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C5H11NO2 L-Valine 117.07898 CHEBI:16015 ChEBI 1005 ChemSpider 4-aminobutyrate aminotransferase, mitochondrial 6022 PubChem-compound http://identifiers.org/smpdb/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 57 ChemSpider D-METHYL-MALONYL-COA BioCyc ReactionCatalysis703 ACTIVATION Enoyl-CoA hydratase, mitochondrial 50 ChemSpider 4.2.1.17 false 4.2.1.17 3-Hydroxypropionyl-CoA ↔ Acrylyl-CoA + Water REVERSIBLE 3.1.2.4 false 3.1.2.4 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 6.4.1.3 false 6.4.1.3 Adenosine triphosphate + Hydrogen carbonate + Propionyl-CoA → Adenosine diphosphate + Hydrogen + S-Methylmalonyl-CoA LEFT_TO_RIGHT Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Acetoacetyl-CoA 2.3.1.168 false 2.3.1.168 2-Ketobutyric acid + Coenzyme A + NAD → NADH + Propionyl-CoA LEFT_TO_RIGHT 559142 ChemSpider 4.1.1.9 false 4.1.1.9 Malonyl-CoA → Acetyl-CoA + Carbon dioxide LEFT_TO_RIGHT http://identifiers.org/smpdb/SMP00032 SMPDB 6287 PubChem-compound 1.0 HMDB00595 HMDB ReactionCatalysis972 ACTIVATION PANTETHEINE-P BioCyc ReactionCatalysis970 ACTIVATION HMDB00237 HMDB P09622 UniProt HMDB01206 HMDB HCO3 BioCyc CHEBI:15380 ChEBI ReactionCatalysis62989 ACTIVATION 5742 ChemSpider NADH C25H40N7O19P3S Succinyl-CoA 867.1312 1.0 Acetyl-CoA C00332 KEGG Compound 6.2.1.1 false 6.2.1.1 Propinol adenylate ↔ Adenosine monophosphate + Propionic acid REVERSIBLE ALPHA-GLUCOSE-16-BISPHOSPHATE BioCyc HMDB59597 HMDB 1.0 ReactionCatalysis969 ACTIVATION 388468 ChemSpider 5858 ChemSpider ReactionCatalysis961 ACTIVATION 11266 PubChem-compound PW000032 PathWhiz HMDB00005 HMDB HMDB02307 HMDB C00109 KEGG Compound HMDB00008 HMDB C00100 KEGG Compound 5776-58-9 CAS 1010 ChemSpider C00222 KEGG Compound Reaction1264 false R-Methylmalonyl-CoA ↔ Succinyl-CoA REVERSIBLE 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 6.2.1.1 false 6.2.1.1 Adenosine monophosphate + Propionyl-CoA + Pyrophosphate ↔ Adenosine triphosphate + Pantetheine + Propinol adenylate REVERSIBLE 5.1.99.1 false 5.1.99.1 S-Methylmalonyl-CoA → R-Methylmalonyl-CoA LEFT_TO_RIGHT Propionyl-CoA carboxylase alpha chain, mitochondrial 1.3.8.7 false 1.3.8.7 Acrylyl-CoA + electron-transfer flavoprotein ↔ Propionyl-CoA + Reduced electron-transfer flavoprotein REVERSIBLE 439164 PubChem-compound CHEBI:30768 ChEBI 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial PPI BioCyc Enoyl-CoA hydratase, mitochondrial http://identifiers.org/smpdb/SMP00057 SMPDB 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial 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 2-Hydroxybutyric acid 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 2-OXOBUTANOATE BioCyc C00002 KEGG Compound Acetyl-CoA acetyltransferase, mitochondrial Reaction1282 false (S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH LEFT_TO_RIGHT Coenzyme A 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 SubPathwayInteraction22714 SubPathway22714Reaction SubPathwayReaction 73173-91-8 CAS C00011 KEGG Compound C00010 KEGG Compound SubPathwayInteraction22713 SubPathwayReaction SubPathway22713Reaction C00013 KEGG Compound P21953 UniProt Succinyl-CoA 4-aminobutyrate aminotransferase, mitochondrial 4-aminobutyrate aminotransferase, mitochondrial H Hydrogen Ion 1.007825 HMDB00202 HMDB http://identifiers.org/smpdb/SMP00016 SMPDB 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 1.0 Intestinal Microflora SubPathway Methylmalonyl-CoA epimerase, mitochondrial Propionyl-CoA carboxylase alpha chain, mitochondrial Propionyl-CoA carboxylase beta chain, mitochondrial 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 C05984 KEGG Compound 72-18-4 CAS NAD 61-19-8 CAS SMILES NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1 1.0 C00183 KEGG Compound 926-61-4 CAS CHEBI:33404 ChEBI 56-86-0 CAS 1.0 NAD HMDB00883 HMDB C10H16N5O13P3 Adenosine triphosphate 506.99576 Phosphoric acid C4H6O3 (S)-Methylmalonic acid semialdehyde 102.03169 SMILES CCC(O)=O Enoyl-CoA hydratase, mitochondrial NAD S-Methylmalonyl-CoA Reaction5 false Beta-Alanine + Oxoglutaric acid → L-Glutamic acid + Malonic semialdehyde LEFT_TO_RIGHT HMDB01967 HMDB SMILES NCCC(O)=O 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial 962 PubChem-compound 5800 ChemSpider CO2 Carbon dioxide 43.98983 979 ChemSpider C24H38N7O17P3S Acrylyl-CoA 821.1258 C00080 KEGG Compound C25H40N7O19P3S S-Methylmalonyl-CoA 867.1312 Water 2.0 ACRYLYL-COA BioCyc H3O4P Phosphoric acid 97.9769 1.0 C00083 KEGG Compound SMILES OC(=O)CC=O 3-HYDROXY-PROPIONYL-COA BioCyc BiologicalState3 Homo sapiens, Cell, Mitochondrion SMILES N[C@@H](CCC(O)=O)C(O)=O 6.0 1.0 BiologicalState4 Homo sapiens, Cell, Mitochondrial Matrix BiologicalState2 Homo sapiens, Cell, Cytosol 58-64-0 CAS Pantetheine 1.0 99043-16-0 CAS 1.0 2-Ketobutyric acid 2-Ketobutyric acid SubPathwayOutput 845 ChemSpider C00091 KEGG Compound METHYL-MALONYL-COA BioCyc C4H6O3 2-Ketobutyric acid 102.03169 1.0 PROPIONATE BioCyc 644102 PubChem-compound 1.0 444493 PubChem-compound 868 PubChem-compound 6083 PubChem-compound 4575365 ChemSpider C05668 KEGG Compound C00099 KEGG Compound 10663 PubChem-compound 1.0 1.0 ACETYL-COA BioCyc H2O Water 18.010565 1.0 (S)-Methylmalonic acid semialdehyde NADH BioCyc P11182 UniProt NAD BioCyc CPD-3564 BioCyc Malonyl-CoA semialdehyde 1.0 1.0 Propionic acid 749 ChemSpider 71-52-3 CAS 1.0 Acetyl-CoA SubPathwayOutput 61460 ChemSpider C02170 KEGG Compound CHO3 Hydrogen carbonate 60.99257 SMILES [H][H] 487 PubChem-compound 30572 ChemSpider 2.0 Pantothenate and CoA Biosynthesis SubPathway S-Methylmalonyl-CoA ReactionCatalysis1205 ACTIVATION Adenosine monophosphate ReactionCatalysis1204 ACTIVATION Q02252 UniProt SMILES O P12694 UniProt Propionyl-CoA SubPathwayInput Acetyl-CoA C10H14N5O7P Adenosine monophosphate 347.06308 58838673 PubChem-compound Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial 479 PubChem-compound 1.0 HMDB00700 HMDB C3H6O2 Propionic acid 74.03678 239 PubChem-compound 1004 PubChem-compound L-lactate dehydrogenase A-like 6B Propionic acid SubPathwayOutput 1.0 Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial CHEBI:27821 ChEBI 6816 PubChem-compound 51 PubChem-compound 1.0 SMILES OC([O-])=O CHEBI:15846 ChEBI 1.0 ADP BioCyc 58 PubChem-compound C3H7NO2 Beta-Alanine 89.047676 Q9BYZ2 UniProt ReactionCatalysis178 ACTIVATION Malonyl-CoA 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial C4H8O3 2-Hydroxybutyric acid 104.04734 C24H38N7O18P3S Malonyl-CoA semialdehyde 837.12067 1038 PubChem-compound 1032 PubChem-compound GO:0005829 GENE ONTOLOGY 1.0 SMILES OCCC(O)=O 274 ChemSpider 1.0 C5H6O5 Oxoglutaric acid 146.02153 HMDB00902 HMDB C4H6O4 Methylmalonic acid 118.02661 CHEBI:16908 ChEBI 1.0 Pyrophosphate ReactionCatalysis161 ACTIVATION SMILES CC(C)(CO)C(O)C(=O)NCCC(=O)NCCS 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 600-18-0 CAS Reaction666 false Coenzyme A + Malonic semialdehyde + NAD → Acetyl-CoA + Carbon dioxide + NADH LEFT_TO_RIGHT Mitochondrion 4.0 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)CC=O Carbon dioxide 1.0 440865 PubChem-compound 1.0 440863 PubChem-compound 58-68-4 CAS SMILES CC(C(O)=O)C(O)=O 4.0 SMILES OC(=O)CCC(=O)C(O)=O 124-38-9 CAS CHEBI:30915 ChEBI 1.0 1.0 ReactionCatalysis6 ACTIVATION P30084 UniProt CHEBI:15531 ChEBI 1.0 Q13085 UniProt 1.0 Malonic semialdehyde 6050 ChemSpider GO:0005759 GENE ONTOLOGY P24752 UniProt Hydrogen GLT BioCyc 107-95-9 CAS Hydrogen carbonate H2 Hydrogen 2.01565 Oxoglutaric acid SMILES O=C=O 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=C(N)N=CN=C12)C(O)C(=O)NCCC(=O)NCCSC(=O)C=C Hydrogen Ion C06002 KEGG Compound 1.0 SMILES OP(O)(O)=O O7P2 Pyrophosphate 173.91193 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 234 ChemSpider 496-65-1 CAS C21H29N7O14P2 NADH 665.12476 HMDB02170 HMDB 473 ChemSpider 5957 PubChem-compound CHEBI:1148 ChEBI 1.0 3-Hydroxypropionyl-CoA CHEBI:15513 ChEBI L-lactate dehydrogenase A-like 6B 1.0 Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial 1.0 Cysteine and Methionine Metabolism SubPathway O95822 UniProt Glycine, Serine, and Threonine Metabolism SubPathway 440753 PubChem-compound 1.0 466 ChemSpider Adenosine triphosphate GO:0005739 GENE ONTOLOGY 1.0 C10H15N5O10P2 Adenosine diphosphate 427.02942 SMILES CCC(O)C(O)=O SMILES CCC(=O)C(O)=O CHEBI:16958 ChEBI 79-09-4 CAS Acetyl-CoA carboxylase 1 C00831 KEGG Compound CHEBI:16414 ChEBI 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 electron-transfer flavoprotein R-Methylmalonyl-CoA 5682 ChemSpider Reduced electron-transfer flavoprotein CHEBI:30831 ChEBI CHEBI:1146900 ChEBI 17216177 ChemSpider 1.0 Mitochondrial Matrix 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial 21252287 PubChem-compound 14000-31-8 CAS Hydroxypropionic acid SubPathwayOutput CO-A BioCyc B-ALANINE BioCyc 72-89-9 CAS Carbon dioxide 600-15-7 CAS C21H36N7O16P3S Coenzyme A 767.11523 Adenosine diphosphate electron-transfer flavoprotein 1.0 Reduced electron-transfer flavoprotein ReactionCatalysis1058 ACTIVATION Cell 10213 ChemSpider ReactionCatalysis1057 ACTIVATION Acetyl-CoA carboxylase 1 ACETOACETYL-COA BioCyc 1.0 Propionyl-CoA carboxylase CHEBI:15422 ChEBI CHEBI:16753 ChEBI Malonyl-CoA decarboxylase, mitochondrial Coenzyme A 6557 ChemSpider Acetyl-coenzyme A synthetase 2-like, mitochondrial 1.0 P80404 UniProt 1.0 524-14-1 CAS 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 Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial CHEBI:17960 ChEBI HMDB02255 HMDB 1.0 C3H4O3 Malonic semialdehyde 88.016045 1.0 439214 PubChem-compound 1.0 CHEBI:15539 ChEBI C5H9NO4 L-Glutamic acid 147.05316 Water 33032 PubChem-compound Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:16526 ChEBI 1.0 P11310 UniProt HMDB01175 HMDB HMDB02142 HMDB CHEBI:16761 ChEBI CHEBI:27762 ChEBI Beta-Alanine Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial 10792 ChemSpider Methylmalonic acid 1.0 Q96PE7 UniProt NADH 157786-88-4 CAS 9606 TAXONOMY HMDB00056 HMDB P05166 UniProt 1333-74-0 CAS P05165 UniProt CHEBI:17544 ChEBI PW000149 PathWhiz 2-KETOGLUTARATE BioCyc 392413 ChemSpider 6244-93-5 CAS 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=C(N)N=CN=C12)C(O)C(=O)NCCC(=O)NCCSC(=O)CCO 5893 PubChem-compound HMDB02111 HMDB HMDB01022 HMDB http://identifiers.org/smpdb/SMP00060 SMPDB SMILES C[C@@H](C=O)C(O)=O 1.1.1.27 false 1.1.1.27 2-Hydroxybutyric acid + NAD ↔ 2-Ketobutyric acid + Hydrogen Ion + NADH REVERSIBLE 13628334 ChemSpider 1420-36-6 CAS branched-chain alpha-keto dehydrogenase complex CHEBI:15377 ChEBI Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial C24H38N7O19P3S Malonyl-CoA 853.1156 CHEBI:15378 ChEBI SMILES [H+] 516-05-2 CAS 4.0 4.0 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 R-Methylmalonyl-CoA 1.0 VAL BioCyc HMDB01275 HMDB Q6NVY1 UniProt Acetyl-coenzyme A synthetase 2-like, mitochondrial 280 PubChem-compound 439340 PubChem-compound Acetyl-CoA acetyltransferase, mitochondrial SubPathwayInteraction183 SubPathway183Reaction SubPathwayReaction Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial SubPathwayInteraction181 SubPathwayReaction SubPathway181Reaction SubPathwayInteraction182 SubPathwayReaction SubPathway182Reaction SubPathwayInteraction187 SubPathway187Reaction SubPathwayReaction 2.3.1.9 false 2.3.1.9 2 Acetyl-CoA ↔ Acetoacetyl-CoA + Coenzyme A REVERSIBLE SubPathwayInteraction185 SubPathwayReaction SubPathway185Reaction HMDB01487 HMDB CHEBI:15345 ChEBI HMDB02217 HMDB Pyruvate Metabolism SubPathway Propionyl-CoA PW000005 PathWhiz Q9NUB1 UniProt HMDB03426 HMDB Valine, Leucine and Isoleucine Degradation SubPathway Beta-Alanine SubPathwayInput CHEBI:30860 ChEBI HMDB01362 HMDB C00894 KEGG Compound HMDB01484 HMDB 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 1.0 1.0 Propanoate Metabolism HMDB06807 HMDB 3-METHYLBENZYLSUCCINYL-COA BioCyc Citric Acid Cycle SubPathway HMDB00045 HMDB CHEBI:15351 ChEBI 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial 68152 PubChem-compound 6.2.1.1 false 6.2.1.1 Adenosine triphosphate + Pantetheine + Propinol adenylate ↔ Adenosine monophosphate + Propionyl-CoA + Pyrophosphate REVERSIBLE SMILES CCC(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2N 1.0 CHEBI:62415 ChEBI 85-61-0 CAS 1.0 MALONYL-COA BioCyc 6.2.1.1 false 6.2.1.1 Propinol adenylate ↔ Adenosine monophosphate + Propionic acid REVERSIBLE Methylmalonyl- CoA epimerase, mitochondrial Methylmalonyl- CoA mutase, mitochondrial Malonyl-CoA decarboxylase, mitochondrial Acetyl-CoA carboxylase 1 Acetyl-CoA acetyltransferase, mitochondrial Methylmalonate- semialdehyde dehydrogenase [acylating], mitochondrial 4-aminobutyrate aminotransferase, mitochondrial Enoyl-CoA hydratase, mitochondrial 3- hydroxyisobutyryl- CoA hydrolase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Acetyl-coenzyme A synthetase 2-like, mitochondrial Acyl-CoA synthetase short-chain family member 3, mitochondrial Acyl-CoA synthetase short-chain family member 3, mitochondrial Acetyl-coenzyme A synthetase 2-like, mitochondrial Methylmalonate- semialdehyde dehydrogenase [acylating], mitochondrial Propionyl-CoA carboxylase alpha chain, mitochondrial Propionyl-CoA carboxylase beta chain, mitochondrial branched-chain alpha-keto dehydrogenase complex L-lactate dehydrogenase A-like 6B Acetyl-CoA Succinyl-CoA Propionic acid Hydroxypropionic acid Propionyl-CoA Beta-Alanine 2-Ketobutyric acid Malonyl-CoA semialdehyde 3-Hydroxypropionyl-CoA S-Methylmalonyl-CoA R-Methylmalonyl-CoA L-Valine (S)- Methylmalonic acid semialdehyde Methylmalonic acid Malonyl-CoA CO 2 ATP Hydrogen carbonate P i ADP Acetoacetyl-CoA CoA Malonic semialdehyde CoA NAD CO 2 NADH Oxoglutaric acid L-Glutamic acid Acrylyl-CoA H 2 O H 2 O CoA AMP PP i Propinol adenylate ATP Pantetheine ATP Pantetheine AMP PP i AMP AMP H 2 O NAD CoA NADH Hydrogen carbonate Hydrogen carbonate ATP ADP H + CoA NAD NADH 2-Hydroxybutyric acid NAD NADH H + Adenosylcobalamin Biotin Manganese Pyridoxal 5'-phosphate FAD Biotin FAD Thiamine pyrophosphate electron- transfer flavoprotein Reduced electron- transfer flavoprotein Pyruvate Metabolism Citric Acid Cycle Valine, Leucine and Isoleucine Degradation Pantothenate and CoA Biosynthesis Cysteine and Methionine Metabolism Glycine, Serine, and Threonine Metabolism Mitochondria Mitochondrial Matrix Intracellular Space Extracellular Space
C5H11NO2 L-Valine 117.07898 CHEBI:16015 ChEBI 1005 ChemSpider 4-aminobutyrate aminotransferase, mitochondrial 6022 PubChem-compound http://identifiers.org/smpdb/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 57 ChemSpider D-METHYL-MALONYL-COA BioCyc ReactionCatalysis703 ACTIVATION Enoyl-CoA hydratase, mitochondrial 50 ChemSpider 4.2.1.17 false 4.2.1.17 3-Hydroxypropionyl-CoA ↔ Acrylyl-CoA + Water REVERSIBLE 3.1.2.4 false 3.1.2.4 3-Hydroxypropionyl-CoA + Water ↔ Coenzyme A + Hydroxypropionic acid REVERSIBLE Propinol adenylate CHEBI:16027 ChEBI PW000054 PathWhiz HMDB00148 HMDB HMDB06806 HMDB