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C5H11NO2 L-Valine 117.07898 CHEBI:16015 ChEBI 1005 ChemSpider 4-aminobutyrate aminotransferase, mitochondrial 6022 PubChem-compound C23H38N7O17P3S Acetyl-CoA 809.1258 439161 PubChem-compound 937 ChemSpider Acyl-CoA synthetase short-chain family member 3, mitochondrial 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 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 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 false 2-Ketobutyric acid + Coenzyme A + NAD → NADH + Propionyl-CoA LEFT_TO_RIGHT 559142 ChemSpider false Malonyl-CoA → Acetyl-CoA + Carbon dioxide LEFT_TO_RIGHT 6287 PubChem-compound 1.0 ReactionCatalysis972 ACTIVATION PANTETHEINE-P BioCyc ReactionCatalysis970 ACTIVATION HMDB0002170 HMDB P09622 UniProt HCO3 BioCyc CHEBI:15380 ChEBI ReactionCatalysis62989 ACTIVATION 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 1.0 ReactionCatalysis969 ACTIVATION 388468 ChemSpider 5858 ChemSpider ReactionCatalysis961 ACTIVATION 11266 PubChem-compound PW000032 PathWhiz C00109 KEGG Compound 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@@H](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 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial PPI BioCyc Enoyl-CoA hydratase, mitochondrial Malonyl-CoA Decarboxylase Deficiency 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 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 ReactionCatalysis989 ACTIVATION 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 SMP0000502 SMPDB Coenzyme A C21H28N7O14P2 NAD 664.11694 SubPathwayInteraction22714 SubPathway22714Reaction SubPathwayReaction 73173-91-8 CAS C00011 KEGG Compound C00010 KEGG Compound SubPathwayInteraction22713 SubPathwayReaction SubPathway22713Reaction HMDB0000902 HMDB C00013 KEGG Compound P21953 UniProt Succinyl-CoA 4-aminobutyrate aminotransferase, mitochondrial 4-aminobutyrate aminotransferase, mitochondrial H Hydrogen Ion 1.007825 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 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 HMDB0002310 HMDB HMDB0000250 HMDB 3-Hydroxypropionyl-CoA SMILES [O-]P([O-])(=O)OP([O-])([O-])=O HMDB0001341 HMDB 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 HMDB0006806 HMDB 762 ChemSpider 604-98-8 CAS 1.0 HMDB0000148 HMDB 389700 ChemSpider Succinyl-CoA SubPathwayOutput 389702 ChemSpider C13H18N5O8P Propinol adenylate 403.0893 C24H40N7O18P3S 3-Hydroxypropionyl-CoA 839.13635 C05989 KEGG Compound HMDB0006807 HMDB 7732-18-5 CAS C05983 KEGG Compound C05984 KEGG Compound 72-18-4 CAS HMDB0001487 HMDB 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 HMDB0003426 HMDB HMDB0002217 HMDB 56-86-0 CAS 1.0 HMDB0001362 HMDB HMDB0001484 HMDB NAD C10H16N5O13P3 Adenosine triphosphate 506.99576 Phosphoric acid C4H6O3 (S)-Methylmalonic acid semialdehyde 102.03169 SMILES CCC(O)=O Enoyl-CoA hydratase, mitochondrial NAD HMDB0000045 HMDB S-Methylmalonyl-CoA Reaction5 false Oxoglutaric acid + beta-Alanine → L-Glutamic acid + Malonic semialdehyde LEFT_TO_RIGHT 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 HMDB0000056 HMDB 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 HMDB0002111 HMDB SMILES N[C@@H](CCC(O)=O)C(O)=O 6.0 1.0 HMDB0001022 HMDB 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 HMDB0001275 HMDB 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 HMDB0002255 HMDB P11182 UniProt NAD BioCyc CPD-3564 BioCyc Malonyl-CoA semialdehyde 1.0 HMDB0059597 HMDB 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 HMDB0001175 HMDB SMILES [H][H] HMDB0002142 HMDB 487 PubChem-compound 30572 ChemSpider 2.0 S-Methylmalonyl-CoA ReactionCatalysis1205 ACTIVATION Valine, Leucine, and Isoleucine Degradation SubPathway 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 C3H6O2 Propionic acid 74.03678 239 PubChem-compound HMDB0000538 HMDB 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 C4H6O4 Methylmalonic acid 118.02661 CHEBI:16908 ChEBI Citric Acid Cycle SubPathway 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 HMDB0000202 HMDB 600-18-0 CAS Pyruvate Metabolism SubPathway Reaction666 false Coenzyme A + Malonic semialdehyde + NAD → Acetyl-CoA + Carbon dioxide + NADH LEFT_TO_RIGHT Mitochondrion 4.0 HMDB0000208 HMDB 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 HMDB0001423 HMDB 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 HMDB0011111 HMDB 6050 ChemSpider GO:0005759 GENE ONTOLOGY P24752 UniProt Hydrogen GLT BioCyc 107-95-9 CAS SMP0000057 SMPDB 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 HMDB0000595 HMDB 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 SMP0000060 SMPDB C21H29N7O14P2 NADH 665.12476 HMDB0000237 HMDB 473 ChemSpider HMDB0001206 HMDB 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 HMDB0002307 HMDB HMDB0000005 HMDB SMILES CCC(=O)C(O)=O HMDB0000008 HMDB CHEBI:16958 ChEBI 79-09-4 CAS Pantothenate and CoA Biosynthesis SubPathway 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 HMDB0000700 HMDB electron-transfer flavoprotein 1.0 Reduced electron-transfer flavoprotein ReactionCatalysis1058 ACTIVATION 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 SMP0000027 SMPDB 1.0 P80404 UniProt Homo sapiens 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 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 CHEBI:16761 ChEBI CHEBI:27762 ChEBI beta-Alanine Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial 10792 ChemSpider Methylmalonic acid 1.0 SMP0000032 SMPDB Q96PE7 UniProt NADH 157786-88-4 CAS 9606 TAXONOMY P05166 UniProt 1333-74-0 CAS P05165 UniProt CHEBI:17544 ChEBI 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 SMILES C[C@@H](C=O)C(O)=O false 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 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 false 2 Acetyl-CoA ↔ Acetoacetyl-CoA + Coenzyme A REVERSIBLE SubPathwayInteraction185 SubPathwayReaction SubPathway185Reaction CHEBI:15345 ChEBI Propionyl-CoA PW000005 PathWhiz Q9NUB1 UniProt beta-Alanine SubPathwayInput CHEBI:30860 ChEBI C00894 KEGG Compound 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 3-METHYLBENZYLSUCCINYL-COA BioCyc PW000478 PathWhiz HMDB0001967 HMDB CHEBI:15351 ChEBI 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial 68152 PubChem-compound false Adenosine triphosphate + Pantetheine + Propinol adenylate ↔ Adenosine monophosphate + Propionyl-CoA + Pyrophosphate REVERSIBLE HMDB0000883 HMDB 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 false 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 Accumulation 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