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CHEBI:16015 ChEBI 7722-84-1 CAS C23H38N7O17P3S Acetyl-CoA 809.1258 937 ChemSpider 6.0 Mitochondrial ornithine transporter 2 388353 ChemSpider 439153 PubChem-compound Enoyl-CoA hydratase, mitochondrial 50 ChemSpider C00449 KEGG Compound HMDB00148 HMDB 1.0 388365 ChemSpider C00322 KEGG Compound 1.3.8.6 false 1.3.8.6 Glutaryl-CoA + electron-transfer flavoprotein → Carbon dioxide + Crotonoyl-CoA + Reduced electron-transfer flavoprotein LEFT_TO_RIGHT Acetoacetyl-CoA SMP00037 SMPDB SACCHAROPINE BioCyc 5747 ChemSpider P09622 UniProt HMDB01206 HMDB Acetyl-CoA SubPathwayInput CHEBI:16474 ChEBI C00332 KEGG Compound 952 ChemSpider Alpha-aminoadipic semialdehyde dehydrogenase L-Lysine 440045 PubChem-compound Low affinity cationic amino acid transporter 2 HMDB59597 HMDB 70 ChemSpider L-Glutamic acid HMDB01339 HMDB 1.0 CHEBI:16240 ChEBI Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial 1010 ChemSpider Q9BXI2 UniProt 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 C6H9NO2 (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate 127.06333 SMP00057 SMPDB PW000029 PathWhiz Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial HMDB01423 HMDB CHEBI:17027 ChEBI HMDB00217 HMDB CHEBI:5086 ChEBI 165067 PubChem-compound 1.0 56-87-1 CAS HMDB00208 HMDB 3-Hydroxybutyryl-CoA HMDB00221 HMDB C00007 KEGG Compound C00006 KEGG Compound HMDB00225 HMDB CHEBI:18009 ChEBI C00001 KEGG Compound P52569 UniProt C00005 KEGG Compound C00004 KEGG Compound C00003 KEGG Compound Acetyl-CoA acetyltransferase, mitochondrial Peroxisomal sarcosine oxidase 103192-48-9 CAS 2.0 SMILES OC(=O)C1CCCC=N1 SMILES C[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=C1N=CN=C2N C21H28N7O14P2 NAD 664.11694 Hydrogen peroxide C00011 KEGG Compound C00010 KEGG Compound Extracellular Space NAD(P) BioCyc H Hydrogen Ion 1.007825 C6H11NO3 Allysine 145.0739 C01475 KEGG Compound 53-84-9 CAS C00027 KEGG Compound C00026 KEGG Compound C00025 KEGG Compound Q8N5Z0 UniProt C00024 KEGG Compound 1.2.4.2 false 1.2.4.2 Coenzyme A + Oxoadipic acid → Carbon dioxide + Glutaryl-CoA LEFT_TO_RIGHT Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial 3184-35-8 CAS (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate CL:0000000 CELL TYPE ONTOLOGY 328-50-7 CAS 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 Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial 2871-66-1 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 Peroxisome 1.0 HYDROGEN-PEROXIDE BioCyc 1.0 Acetyl-CoA acetyltransferase, mitochondrial Alpha-aminoadipic semialdehyde synthase, mitochondrial C00047 KEGG Compound 763 ChemSpider 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)CCCC(O)=O C03561 KEGG Compound SMILES N[C@@H](CCCCN[C@@H](CCC(O)=O)C(O)=O)C(O)=O 542-32-5 CAS CHEBI:18019 ChEBI 784 PubChem-compound Oxoglutaric acid HMDB00510 HMDB 7732-18-5 CAS Reaction818 false L-Lysine + NADPH + Oxoglutaric acid → NADP + Saccharopine + Water LEFT_TO_RIGHT Reaction819 false NAD + Saccharopine + Water → Allysine + L-Glutamic acid + NADH LEFT_TO_RIGHT NAD 56-86-0 CAS SMILES OC(=O)CCCC(=O)C(O)=O Q92947 UniProt SMILES NC(CCCC(O)=O)C(O)=O 1.0 Reaction825 false L-Pipecolic acid + Oxygen → (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate + Hydrogen peroxide LEFT_TO_RIGHT 1.0 Reaction824 true (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate + Water ↔ Allysine REVERSIBLE Reaction821 false Aminoadipic acid + Oxoglutaric acid ↔ L-Glutamic acid + Oxoadipic acid REVERSIBLE Reaction820 false Allysine + NAD + Water → Aminoadipic acid + NADH LEFT_TO_RIGHT HMDB01967 HMDB 962 PubChem-compound GLUTARYL-COA BioCyc Alpha-aminoadipic semialdehyde dehydrogenase CO2 Carbon dioxide 43.98983 Allysine C00080 KEGG Compound SMILES O=O 2.0 Alpha-aminoadipic semialdehyde synthase, mitochondrial BiologicalState5 BiologicalState3 SMILES N[C@@H](CCC(O)=O)C(O)=O SubPathwayInteraction862 SubPathway862Reaction SubPathwayReaction SubPathwayInteraction863 SubPathwayReaction SubPathway863Reaction C6H14N2O2 L-Lysine 146.10553 1.0 NADP Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial 1.0 144715 ChemSpider 3105-95-1 CAS 444493 PubChem-compound C6H11NO2 L-Pipecolic acid 129.07898 1.0 Crotonoyl-CoA Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial 22833512 PubChem-compound ACETYL-COA BioCyc H2O Water 18.010565 H2O2 Hydrogen peroxide 34.005478 Glutaryl-CoA Dihydrolipoyl dehydrogenase, mitochondrial 1.0 NADH BioCyc NAD BioCyc NADPH 977 PubChem-compound L-Lysine P49419 UniProt HMDB00716 HMDB 30572 ChemSpider 17215925 ChemSpider 1.0 2.0 Allysine SMILES OC(=O)[C@@H]1CCCCN1 2K-ADIPATE BioCyc C6H11NO4 Aminoadipic acid 161.0688 Enoyl-CoA hydratase, mitochondrial 1.0 SMILES O SMILES OO CHEBI:16927 ChEBI Alpha-aminoadipic semialdehyde dehydrogenase C6H8O5 Oxoadipic acid 160.03717 1.0 TransportCatalysis4 ACTIVATION 5962 PubChem-compound TransportCatalysis5 ACTIVATION 2.0 6816 PubChem-compound 51 PubChem-compound Water CHEBI:15846 ChEBI 102680-35-3 CAS ReactionCatalysis177 ACTIVATION Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial ReactionCatalysis178 ACTIVATION 53-59-8 CAS ReactionCatalysis176 ACTIVATION 5280381 PubChem-compound 1038 PubChem-compound Peroxisomal sarcosine oxidase LYS BioCyc C25H40N7O17P3S Crotonoyl-CoA 835.1414 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 Hydrogen Ion Reduced electron-transfer flavoprotein HMDB00902 HMDB CHEBI:16908 ChEBI 71 PubChem-compound C21H30N7O17P3 NADPH 745.0911 1.0 Mitochondrion C21H29N7O17P3 NADP 744.08325 1.0 NADPH BioCyc 207 PubChem-compound CHEBI:15524 ChEBI electron-transfer flavoprotein CPD-6641 BioCyc Glutaryl-CoA dehydrogenase, mitochondrial Alpha-aminoadipic semialdehyde synthase, mitochondrial 4444072 ChemSpider 58-68-4 CAS 456 ChemSpider Water SMILES C\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=C(N)N=CN=C12 SMILES OC(=O)CCC(=O)C(O)=O CHEBI:30915 ChEBI 124-38-9 CAS Q96HY7 UniProt CHEBI:30913 ChEBI P30084 UniProt Mitochondrial ornithine transporter 2 CPD-468 BioCyc 1.0 CPD-1083 BioCyc SMILES NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O 1.0 202 ChemSpider SMILES NC(=O)C1=CN(C=CC1)[C@H]1O[C@@H](COP(O)(=O)OP(O)(=O)OC[C@@H]2O[C@@H]([C@@H](OP(O)(O)=O)[C@H]2O)N2C=NC3=C(N)N=CN=C23)[C@H](O)[C@@H]1O P24752 UniProt GLT BioCyc 4.0 53-57-6 CAS 469 PubChem-compound SMILES O=C=O Oxoadipic acid Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial C25H40N7O18P3S Acetoacetyl-CoA 851.13635 (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate C21H29N7O14P2 NADH 665.12476 1.0 CHEBI:15753 ChEBI 1.0 SMILES NCCCC[C@H](N)C(O)=O ALLYSINE BioCyc 1.0 Low affinity cationic amino acid transporter 2 Enoyl-CoA hydratase, mitochondrial 1.0 C26H42N7O19P3S Glutaryl-CoA 881.1469 GO:0005739 GENE ONTOLOGY GO:0005615 GENE ONTOLOGY GO:0005737 GENE ONTOLOGY C11H20N2O6 Saccharopine 276.13214 1.0 Citric Acid Cycle SubPathway Biotin Metabolism SubPathway HMDB03125 HMDB Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial Q9UDR5 UniProt C00956 KEGG Compound Transport29 false L-Lysine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm LEFT_TO_RIGHT 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 5682 ChemSpider 2-METHYL-3-HYDROXY-BUTYRYL-COA BioCyc L-PIPECOLATE BioCyc Reduced electron-transfer flavoprotein CHEBI:1146900 ChEBI 1.0 Q9P0Z9 UniProt 141086 ChemSpider CO-A BioCyc SMILES NC(CCCC=O)C(O)=O 5675 ChemSpider 72-89-9 CAS 1.0 CHEBI:15452 ChEBI 389056 ChemSpider Oxygen Carbon dioxide C21H36N7O16P3S Coenzyme A 767.11523 Cytoplasm 997-68-2 CAS Oxoglutarate dehydrogenase complex ReactionCatalysis2143 ACTIVATION Q16836 UniProt Cell 3038-89-9 CAS ACETOACETYL-COA BioCyc Coenzyme A 6557 ChemSpider 1962-83-0 CAS 160556 PubChem-compound 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 HMDB01166 HMDB O2 Oxygen 31.98983 439214 PubChem-compound 4.0 C5H9NO4 L-Glutamic acid 147.05316 33032 PubChem-compound P36957 UniProt Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:16526 ChEBI Transport30 false L-Lysine (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrion LEFT_TO_RIGHT 7782-44-7 CAS 1.0 2.0 GO:0005777 GENE ONTOLOGY CHEBI:49015 ChEBI NADH 9606 TAXONOMY C00877 KEGG Compound 2-KETOGLUTARATE BioCyc Glutaryl-CoA dehydrogenase, mitochondrial 4.2.1.17 false 4.2.1.17 3-Hydroxybutyryl-CoA ↔ Crotonoyl-CoA + Water REVERSIBLE 392413 ChemSpider HMDB01263 HMDB 5893 PubChem-compound C25H42N7O18P3S 3-Hydroxybutyryl-CoA 853.152 HMDB02111 HMDB 1.0 1420-36-6 CAS SMP00066 SMPDB CHEBI:15377 ChEBI PW000013 PathWhiz CHEBI:15379 ChEBI CHEBI:15378 ChEBI SMILES [H+] C00408 KEGG Compound HMDB02009 HMDB C00527 KEGG Compound 4.0 HMDB00182 HMDB 5886 PubChem-compound CHEBI:37024 ChEBI 1.1.1.35 false 1.1.1.35 3-Hydroxybutyryl-CoA + NAD → Acetoacetyl-CoA + Hydrogen Ion + NADH LEFT_TO_RIGHT 280 PubChem-compound Acetyl-CoA acetyltransferase, mitochondrial Glutaryl-CoA dehydrogenase, mitochondrial 1.0 L-Pipecolic acid 439227 PubChem-compound 2.3.1.9 false 2.3.1.9 2 Acetyl-CoA ↔ Acetoacetyl-CoA + Coenzyme A REVERSIBLE HMDB01487 HMDB Saccharopine L-Lysine SubPathwayInput HMDB00279 HMDB CHEBI:15345 ChEBI PW000005 PathWhiz ReactionCatalysis371 ACTIVATION 1.0 HMDB01484 HMDB 1.0 439252 PubChem-compound Lysine Degradation Aminoadipic acid ReactionCatalysis2233 ACTIVATION ReactionCatalysis368 ACTIVATION ReactionCatalysis366 ACTIVATION HMDB01377 HMDB ReactionCatalysis367 ACTIVATION ReactionCatalysis365 ACTIVATION CHEBI:15351 ChEBI 388388 ChemSpider CHEBI:15473 ChEBI HMDB12130 HMDB 1.0 1.0 85-61-0 CAS Low affinity cationic amino acid transporter 2 Mitochondrial ornithine transporter 2 Alpha- aminoadipic semialdehyde synthase, mitochondrial Alpha- aminoadipic semialdehyde synthase, mitochondrial Alpha- aminoadipic semialdehyde dehydrogenase Kynurenine/alpha- aminoadipate aminotransferase, mitochondrial Enoyl-CoA hydratase, mitochondrial Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Peroxisomal sarcosine oxidase Glutaryl-CoA dehydrogenase, mitochondrial Oxoglutarate dehydrogenase complex Acetyl-CoA L-Lysine L-Lysine L-Lysine NADPH Oxoglutaric acid Saccharopine NADP H 2 O H 2 O NAD Allysine L-Glutamic acid NADH NAD H 2 O Aminoadipic acid NADH Oxoglutaric acid Oxoadipic acid L-Glutamic acid 3-Hydroxybutyryl-CoA Crotonoyl-CoA H 2 O NAD Acetoacetyl-CoA NADH CoA (S)-2,3,4,5- Tetrahydropiperidine-2- carboxylate H 2 O L-Pipecolic acid O 2 H 2 O 2 Glutaryl-CoA CO 2 CoA Pyridoxal 5'-phosphate FAD FAD Thiamine pyrophosphate Lipoamide FAD Citric Acid Cycle Biotin Metabolism Inner Mitochondria Membrane Outer Mitochondria Membrane Mitochondrial Intermembrane Space Peroxisome Mitochondria Extracellular Space Intracellular Space Liver Extracellular Space Mitochondrial matrix
CHEBI:16015 ChEBI 7722-84-1 CAS C23H38N7O17P3S Acetyl-CoA 809.1258 937 ChemSpider 6.0 Mitochondrial ornithine transporter 2 388353 ChemSpider 439153 PubChem-compound Enoyl-CoA hydratase, mitochondrial 50 ChemSpider C00449 KEGG Compound HMDB00148 HMDB 1.0 388365 ChemSpider C00322 KEGG Compound 1.3.8.6 false 1.3.8.6 Glutaryl-CoA + electron-transfer flavoprotein → Carbon dioxide + Crotonoyl-CoA + Reduced electron-transfer flavoprotein LEFT_TO_RIGHT Acetoacetyl-CoA SMP00037 SMPDB SACCHAROPINE BioCyc 5747 ChemSpider P09622 UniProt HMDB01206 HMDB Acetyl-CoA SubPathwayInput CHEBI:16474 ChEBI C00332 KEGG Compound 952 ChemSpider Alpha-aminoadipic semialdehyde dehydrogenase L-Lysine 440045 PubChem-compound Low affinity cationic amino acid transporter 2 HMDB59597 HMDB 70 ChemSpider L-Glutamic acid HMDB01339 HMDB 1.0 CHEBI:16240 ChEBI Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial 1010 ChemSpider Q9BXI2 UniProt 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 C6H9NO2 (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate 127.06333 SMP00057 SMPDB PW000029 PathWhiz Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial HMDB01423 HMDB CHEBI:17027 ChEBI HMDB00217 HMDB CHEBI:5086 ChEBI 165067 PubChem-compound 1.0 56-87-1 CAS HMDB00208 HMDB 3-Hydroxybutyryl-CoA HMDB00221 HMDB C00007 KEGG Compound C00006 KEGG Compound HMDB00225 HMDB CHEBI:18009 ChEBI C00001 KEGG Compound P52569 UniProt C00005 KEGG Compound C00004 KEGG Compound C00003 KEGG Compound Acetyl-CoA acetyltransferase, mitochondrial Peroxisomal sarcosine oxidase 103192-48-9 CAS 2.0 SMILES OC(=O)C1CCCC=N1 SMILES C[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=C1N=CN=C2N C21H28N7O14P2 NAD 664.11694 Hydrogen peroxide C00011 KEGG Compound C00010 KEGG Compound Extracellular Space NAD(P) BioCyc H Hydrogen Ion 1.007825 C6H11NO3 Allysine 145.0739 C01475 KEGG Compound 53-84-9 CAS C00027 KEGG Compound C00026 KEGG Compound C00025 KEGG Compound Q8N5Z0 UniProt C00024 KEGG Compound 1.2.4.2 false 1.2.4.2 Coenzyme A + Oxoadipic acid → Carbon dioxide + Glutaryl-CoA LEFT_TO_RIGHT Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial 3184-35-8 CAS (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate CL:0000000 CELL TYPE ONTOLOGY 328-50-7 CAS 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 Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial 2871-66-1 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 Peroxisome 1.0 HYDROGEN-PEROXIDE BioCyc 1.0 Acetyl-CoA acetyltransferase, mitochondrial Alpha-aminoadipic semialdehyde synthase, mitochondrial C00047 KEGG Compound 763 ChemSpider 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)CCCC(O)=O C03561 KEGG Compound SMILES N[C@@H](CCCCN[C@@H](CCC(O)=O)C(O)=O)C(O)=O 542-32-5 CAS CHEBI:18019 ChEBI 784 PubChem-compound Oxoglutaric acid HMDB00510 HMDB 7732-18-5 CAS Reaction818 false L-Lysine + NADPH + Oxoglutaric acid → NADP + Saccharopine + Water LEFT_TO_RIGHT Reaction819 false NAD + Saccharopine + Water → Allysine + L-Glutamic acid + NADH LEFT_TO_RIGHT NAD 56-86-0 CAS SMILES OC(=O)CCCC(=O)C(O)=O Q92947 UniProt SMILES NC(CCCC(O)=O)C(O)=O 1.0 Reaction825 false L-Pipecolic acid + Oxygen → (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate + Hydrogen peroxide LEFT_TO_RIGHT 1.0 Reaction824 true (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate + Water ↔ Allysine REVERSIBLE Reaction821 false Aminoadipic acid + Oxoglutaric acid ↔ L-Glutamic acid + Oxoadipic acid REVERSIBLE Reaction820 false Allysine + NAD + Water → Aminoadipic acid + NADH LEFT_TO_RIGHT HMDB01967 HMDB 962 PubChem-compound GLUTARYL-COA BioCyc Alpha-aminoadipic semialdehyde dehydrogenase CO2 Carbon dioxide 43.98983 Allysine C00080 KEGG Compound SMILES O=O 2.0 Alpha-aminoadipic semialdehyde synthase, mitochondrial BiologicalState5 BiologicalState3 SMILES N[C@@H](CCC(O)=O)C(O)=O SubPathwayInteraction862 SubPathway862Reaction SubPathwayReaction SubPathwayInteraction863 SubPathwayReaction SubPathway863Reaction C6H14N2O2 L-Lysine 146.10553 1.0 NADP Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial 1.0 144715 ChemSpider 3105-95-1 CAS 444493 PubChem-compound C6H11NO2 L-Pipecolic acid 129.07898 1.0 Crotonoyl-CoA Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial 22833512 PubChem-compound ACETYL-COA BioCyc H2O Water 18.010565 H2O2 Hydrogen peroxide 34.005478 Glutaryl-CoA Dihydrolipoyl dehydrogenase, mitochondrial 1.0 NADH BioCyc NAD BioCyc NADPH 977 PubChem-compound L-Lysine P49419 UniProt HMDB00716 HMDB 30572 ChemSpider 17215925 ChemSpider 1.0 2.0 Allysine SMILES OC(=O)[C@@H]1CCCCN1 2K-ADIPATE BioCyc C6H11NO4 Aminoadipic acid 161.0688 Enoyl-CoA hydratase, mitochondrial 1.0 SMILES O SMILES OO CHEBI:16927 ChEBI Alpha-aminoadipic semialdehyde dehydrogenase C6H8O5 Oxoadipic acid 160.03717 1.0 TransportCatalysis4 ACTIVATION 5962 PubChem-compound TransportCatalysis5 ACTIVATION 2.0 6816 PubChem-compound 51 PubChem-compound Water CHEBI:15846 ChEBI 102680-35-3 CAS ReactionCatalysis177 ACTIVATION Kynurenine/alpha-aminoadipate aminotransferase, mitochondrial ReactionCatalysis178 ACTIVATION 53-59-8 CAS ReactionCatalysis176 ACTIVATION 5280381 PubChem-compound 1038 PubChem-compound Peroxisomal sarcosine oxidase LYS BioCyc C25H40N7O17P3S Crotonoyl-CoA 835.1414 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 Hydrogen Ion Reduced electron-transfer flavoprotein HMDB00902 HMDB CHEBI:16908 ChEBI 71 PubChem-compound C21H30N7O17P3 NADPH 745.0911 1.0 Mitochondrion C21H29N7O17P3 NADP 744.08325 1.0 NADPH BioCyc 207 PubChem-compound CHEBI:15524 ChEBI electron-transfer flavoprotein CPD-6641 BioCyc Glutaryl-CoA dehydrogenase, mitochondrial Alpha-aminoadipic semialdehyde synthase, mitochondrial 4444072 ChemSpider 58-68-4 CAS 456 ChemSpider Water SMILES C\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=C(N)N=CN=C12 SMILES OC(=O)CCC(=O)C(O)=O CHEBI:30915 ChEBI 124-38-9 CAS Q96HY7 UniProt CHEBI:30913 ChEBI P30084 UniProt Mitochondrial ornithine transporter 2 CPD-468 BioCyc 1.0 CPD-1083 BioCyc SMILES NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O 1.0 202 ChemSpider SMILES NC(=O)C1=CN(C=CC1)[C@H]1O[C@@H](COP(O)(=O)OP(O)(=O)OC[C@@H]2O[C@@H]([C@@H](OP(O)(O)=O)[C@H]2O)N2C=NC3=C(N)N=CN=C23)[C@H](O)[C@@H]1O P24752 UniProt GLT BioCyc 4.0 53-57-6 CAS 469 PubChem-compound SMILES O=C=O Oxoadipic acid Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial C25H40N7O18P3S Acetoacetyl-CoA 851.13635 (S)-2,3,4,5-Tetrahydropiperidine-2-carboxylate C21H29N7O14P2 NADH 665.12476 1.0 CHEBI:15753 ChEBI 1.0 SMILES NCCCC[C@H](N)C(O)=O ALLYSINE BioCyc 1.0 Low affinity cationic amino acid transporter 2 Enoyl-CoA hydratase, mitochondrial 1.0 C26H42N7O19P3S Glutaryl-CoA 881.1469 GO:0005739 GENE ONTOLOGY GO:0005615 GENE ONTOLOGY GO:0005737 GENE ONTOLOGY C11H20N2O6 Saccharopine 276.13214 1.0 Citric Acid Cycle SubPathway Biotin Metabolism SubPathway HMDB03125 HMDB Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial Q9UDR5 UniProt C00956 KEGG Compound Transport29 false L-Lysine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm LEFT_TO_RIGHT 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 5682 ChemSpider 2-METHYL-3-HYDROXY-BUTYRYL-COA BioCyc L-PIPECOLATE BioCyc Reduced electron-transfer flavoprotein CHEBI:1146900 ChEBI 1.0 Q9P0Z9 UniProt 141086 ChemSpider CO-A BioCyc SMILES NC(CCCC=O)C(O)=O 5675 ChemSpider 72-89-9 CAS 1.0 CHEBI:15452 ChEBI 389056 ChemSpider Oxygen Carbon dioxide C21H36N7O16P3S Coenzyme A 767.11523 Cytoplasm 997-68-2 CAS Oxoglutarate dehydrogenase complex ReactionCatalysis2143 ACTIVATION Q16836 UniProt Cell 3038-89-9 CAS ACETOACETYL-COA BioCyc Coenzyme A 6557 ChemSpider 1962-83-0 CAS 160556 PubChem-compound 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 HMDB01166 HMDB O2 Oxygen 31.98983 439214 PubChem-compound 4.0 C5H9NO4 L-Glutamic acid 147.05316 33032 PubChem-compound P36957 UniProt Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:16526 ChEBI Transport30 false L-Lysine (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrion LEFT_TO_RIGHT 7782-44-7 CAS 1.0 2.0 GO:0005777 GENE ONTOLOGY CHEBI:49015 ChEBI NADH 9606 TAXONOMY C00877 KEGG Compound 2-KETOGLUTARATE BioCyc Glutaryl-CoA dehydrogenase, mitochondrial 4.2.1.17 false 4.2.1.17 3-Hydroxybutyryl-CoA ↔ Crotonoyl-CoA + Water REVERSIBLE 392413 ChemSpider HMDB01263 HMDB 5893 PubChem-compound C25H42N7O18P3S 3-Hydroxybutyryl-CoA 853.152 HMDB02111 HMDB 1.0 1420-36-6 CAS SMP00066 SMPDB CHEBI:15377 ChEBI PW000013 PathWhiz CHEBI:15379 ChEBI CHEBI:15378 ChEBI SMILES [H+] C00408 KEGG Compound HMDB02009 HMDB C00527 KEGG Compound 4.0 HMDB00182 HMDB 5886 PubChem-compound CHEBI:37024 ChEBI 1.1.1.35 false 1.1.1.35 3-Hydroxybutyryl-CoA + NAD → Acetoacetyl-CoA + Hydrogen Ion + NADH LEFT_TO_RIGHT 280 PubChem-compound Acetyl-CoA acetyltransferase, mitochondrial Glutaryl-CoA dehydrogenase, mitochondrial 1.0 L-Pipecolic acid 439227 PubChem-compound 2.3.1.9 false 2.3.1.9 2 Acetyl-CoA ↔ Acetoacetyl-CoA + Coenzyme A REVERSIBLE HMDB01487 HMDB Saccharopine L-Lysine SubPathwayInput HMDB00279 HMDB CHEBI:15345 ChEBI PW000005 PathWhiz ReactionCatalysis371 ACTIVATION 1.0 HMDB01484 HMDB 1.0 439252 PubChem-compound Lysine Degradation Aminoadipic acid ReactionCatalysis2233 ACTIVATION ReactionCatalysis368 ACTIVATION ReactionCatalysis366 ACTIVATION HMDB01377 HMDB ReactionCatalysis367 ACTIVATION ReactionCatalysis365 ACTIVATION CHEBI:15351 ChEBI 388388 ChemSpider CHEBI:15473 ChEBI HMDB12130 HMDB 1.0 1.0 85-61-0 CAS