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SubPathwayInteraction398 SubPathway398Reaction SubPathwayReaction SubPathwayInteraction399 SubPathwayReaction SubPathway399Reaction C23H38N7O17P3S Acetyl-CoA 809.1258 C00318 KEGG Compound 937 ChemSpider HMDB00250 HMDB 439153 PubChem-compound L-Carnitine Trifunctional enzyme subunit alpha, mitochondrial Enoyl-CoA hydratase, mitochondrial 1.0 Trifunctional enzyme subunit beta, mitochondrial C33H58N7O17P3S Lauroyl-CoA 949.2823 HMDB01114 HMDB CHEBI:16027 ChEBI 53481913 PubChem-compound 38795-33-4 CAS 5281 PubChem-compound C01530 KEGG Compound ATP BioCyc PW000173 PathWhiz 388366 ChemSpider PW000172 PathWhiz Mitochondrial carnitine/acylcarnitine carrier protein 559142 ChemSpider Transport129 false Stearoylcarnitine (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT 1.0 Transport128 false L-Carnitine (→) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Cytoplasm LEFT_TO_RIGHT 1.0 PW000167 PathWhiz Tetradecanoyl-CoA HMDB01206 HMDB (S)-3-Hydroxytetradecanoyl-CoA 5742 ChemSpider 1.0 11966197 PubChem-compound 1.0 Trifunctional enzyme, mitochondrial HMDB59597 HMDB CHEBI:73074 ChEBI L-Carnitine 5858 ChemSpider Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids HMDB01338 HMDB Stearoyl-CoA Lauroyl-CoA 1.0 Stearic acid 1010 ChemSpider Enoyl-CoA hydratase, mitochondrial 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 PPI BioCyc SMP00057 SMPDB SMP00054 SMPDB HMDB01423 HMDB trans-Octadec-2-enoyl-CoA Trifunctional enzyme, mitochondrial CHEBI:5086 ChEBI LAUROYLCOA-CPD BioCyc Lauroyl-CoA SubPathwayInput 1.0 Long-chain-fatty-acid--CoA ligase 1 Stearoylcarnitine C39H70N7O17P3S Stearoyl-CoA 1033.3762 Fatty Acid Biosynthesis SubPathway Fatty Acid Elongation In Mitochondria SubPathway 53477850 PubChem-compound 3-ketoacyl-CoA thiolase, mitochondrial C00001 KEGG Compound 1.0 CHEBI:18361 ChEBI Long-chain specific acyl-CoA dehydrogenase, mitochondrial NADH C00004 KEGG Compound C00003 KEGG Compound C00002 KEGG Compound P42765 UniProt Coenzyme A Mitochondrial Inner Membrane L-Carnitine HMDB13640 HMDB 3130-72-1 CAS HMDB01521 HMDB 389500 ChemSpider HMDB03945 HMDB C21H28N7O14P2 NAD 664.11694 HMDB03946 HMDB C00010 KEGG Compound C00013 KEGG Compound trans-Octadec-2-enoyl-CoA SMP00481 SMPDB SMP00482 SMPDB Stearoylcarnitine P28330 UniProt H Hydrogen Ion 1.007825 CARNITINE BioCyc 56-65-5 CAS C00020 KEGG Compound 53-84-9 CAS Carnitine O-palmitoyltransferase 1 C00024 KEGG Compound Trifunctional enzyme subunit beta, mitochondrial SMILES CCCCCCCCCCCCCCCC(O)CC(=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=C1C=CN=C2N Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial Adenosine monophosphate 4460-95-1 CAS Stearic acid SubPathwayOutput Stearoylcarnitine 3-Oxooctadecanoyl-CoA BiologicalState22 3-Oxohexadecanoyl-CoA CL:0000000 CELL TYPE ONTOLOGY C00154 KEGG Compound C37H66N7O18P3S (S)-3-Hydroxyhexadecanoyl-CoA 1021.3398 C35H62N7O18P3S (S)-3-Hydroxytetradecanoyl-CoA 993.3085 Carnitine O-palmitoyltransferase 1, liver isoform C35H60N7O18P3S 3-Oxotetradecanoyl-CoA 991.29285 Carnitine O-palmitoyltransferase 2, mitochondrial Long-chain-fatty-acid--CoA ligase 1 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 AMP BioCyc 35106-50-4 CAS STEARIC_ACID BioCyc Tetradecanoyl-CoA SMILES [O-]P([O-])(=O)OP([O-])([O-])=O HMDB03934 HMDB HMDB03935 HMDB 1.0 HMDB03932 HMDB C25H49NO4 Stearoylcarnitine 427.36615 trans-Octadec-2-enoyl-CoA SMILES CCCCCCCCCCCCCCCC(=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 C02593 KEGG Compound C37H64N7O18P3S 3-Oxohexadecanoyl-CoA 1019.32416 1.0 HMDB00538 HMDB 7732-18-5 CAS 4.0 Acetyl-CoA 3-OXOPALMITOYL-COA BioCyc 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 3-Oxooctadecanoyl-CoA O43772 UniProt 1.0 BiologicalState17 1.0 C10H16N5O13P3 Adenosine triphosphate 506.99576 SMP00456 SMPDB Stearoyl-CoA 165436 PubChem-compound NAD SMILES C[N+](C)(C)C[C@H](O)CC([O-])=O 1.0 C18H36O2 Stearic acid 284.27155 Adenosine triphosphate 22833663 PubChem-compound 962 PubChem-compound C00080 KEGG Compound P50416 UniProt Water C39H66N7O18P3S 3-Oxooctadecanoyl-CoA 1045.3398 Hydrogen Ion Enoyl-CoA hydratase, mitochondrial 1.0 HMDB00848 HMDB BiologicalState3 BiologicalState4 57-11-4 CAS 6.0 1.0 1.0 6426855 PubChem-compound 6.0 1.0 1.0 644102 PubChem-compound 1.0 444493 PubChem-compound SubPathwayInteraction416 SubPathwayReaction SubPathway416Reaction 6083 PubChem-compound Palmityl-CoA SMILES CCCCCCCCCCCCC\C=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 HMDB06498 HMDB ACETYL-COA BioCyc SMILES CCCCCCCCCCC\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=C1N=CN=C2N H2O Water 18.010565 Carnitine O-palmitoyltransferase 2, mitochondrial P55084 UniProt HMDB00827 HMDB NADH BioCyc NAD BioCyc SubPathwayInteraction400 SubPathway400Reaction SubPathwayReaction 1976-27-8 CAS Acetyl-CoA SubPathwayInput Trifunctional enzyme subunit alpha, mitochondrial 1.0 SMILES CCCCCCCCCCCCCC(=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 65113 PubChem-compound (S)-3-Hydroxytetradecanoyl-CoA 1.0 TransportCatalysis99 ACTIVATION ReactionCatalysis1324 ACTIVATION ReactionCatalysis1323 ACTIVATION 3-Oxotetradecanoyl-CoA ReactionCatalysis1328 ACTIVATION ReactionCatalysis1327 ACTIVATION Coenzyme A 10140190 ChemSpider SMILES O C16216 KEGG Compound Palmityl-CoA C16218 KEGG Compound C10H14N5O7P Adenosine monophosphate 347.06308 ReactionCatalysis1320 ACTIVATION ReactionCatalysis1322 ACTIVATION ReactionCatalysis1321 ACTIVATION P40939 UniProt 4.2.1.17 false 4.2.1.17 (S)-3-Hydroxyhexadecanoyl-CoA ↔ (2E)-Hexadecenoyl-CoA + Water REVERSIBLE 2.3.1.16 false 2.3.1.16 Acetyl-CoA + Tetradecanoyl-CoA ↔ 3-Oxohexadecanoyl-CoA + Coenzyme A REVERSIBLE 1.1.1.35 false 1.1.1.35 (S)-3-Hydroxyhexadecanoyl-CoA + NAD ↔ 3-Oxohexadecanoyl-CoA + Hydrogen Ion + NADH REVERSIBLE Carnitine O-palmitoyltransferase 1, liver isoform 6816 PubChem-compound 4.2.1.17 false 4.2.1.17 (S)-3-Hydroxytetradecanoyl-CoA ↔ (2E)-Tetradecenoyl-CoA + Water REVERSIBLE CHEBI:15846 ChEBI (S)-3-Hydroxyhexadecanoyl-CoA 2.3.1.16 false 2.3.1.16 Acetyl-CoA + Lauroyl-CoA → 3-Oxotetradecanoyl-CoA + Coenzyme A LEFT_TO_RIGHT C7H15NO3 L-Carnitine 161.1052 1.1.1.35 false 1.1.1.35 (S)-3-Hydroxytetradecanoyl-CoA + NAD ↔ 3-Oxotetradecanoyl-CoA + Hydrogen Ion + NADH REVERSIBLE ReactionCatalysis1331 ACTIVATION Citric Acid Cycle SubPathway ReactionCatalysis1330 ACTIVATION NADH PALMITYL-COA BioCyc ReactionCatalysis1333 ACTIVATION Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids SubPathway 3-Oxohexadecanoyl-CoA 1.0 SMILES CCCCCCCCCCCC(=O)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=C(N)N=CN=C12 ReactionCatalysis292 ACTIVATION SMILES CCCCCCCCCCC[C@H](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=C(N)N=CN=C12 1038 PubChem-compound SMILES CCCCCCCCCCCCC[C@H](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=C(N)N=CN=C12 440602 PubChem-compound 440601 PubChem-compound 440600 PubChem-compound ReactionCatalysis1346 ACTIVATION ReactionCatalysis1345 ACTIVATION SMILES CCCCCCCCCCCCCCCCCC(=O)OC(CC([O-])=O)C[N+](C)(C)C C39H68N7O17P3S trans-Octadec-2-enoyl-CoA 1031.3605 1.0 Reduced electron-transfer flavoprotein HMDB00902 HMDB 1.3.8.8 false 1.3.8.8 Palmityl-CoA + electron-transfer flavoprotein → (2E)-Hexadecenoyl-CoA + Reduced electron-transfer flavoprotein LEFT_TO_RIGHT Stearoylcarnitine 1.0 ReactionCatalysis1344 ACTIVATION CHEBI:16908 ChEBI ReactionCatalysis1343 ACTIVATION 1.0 ReactionCatalysis285 ACTIVATION ReactionCatalysis284 ACTIVATION ReactionCatalysis281 ACTIVATION ReactionCatalysis280 ACTIVATION SMILES CCCCCCCCCCCCCC(=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=C(N)N=CN=C12 1.0 1.0 NAD Coenzyme A Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial 1.0 CHEBI:61526 ChEBI Mitochondrion TransportCatalysis100 ACTIVATION CHEBI:15521 ChEBI electron-transfer flavoprotein 541-15-1 CAS CHEBI:15525 ChEBI C37H60N7O17P3S (2E)-Hexadecenoyl-CoA 999.2979 58-68-4 CAS SMILES CCCCCCCCCCCCCCC\C=C\C(=O)SCCNC(=O)CCNC(=O)[C@@](O)([H])C(C)(C)COP(=O)(O)OP(=O)(O)OC[C@H]1O[C@H](C(O)[C@H]1OP(=O)(O)O)N1C=NC2=C1N=CN=C2N C35H60N7O17P3S (2E)-Tetradecenoyl-CoA 975.2979 CHEBI:28842 ChEBI (2E)-Tetradecenoyl-CoA 5091 ChemSpider CHEBI:28726 ChEBI 1.0 4.0 Pyrophosphate P30084 UniProt 1.0 CHEBI:15532 ChEBI 1.0 (2E)-Hexadecenoyl-CoA 1.0 1763-10-6 CAS TETRADECANOYL-COA BioCyc (2E)-Hexadecenoyl-CoA C05259 KEGG Compound C05258 KEGG Compound P23786 UniProt C05260 KEGG Compound GO:0005759 GENE ONTOLOGY 4.0 C05261 KEGG Compound C35H62N7O17P3S Tetradecanoyl-CoA 977.3136 1.0 1.0 Mitochondrial carnitine/acylcarnitine carrier protein 1.0 Hydrogen Ion 1.0 O7P2 Pyrophosphate 173.91193 SMILES CCCCCCCCCCCCCCCC(=O)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 (S)-Hydroxyoctadecanoyl-CoA C21H29N7O14P2 NADH 665.12476 C05273 KEGG Compound 5957 PubChem-compound C05272 KEGG Compound GO:0005743 GENE ONTOLOGY GO:0005741 GENE ONTOLOGY 1.0 (S)-3-Hydroxyhexadecanoyl-CoA 1.0 34619-89-1 CAS 1.0 1.0 Water Palmityl-CoA (2E)-Hexadecenoyl-CoA C37H66N7O17P3S Palmityl-CoA 1005.34485 CHEBI:27721 ChEBI GO:0005739 GENE ONTOLOGY ReactionCatalysis1317 ACTIVATION ReactionCatalysis1316 ACTIVATION GO:0005737 GENE ONTOLOGY 1.0 ReactionCatalysis1319 ACTIVATION ReactionCatalysis1318 ACTIVATION P33121 UniProt Mitochondrial Outer Membrane 58623 ChemSpider 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 1.0 5682 ChemSpider Reduced electron-transfer flavoprotein CHEBI:1146900 ChEBI 1.0 4.0 Mitochondrial Matrix STEAROYL-COA BioCyc 5280767 PubChem-compound 14000-31-8 CAS CO-A BioCyc HMDB06529 HMDB Stearoyl-CoA 1.0 72-89-9 CAS HMDB06402 HMDB 1.0 10917 PubChem-compound C21H36N7O16P3S Coenzyme A 767.11523 1.0 1.0 4.0 Cytoplasm 10455 ChemSpider 1.0 Q16836 UniProt Cell Trifunctional enzyme subunit beta, mitochondrial CHEBI:15541 ChEBI 14902 ChemSpider CHEBI:15422 ChEBI 1.0 6557 ChemSpider 1.0 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 2.0 SMILES CCCCCCCCCCCC(=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=C(N)N=CN=C12 C01832 KEGG Compound 122364-86-7 CAS CHEBI:27402 ChEBI 9606 TAXONOMY Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial PW000022 PathWhiz 392413 ChemSpider 3-ketoacyl-CoA thiolase, mitochondrial 1.0 145018 ChemSpider 5893 PubChem-compound HMDB02111 HMDB 1.0 1.0 17216373 ChemSpider Trifunctional enzyme subunit alpha, mitochondrial 4932275 ChemSpider CHEBI:15377 ChEBI CHEBI:16347 ChEBI 3-ketoacyl-CoA thiolase, mitochondrial 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 HMDB03571 HMDB HMDB00062 HMDB CHEBI:15491 ChEBI 389499 ChemSpider 389498 ChemSpider CHEBI:27466 ChEBI C40H71N6O18P3S (S)-Hydroxyoctadecanoyl-CoA 1048.3759 SMILES CCCCCCCCCCCCCCCCCC(O)=O 86370-20-9 CAS 26332261 ChemSpider 439229 PubChem-compound HMDB01487 HMDB 4444333 ChemSpider 362-66-3 CAS 1.0 (2E)-Tetradecenoyl-CoA PW000005 PathWhiz 4.0 L-Carnitine Long-chain specific acyl-CoA dehydrogenase, mitochondrial 46173176 PubChem-compound 6244-92-4 CAS C00412 KEGG Compound SMILES CCCCCCCCCCCCCCCCCC(=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 HMDB00045 HMDB 15667 PubChem-compound Long-chain specific acyl-CoA dehydrogenase, mitochondrial 1.0 CHEBI:15351 ChEBI 2.3.1.21 false 2.3.1.21 L-Carnitine + Stearoyl-CoA ↔ Coenzyme A + Stearoylcarnitine REVERSIBLE 1.0 6.2.1.3 false 6.2.1.3 Adenosine triphosphate + Coenzyme A + Stearic acid → Adenosine monophosphate + Pyrophosphate + Stearoyl-CoA LEFT_TO_RIGHT 3-Oxotetradecanoyl-CoA (S)-Hydroxyoctadecanoyl-CoA 85-61-0 CAS 1.1.1.35 false 1.1.1.35 (S)-Hydroxyoctadecanoyl-CoA + NAD → 3-Oxooctadecanoyl-CoA + Hydrogen Ion + NADH LEFT_TO_RIGHT Reaction1588 false 3-Oxooctadecanoyl-CoA + Coenzyme A → Acetyl-CoA + Palmityl-CoA LEFT_TO_RIGHT 1.3.8.8 false 1.3.8.8 Stearoyl-CoA + electron-transfer flavoprotein → Reduced electron-transfer flavoprotein + trans-Octadec-2-enoyl-CoA LEFT_TO_RIGHT 4.2.1.17 false 4.2.1.17 Water + trans-Octadec-2-enoyl-CoA → (S)-Hydroxyoctadecanoyl-CoA LEFT_TO_RIGHT Mitochondrial carnitine/acylcarnitine carrier protein Mitochondrial carnitine/acylcarnitine carrier protein Carnitine O- palmitoyltransferase 1, liver isoform Long-chain- fatty-acid-- CoA ligase 1 Carnitine O- palmitoyltransferase 2, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Stearic acid Acetyl-CoA Acetyl-CoA Acetyl-CoA Lauroyl-CoA L-Carnitine L-Carnitine Stearoylcarnitine Stearoylcarnitine Stearoyl-CoA CoA CoA ATP PP i AMP Stearoyl-CoA CoA trans-Octadec-2-enoyl-CoA H 2 O (S)-Hydroxyoctadecanoyl-CoA H 2 O NAD 3-Oxooctadecanoyl-CoA NADH H + NAD NADH H + CoA Palmityl-CoA (S)-3-Hydroxyhexadecanoyl-CoA (2E)-Hexadecenoyl-CoA H 2 O H 2 O NAD 3-Oxohexadecanoyl-CoA NADH H + Tetradecanoyl-CoA CoA CoA CoA NAD NADH H + (S)-3-Hydroxytetradecanoyl-CoA (2E)-Tetradecenoyl-CoA H 2 O H 2 O NAD 3-Oxotetradecanoyl-CoA NADH H + NAD NADH H + CoA CoA Magnesium FAD FAD FAD electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Fatty Acid Biosynthesis Fatty Acid Elongation In Mitochondria Citric Acid Cycle Citric Acid Cycle Citric Acid Cycle Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids Extracellular Space Intracellular Space Mitochondrial Outer Membrane Mitochondrial Inner Membrane Mitochondrial Matrix Mitochondria
SubPathwayInteraction398 SubPathway398Reaction SubPathwayReaction SubPathwayInteraction399 SubPathwayReaction SubPathway399Reaction C23H38N7O17P3S Acetyl-CoA 809.1258 C00318 KEGG Compound 937 ChemSpider HMDB00250 HMDB 439153 PubChem-compound L-Carnitine Trifunctional enzyme subunit alpha, mitochondrial Enoyl-CoA hydratase, mitochondrial 1.0 Trifunctional enzyme subunit beta, mitochondrial C33H58N7O17P3S Lauroyl-CoA 949.2823 HMDB01114 HMDB CHEBI:16027 ChEBI 53481913 PubChem-compound 38795-33-4 CAS 5281 PubChem-compound C01530 KEGG Compound ATP BioCyc PW000173 PathWhiz 388366 ChemSpider PW000172 PathWhiz Mitochondrial carnitine/acylcarnitine carrier protein 559142 ChemSpider Transport129 false Stearoylcarnitine (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT 1.0 Transport128 false L-Carnitine (→) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Cytoplasm LEFT_TO_RIGHT 1.0 PW000167 PathWhiz Tetradecanoyl-CoA HMDB01206 HMDB (S)-3-Hydroxytetradecanoyl-CoA 5742 ChemSpider 1.0 11966197 PubChem-compound 1.0 Trifunctional enzyme, mitochondrial HMDB59597 HMDB CHEBI:73074 ChEBI L-Carnitine 5858 ChemSpider Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids HMDB01338 HMDB Stearoyl-CoA Lauroyl-CoA 1.0 Stearic acid 1010 ChemSpider Enoyl-CoA hydratase, mitochondrial 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 PPI BioCyc http://identifiers.org/smpdb/SMP00057 SMPDB http://identifiers.org/smpdb/SMP00054 SMPDB HMDB01423 HMDB trans-Octadec-2-enoyl-CoA Trifunctional enzyme, mitochondrial CHEBI:5086 ChEBI LAUROYLCOA-CPD BioCyc Lauroyl-CoA SubPathwayInput 1.0 Long-chain-fatty-acid--CoA ligase 1 Stearoylcarnitine C39H70N7O17P3S Stearoyl-CoA 1033.3762 Fatty Acid Biosynthesis SubPathway Fatty Acid Elongation In Mitochondria SubPathway 53477850 PubChem-compound 3-ketoacyl-CoA thiolase, mitochondrial C00001 KEGG Compound 1.0 CHEBI:18361 ChEBI Long-chain specific acyl-CoA dehydrogenase, mitochondrial NADH C00004 KEGG Compound C00003 KEGG Compound C00002 KEGG Compound P42765 UniProt Coenzyme A Mitochondrial Inner Membrane L-Carnitine HMDB13640 HMDB 3130-72-1 CAS HMDB01521 HMDB 389500 ChemSpider HMDB03945 HMDB C21H28N7O14P2 NAD 664.11694 HMDB03946 HMDB C00010 KEGG Compound C00013 KEGG Compound trans-Octadec-2-enoyl-CoA http://identifiers.org/smpdb/SMP00481 SMPDB http://identifiers.org/smpdb/SMP00482 SMPDB Stearoylcarnitine P28330 UniProt H Hydrogen Ion 1.007825 CARNITINE BioCyc 56-65-5 CAS C00020 KEGG Compound 53-84-9 CAS Carnitine O-palmitoyltransferase 1 C00024 KEGG Compound Trifunctional enzyme subunit beta, mitochondrial SMILES CCCCCCCCCCCCCCCC(O)CC(=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=C1C=CN=C2N Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial Adenosine monophosphate 4460-95-1 CAS Stearic acid SubPathwayOutput Stearoylcarnitine 3-Oxooctadecanoyl-CoA BiologicalState22 Homo sapiens, Cell, Mitochondrial Outer Membrane 3-Oxohexadecanoyl-CoA CL:0000000 CELL TYPE ONTOLOGY C00154 KEGG Compound C37H66N7O18P3S (S)-3-Hydroxyhexadecanoyl-CoA 1021.3398 C35H62N7O18P3S (S)-3-Hydroxytetradecanoyl-CoA 993.3085 Carnitine O-palmitoyltransferase 1, liver isoform C35H60N7O18P3S 3-Oxotetradecanoyl-CoA 991.29285 Carnitine O-palmitoyltransferase 2, mitochondrial Long-chain-fatty-acid--CoA ligase 1 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 AMP BioCyc 35106-50-4 CAS STEARIC_ACID BioCyc Tetradecanoyl-CoA SMILES [O-]P([O-])(=O)OP([O-])([O-])=O HMDB03934 HMDB HMDB03935 HMDB 1.0 HMDB03932 HMDB C25H49NO4 Stearoylcarnitine 427.36615 trans-Octadec-2-enoyl-CoA SMILES CCCCCCCCCCCCCCCC(=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 C02593 KEGG Compound C37H64N7O18P3S 3-Oxohexadecanoyl-CoA 1019.32416 1.0 HMDB00538 HMDB 7732-18-5 CAS 4.0 Acetyl-CoA 3-OXOPALMITOYL-COA BioCyc 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 3-Oxooctadecanoyl-CoA O43772 UniProt 1.0 BiologicalState17 Homo sapiens, Cell, Mitochondrial Inner Membrane 1.0 C10H16N5O13P3 Adenosine triphosphate 506.99576 http://identifiers.org/smpdb/SMP00456 SMPDB Stearoyl-CoA 165436 PubChem-compound NAD SMILES C[N+](C)(C)C[C@H](O)CC([O-])=O 1.0 C18H36O2 Stearic acid 284.27155 Adenosine triphosphate 22833663 PubChem-compound 962 PubChem-compound C00080 KEGG Compound P50416 UniProt Water C39H66N7O18P3S 3-Oxooctadecanoyl-CoA 1045.3398 Hydrogen Ion Enoyl-CoA hydratase, mitochondrial 1.0 HMDB00848 HMDB BiologicalState3 Homo sapiens, Cell, Mitochondrion BiologicalState4 Homo sapiens, Cell, Mitochondrial Matrix 57-11-4 CAS 6.0 1.0 1.0 6426855 PubChem-compound 6.0 1.0 1.0 644102 PubChem-compound 1.0 444493 PubChem-compound SubPathwayInteraction416 SubPathwayReaction SubPathway416Reaction 6083 PubChem-compound Palmityl-CoA SMILES CCCCCCCCCCCCC\C=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 HMDB06498 HMDB ACETYL-COA BioCyc SMILES CCCCCCCCCCC\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=C1N=CN=C2N H2O Water 18.010565 Carnitine O-palmitoyltransferase 2, mitochondrial P55084 UniProt HMDB00827 HMDB NADH BioCyc NAD BioCyc SubPathwayInteraction400 SubPathway400Reaction SubPathwayReaction 1976-27-8 CAS Acetyl-CoA SubPathwayInput Trifunctional enzyme subunit alpha, mitochondrial 1.0 SMILES CCCCCCCCCCCCCC(=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 65113 PubChem-compound (S)-3-Hydroxytetradecanoyl-CoA 1.0 TransportCatalysis99 ACTIVATION ReactionCatalysis1324 ACTIVATION ReactionCatalysis1323 ACTIVATION 3-Oxotetradecanoyl-CoA ReactionCatalysis1328 ACTIVATION ReactionCatalysis1327 ACTIVATION Coenzyme A 10140190 ChemSpider SMILES O C16216 KEGG Compound Palmityl-CoA C16218 KEGG Compound C10H14N5O7P Adenosine monophosphate 347.06308 ReactionCatalysis1320 ACTIVATION ReactionCatalysis1322 ACTIVATION ReactionCatalysis1321 ACTIVATION P40939 UniProt 4.2.1.17 false 4.2.1.17 (S)-3-Hydroxyhexadecanoyl-CoA ↔ (2E)-Hexadecenoyl-CoA + Water REVERSIBLE 2.3.1.16 false 2.3.1.16 Acetyl-CoA + Tetradecanoyl-CoA ↔ 3-Oxohexadecanoyl-CoA + Coenzyme A REVERSIBLE 1.1.1.35 false 1.1.1.35 (S)-3-Hydroxyhexadecanoyl-CoA + NAD ↔ 3-Oxohexadecanoyl-CoA + Hydrogen Ion + NADH REVERSIBLE Carnitine O-palmitoyltransferase 1, liver isoform 6816 PubChem-compound 4.2.1.17 false 4.2.1.17 (S)-3-Hydroxytetradecanoyl-CoA ↔ (2E)-Tetradecenoyl-CoA + Water REVERSIBLE CHEBI:15846 ChEBI (S)-3-Hydroxyhexadecanoyl-CoA 2.3.1.16 false 2.3.1.16 Acetyl-CoA + Lauroyl-CoA → 3-Oxotetradecanoyl-CoA + Coenzyme A LEFT_TO_RIGHT C7H15NO3 L-Carnitine 161.1052 1.1.1.35 false 1.1.1.35 (S)-3-Hydroxytetradecanoyl-CoA + NAD ↔ 3-Oxotetradecanoyl-CoA + Hydrogen Ion + NADH REVERSIBLE ReactionCatalysis1331 ACTIVATION Citric Acid Cycle SubPathway ReactionCatalysis1330 ACTIVATION NADH PALMITYL-COA BioCyc ReactionCatalysis1333 ACTIVATION Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids SubPathway 3-Oxohexadecanoyl-CoA 1.0 SMILES CCCCCCCCCCCC(=O)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=C(N)N=CN=C12 ReactionCatalysis292 ACTIVATION SMILES CCCCCCCCCCC[C@H](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=C(N)N=CN=C12 1038 PubChem-compound SMILES CCCCCCCCCCCCC[C@H](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=C(N)N=CN=C12 440602 PubChem-compound 440601 PubChem-compound 440600 PubChem-compound ReactionCatalysis1346 ACTIVATION ReactionCatalysis1345 ACTIVATION SMILES CCCCCCCCCCCCCCCCCC(=O)OC(CC([O-])=O)C[N+](C)(C)C C39H68N7O17P3S trans-Octadec-2-enoyl-CoA 1031.3605 1.0 Reduced electron-transfer flavoprotein HMDB00902 HMDB 1.3.8.8 false 1.3.8.8 Palmityl-CoA + electron-transfer flavoprotein → (2E)-Hexadecenoyl-CoA + Reduced electron-transfer flavoprotein LEFT_TO_RIGHT Stearoylcarnitine 1.0 ReactionCatalysis1344 ACTIVATION CHEBI:16908 ChEBI ReactionCatalysis1343 ACTIVATION 1.0 ReactionCatalysis285 ACTIVATION ReactionCatalysis284 ACTIVATION ReactionCatalysis281 ACTIVATION ReactionCatalysis280 ACTIVATION SMILES CCCCCCCCCCCCCC(=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=C(N)N=CN=C12 1.0 1.0 NAD Coenzyme A Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial 1.0 CHEBI:61526 ChEBI Mitochondrion TransportCatalysis100 ACTIVATION CHEBI:15521 ChEBI electron-transfer flavoprotein 541-15-1 CAS CHEBI:15525 ChEBI C37H60N7O17P3S (2E)-Hexadecenoyl-CoA 999.2979 58-68-4 CAS SMILES CCCCCCCCCCCCCCC\C=C\C(=O)SCCNC(=O)CCNC(=O)[C@@](O)([H])C(C)(C)COP(=O)(O)OP(=O)(O)OC[C@H]1O[C@H](C(O)[C@H]1OP(=O)(O)O)N1C=NC2=C1N=CN=C2N C35H60N7O17P3S (2E)-Tetradecenoyl-CoA 975.2979 CHEBI:28842 ChEBI (2E)-Tetradecenoyl-CoA 5091 ChemSpider CHEBI:28726 ChEBI 1.0 4.0 Pyrophosphate P30084 UniProt 1.0 CHEBI:15532 ChEBI 1.0 (2E)-Hexadecenoyl-CoA 1.0 1763-10-6 CAS TETRADECANOYL-COA BioCyc (2E)-Hexadecenoyl-CoA C05259 KEGG Compound C05258 KEGG Compound P23786 UniProt C05260 KEGG Compound GO:0005759 GENE ONTOLOGY 4.0 C05261 KEGG Compound C35H62N7O17P3S Tetradecanoyl-CoA 977.3136 1.0 1.0 Mitochondrial carnitine/acylcarnitine carrier protein 1.0 Hydrogen Ion 1.0 O7P2 Pyrophosphate 173.91193 SMILES CCCCCCCCCCCCCCCC(=O)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 (S)-Hydroxyoctadecanoyl-CoA C21H29N7O14P2 NADH 665.12476 C05273 KEGG Compound 5957 PubChem-compound C05272 KEGG Compound GO:0005743 GENE ONTOLOGY GO:0005741 GENE ONTOLOGY 1.0 (S)-3-Hydroxyhexadecanoyl-CoA 1.0 34619-89-1 CAS 1.0 1.0 Water Palmityl-CoA (2E)-Hexadecenoyl-CoA C37H66N7O17P3S Palmityl-CoA 1005.34485 CHEBI:27721 ChEBI GO:0005739 GENE ONTOLOGY ReactionCatalysis1317 ACTIVATION ReactionCatalysis1316 ACTIVATION GO:0005737 GENE ONTOLOGY 1.0 ReactionCatalysis1319 ACTIVATION ReactionCatalysis1318 ACTIVATION P33121 UniProt Mitochondrial Outer Membrane 58623 ChemSpider 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 1.0 5682 ChemSpider Reduced electron-transfer flavoprotein CHEBI:1146900 ChEBI 1.0 4.0 Mitochondrial Matrix STEAROYL-COA BioCyc 5280767 PubChem-compound 14000-31-8 CAS CO-A BioCyc HMDB06529 HMDB Stearoyl-CoA 1.0 72-89-9 CAS HMDB06402 HMDB 1.0 10917 PubChem-compound C21H36N7O16P3S Coenzyme A 767.11523 1.0 1.0 4.0 Cytoplasm 10455 ChemSpider 1.0 Q16836 UniProt Cell Trifunctional enzyme subunit beta, mitochondrial CHEBI:15541 ChEBI 14902 ChemSpider CHEBI:15422 ChEBI 1.0 6557 ChemSpider 1.0 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 2.0 SMILES CCCCCCCCCCCC(=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=C(N)N=CN=C12 C01832 KEGG Compound 122364-86-7 CAS CHEBI:27402 ChEBI 9606 TAXONOMY Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial PW000022 PathWhiz 392413 ChemSpider 3-ketoacyl-CoA thiolase, mitochondrial 1.0 145018 ChemSpider 5893 PubChem-compound HMDB02111 HMDB 1.0 1.0 17216373 ChemSpider Trifunctional enzyme subunit alpha, mitochondrial 4932275 ChemSpider CHEBI:15377 ChEBI CHEBI:16347 ChEBI 3-ketoacyl-CoA thiolase, mitochondrial 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 HMDB03571 HMDB HMDB00062 HMDB CHEBI:15491 ChEBI 389499 ChemSpider 389498 ChemSpider CHEBI:27466 ChEBI C40H71N6O18P3S (S)-Hydroxyoctadecanoyl-CoA 1048.3759 SMILES CCCCCCCCCCCCCCCCCC(O)=O 86370-20-9 CAS 26332261 ChemSpider 439229 PubChem-compound HMDB01487 HMDB 4444333 ChemSpider 362-66-3 CAS 1.0 (2E)-Tetradecenoyl-CoA PW000005 PathWhiz 4.0 L-Carnitine Long-chain specific acyl-CoA dehydrogenase, mitochondrial 46173176 PubChem-compound 6244-92-4 CAS C00412 KEGG Compound SMILES CCCCCCCCCCCCCCCCCC(=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 HMDB00045 HMDB 15667 PubChem-compound Long-chain specific acyl-CoA dehydrogenase, mitochondrial 1.0 CHEBI:15351 ChEBI 2.3.1.21 false 2.3.1.21 L-Carnitine + Stearoyl-CoA ↔ Coenzyme A + Stearoylcarnitine REVERSIBLE 1.0 6.2.1.3 false 6.2.1.3 Adenosine triphosphate + Coenzyme A + Stearic acid → Adenosine monophosphate + Pyrophosphate + Stearoyl-CoA LEFT_TO_RIGHT 3-Oxotetradecanoyl-CoA (S)-Hydroxyoctadecanoyl-CoA 85-61-0 CAS 1.1.1.35 false 1.1.1.35 (S)-Hydroxyoctadecanoyl-CoA + NAD → 3-Oxooctadecanoyl-CoA + Hydrogen Ion + NADH LEFT_TO_RIGHT Reaction1588 false 3-Oxooctadecanoyl-CoA + Coenzyme A → Acetyl-CoA + Palmityl-CoA LEFT_TO_RIGHT 1.3.8.8 false 1.3.8.8 Stearoyl-CoA + electron-transfer flavoprotein → Reduced electron-transfer flavoprotein + trans-Octadec-2-enoyl-CoA LEFT_TO_RIGHT 4.2.1.17 false 4.2.1.17 Water + trans-Octadec-2-enoyl-CoA → (S)-Hydroxyoctadecanoyl-CoA LEFT_TO_RIGHT