylate 403.0893 C24H40N7O18P3S 3-Hydroxypropionyl-CoA 839.13635 C05989 KEGG Compound 7732-18-5 CAS C05983 KEGG Compound 72-18-4 CAS NAD 61-19-8 CAS