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HMDB00254 HMDB 6022 PubChem-compound C00798 KEGG Compound Propionylcarnitine C23H38N7O17P3S Acetyl-CoA 809.1258 C00318 KEGG Compound 4,8-Dimethylnonanoyl-CoA SubPathwayOutput HMDB00250 HMDB HMDB01341 HMDB CHEBI:49189 ChEBI Phytanoyl-CoA dioxygenase, peroxisomal Pristanal 50 ChemSpider 2.3.1.7 false 2.3.1.7 L-Carnitine + Propionyl-CoA ↔ Coenzyme A + Propionylcarnitine REVERSIBLE 1.0 HMDB01359 HMDB Oxidation of Branched Chain Fatty Acids 441253 PubChem-compound ATP BioCyc Mitochondrial carnitine/acylcarnitine carrier protein 559142 ChemSpider SMP00030 SMPDB 1.0 HMDB01206 HMDB 5742 ChemSpider 1.0 Acetyl-CoA SubPathwayOutput 952 ChemSpider Aldehyde dehydrogenase, mitochondrial PW000155 PathWhiz 1.0 C00100 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 2.3.1.21 false 2.3.1.21 4,8-Dimethylnonanoyl-CoA + L-Carnitine ↔ 4,8 Dimethylnonanoyl carnitine + Coenzyme A REVERSIBLE 2.3.1.7 false 2.3.1.7 Acetyl-CoA + L-Carnitine ↔ Coenzyme A + L-Acetylcarnitine REVERSIBLE 439164 PubChem-compound L-Acetylcarnitine PPI BioCyc Aldehyde dehydrogenase, mitochondrial SubPathwayInteraction236 SubPathwayReaction SubPathway236Reaction HMDB01423 HMDB C24H40N7O17P3S Propionyl-CoA 823.1414 2-hydroxyacyl-CoA lyase 1 Cytosol Formyl-CoA Carnitine O-acetyltransferase 388310 ChemSpider SUC BioCyc Transport71 false Pristanoyl-CoA (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Peroxisome LEFT_TO_RIGHT CHEBI:51340 ChEBI Transport73 false Propionylcarnitine (→) Transport: Homo sapiens, Cell, Peroxisome to Homo sapiens, Cell, Cytosol LEFT_TO_RIGHT Propionyl-CoA HMDB00208 HMDB 1.0 C00008 KEGG Compound C41H74N7O18P3S 2-Hydroxyphytanoyl-CoA 1077.4023 388444 ChemSpider C00007 KEGG Compound Q92968 UniProt 1.0 C19H38O2 Pristanic acid 298.28717 PROPIONYL-COA BioCyc CHEBI:18361 ChEBI C00002 KEGG Compound C20H40O2 Phytanic acid 312.30283 Pristanoyl-CoA 13131-49-2 CAS Adenosine diphosphate ReactionCatalysis1099 ACTIVATION HMDB00795 HMDB 13628282 ChemSpider 441263 PubChem-compound C00011 KEGG Compound C00010 KEGG Compound CHEBI:73024 ChEBI C00013 KEGG Compound 1 PubChem-compound 204120-61-6 CAS 26840 PubChem-compound 1.0 Phytanic acid BiologicalState59 CARNITINE BioCyc HMDB00201 HMDB FORMYL-COA BioCyc Transport79 false 4,8 Dimethylnonanoyl carnitine (→) Transport: Homo sapiens, Cell, Mitochondrial Intermembrane Space to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT Transport78 false L-Acetylcarnitine (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT 56-65-5 CAS Transport75 false 4,8 Dimethylnonanoyl carnitine (→) Transport: Homo sapiens, Cell, Peroxisome to Homo sapiens, Cell, Cytosol LEFT_TO_RIGHT C00026 KEGG Compound Transport74 false L-Acetylcarnitine (→) Transport: Homo sapiens, Cell, Peroxisome to Homo sapiens, Cell, Cytosol LEFT_TO_RIGHT CHEBI:16285 ChEBI Transport77 false Propionylcarnitine (→) Transport: Homo sapiens, Cell, Mitochondrial Intermembrane Space to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT C00024 KEGG Compound Transport76 false Phytanoyl-CoA (→) Transport: Homo sapiens, Cell, Peroxisome to Homo sapiens, Cell, Cytosol LEFT_TO_RIGHT 317-66-8 CAS 1.0 C02571 KEGG Compound Propionylcarnitine Phytanoyl-CoA Propionylcarnitine Carnitine O-acetyltransferase 4,8-Dimethylnonanoyl-CoA SubPathwayInput CL:0000000 CELL TYPE ONTOLOGY Propionylcarnitine Carnitine O-palmitoyltransferase 2, mitochondrial Long-chain-fatty-acid--CoA ligase 1 328-50-7 CAS Peroxisome SMILES OC(=O)CCC(O)=O 105373-75-9 CAS 1.0 C22H36N7O17P3S Formyl-CoA 795.1101 172787-73-4 CAS SMILES CC(C)CCCC(C)CCCC(C)CCC(=O)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 Beta Oxidation SubPathway SMILES [O-]P([O-])(=O)OP([O-])([O-])=O 1.0 C00042 KEGG Compound L-Acetylcarnitine SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O 2-HYDROXYPHYTANOYL-COA BioCyc 110458 ChemSpider HMDB00538 HMDB 1.0 C32H56N7O17P3S 4,8-Dimethylnonanoyl-CoA 935.2666 123929 PubChem-compound 1.0 SubPathwayInteraction213 SubPathwayReaction SubPathway213Reaction L-Carnitine SubPathwayInteraction214 SubPathway214Reaction SubPathwayReaction 1.0 4,8 Dimethylnonanoyl carnitine 25001 ChemSpider Propionyl-CoA SubPathwayOutput SubPathwayInteraction215 SubPathway215Reaction SubPathwayReaction 1.0 Pristanal 1.0 HMDB01958 HMDB O43772 UniProt C10H19NO4 Propionylcarnitine 217.13141 C10H16N5O13P3 Adenosine triphosphate 506.99576 SMILES C[N+](C)(C)C[C@H](O)CC([O-])=O Pristanoyl-CoA 1.0 Pyrophosphate HMDB01967 HMDB 1.0 Aldehyde dehydrogenase, mitochondrial 388712 ChemSpider ATP-binding cassette sub-family D 5800 ChemSpider 1.0 (-)o-acetylcarnitine BioCyc CO2 Carbon dioxide 43.98983 1.0 SMILES O=O Carnitine O-palmitoyltransferase 2, mitochondrial O75381 UniProt O14832 UniProt Coenzyme A BiologicalState5 BiologicalState3 1.0 2-hydroxyacyl-CoA lyase 1 58-64-0 CAS C18H35NO4 4,8 Dimethylnonanoyl carnitine 329.25662 1.0 1.0 644102 PubChem-compound 444493 PubChem-compound HMDB11604 HMDB 53477801 PubChem-compound 1.0 1.0 ACETYL-COA BioCyc SMILES CC(C)CCC[C@@H](C)CCC[C@@H](C)CCC[C@H](C)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 Propionylcarnitine HMDB00824 HMDB 1.0 Beta Oxidation SubPathway Beta Oxidation SubPathway Beta Oxidation SubPathway 977 PubChem-compound Phytanoyl-CoA C03017 KEGG Compound Peroxisomal membrane protein C19H38O Pristanal 282.29227 4,8 Dimethylnonanoyl carnitine 1.0 C02060 KEGG Compound HMDB00801 HMDB 1.0 53481003 PubChem-compound 4.0 1.0 Pristanoyl-CoA Acetyl-CoA SubPathwayOutput L-Acetylcarnitine SMILES CC(C)CCCC(C)CCCC(C)CCCC(C)C=O Peroxisome Membrane 6816 PubChem-compound 51 PubChem-compound Carbon dioxide ADP BioCyc C7H15NO3 L-Carnitine 161.1052 1.0 C9H17NO4 L-Acetylcarnitine 203.11575 17298-37-2 CAS ReactionCatalysis1104 ACTIVATION GO:0005829 GENE ONTOLOGY 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 274 ChemSpider 1.0 C5H6O5 Oxoglutaric acid 146.02153 1.0 ReactionCatalysis1101 ACTIVATION ReactionCatalysis1114 ACTIVATION 1189-37-3 CAS 3-OXOPRISTANOYL-COA BioCyc 1.0 Carnitine O-acetyltransferase SMILES CC(C)CCCC(C)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 Mitochondrion CHEBI:15522 ChEBI 1.0 ATP-binding cassette sub-family D member 1 2-hydroxyacyl-CoA lyase 1 ATP-binding cassette sub-family D member 2 Peroxisomal membrane protein PEX13 Peroxisomal membrane protein PEX14 541-15-1 CAS SMILES CC(=O)OC(CC([O-])=O)C[N+](C)(C)C 1.0 CPD-6641 BioCyc 4,8 Dimethylnonanoyl carnitine SMILES OC(=O)CCC(=O)C(O)=O 1.0 CHEBI:30915 ChEBI 124-38-9 CAS Adenosine triphosphate Carnitine O-palmitoyltransferase 2, mitochondrial C41H74N7O17P3S Phytanoyl-CoA 1061.4075 1.0 1.0 HMDB06202 HMDB P23786 UniProt 1.0 GO:0005758 GENE ONTOLOGY GO:0005759 GENE ONTOLOGY Long-chain-fatty-acid--CoA ligase 1 CHEBI:15741 ChEBI 1.0 SMILES O=C=O Mitochondrial carnitine/acylcarnitine carrier protein HMDB02057 HMDB 1110 PubChem-compound O7P2 Pyrophosphate 173.91193 5957 PubChem-compound L-Carnitine 1.0 C4H6O4 Succinic acid 118.02661 C40H70N7O18P3S Pristanoyl-CoA 1061.3711 CPD-206 BioCyc 1.0 SMILES CC(C)CCCC(C)CCC(=O)OC(CC([O-])=O)C[N+](C)(C)C P43155 UniProt GO:0005739 GENE ONTOLOGY C07343 KEGG Compound 1.0 C10H15N5O10P2 Adenosine diphosphate 427.02942 GO:0005737 GENE ONTOLOGY 439640 PubChem-compound 1.14.11.18 false 1.14.11.18 Oxoglutaric acid + Oxygen + Phytanoyl-CoA → 2-Hydroxyphytanoyl-CoA + Carbon dioxide + Succinic acid LEFT_TO_RIGHT 4.1.-.- false 4.1.-.- 2-Hydroxyphytanoyl-CoA → Formyl-CoA + Pristanal LEFT_TO_RIGHT P33121 UniProt Pristanoyl-CoA SubPathwayInput 6.2.1.3 false 6.2.1.3 Adenosine triphosphate + Coenzyme A + Phytanic acid → Adenosine diphosphate + Phytanoyl-CoA + Pyrophosphate LEFT_TO_RIGHT ReactionCatalysis432 ACTIVATION ReactionCatalysis433 ACTIVATION 21233922 ChemSpider 4.0 Phytanoyl-CoA dioxygenase, peroxisomal CHEBI:1146900 ChEBI 439313 PubChem-compound 390032 ChemSpider L-Acetylcarnitine 2-Hydroxyphytanoyl-CoA Mitochondrial Matrix 96904 ChemSpider CHEBI:63874 ChEBI Phytanoyl-CoA 14000-31-8 CAS 1.0 CO-A BioCyc P05091 UniProt 1078 ChemSpider 72-89-9 CAS Oxygen C21H36N7O16P3S Coenzyme A 767.11523 10917 PubChem-compound Cytoplasm 10455 ChemSpider Cell CHEBI:15422 ChEBI Pristanic acid Coenzyme A 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 TransportCatalysis39 ACTIVATION TransportCatalysis38 ACTIVATION TransportCatalysis37 ACTIVATION O2 Oxygen 31.98983 CHEBI:28867 ChEBI CHEBI:15539 ChEBI CHEBI:15538 ChEBI ReactionCatalysis1066 ACTIVATION 870-77-9 CAS ReactionCatalysis1070 ACTIVATION 390025 ChemSpider CHEBI:16526 ChEBI 1.0 TransportCatalysis44 ACTIVATION TransportCatalysis43 ACTIVATION HMDB01295 HMDB CHEBI:16761 ChEBI 7782-44-7 CAS TransportCatalysis42 ACTIVATION TransportCatalysis41 ACTIVATION GO:0005778 GENE ONTOLOGY TransportCatalysis40 ACTIVATION GO:0005777 GENE ONTOLOGY 4,8 Dimethylnonanoyl carnitine 9606 TAXONOMY Coenzyme A 14721-66-5 CAS Succinic acid Oxoglutaric acid C01607 KEGG Compound 2-KETOGLUTARATE BioCyc Q9UBJ2 UniProt 392413 ChemSpider 4,8 Dimethylnonanoyl carnitine 110-15-6 CAS 14671060 PubChem-compound 1.0 Mitochondrial Intermembrane Space 1.0 CHEBI:15379 ChEBI CHEBI:16347 ChEBI 107738 PubChem-compound 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 HMDB00062 HMDB HMDB01275 HMDB SMILES CCC(=O)OC(CC([O-])=O)C[N+](C)(C)C 280 PubChem-compound P33897 UniProt 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 6.2.1.3 false 6.2.1.3 Adenosine triphosphate + Coenzyme A + Pristanic acid → Adenosine diphosphate + Pristanoyl-CoA + Pyrophosphate LEFT_TO_RIGHT 21243783 ChemSpider 146622-45-9 CAS C07297 KEGG Compound HMDB03419 HMDB 1.2.1.3 false 1.2.1.3 Coenzyme A + Pristanal → Pristanoyl-CoA LEFT_TO_RIGHT CHEBI:28542 ChEBI HMDB01377 HMDB CHEBI:15475 ChEBI 1.0 SMILES CC(C)CCCC(C)CCCC(C)CCCC(C)C(O)=O Peroxisomal membrane protein PEX14 ATP-binding cassette sub-family D member 1 Q9UJ83 UniProt Peroxisomal membrane protein PEX13 CHEBI:15351 ChEBI SMILES CC(C)CCCC(C)CCCC(C)CCCC(C)CC(O)=O ATP-binding cassette sub-family D member 2 SMILES CC(C)CCC[C@@H](C)CCC[C@@H](C)CCC[C@H](C)C(O)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 1.0 85-61-0 CAS Peroxisomal membrane protein PEX13 Peroxisomal membrane protein PEX14 Peroxisomal membrane protein PEX13 Peroxisomal membrane protein PEX14 Peroxisomal membrane protein PEX13 Peroxisomal membrane protein PEX14 Peroxisomal membrane protein PEX13 Peroxisomal membrane protein PEX14 ATP-binding cassette sub-family D member 1 ATP-binding cassette sub-family D member 2 Mitochondrial carnitine/acylcarnitine carrier protein Mitochondrial carnitine/acylcarnitine carrier protein Mitochondrial carnitine/acylcarnitine carrier protein Long-chain- fatty-acid-- CoA ligase 1 Aldehyde dehydrogenase, mitochondrial 2- hydroxyacyl- CoA lyase 1 Phytanoyl-CoA dioxygenase, peroxisomal Carnitine O- acetyltransferase Peroxisomal carnitine O- octanoyltransferase Carnitine O- acetyltransferase Long-chain- fatty-acid-- CoA ligase 1 Carnitine O- acetyltransferase Carnitine O- acetyltransferase Carnitine O- palmitoyltransferase 2, mitochondrial Pristanoyl-CoA Propionyl-CoA Acetyl-CoA 4,8-Dimethylnonanoyl-CoA 4,8-Dimethylnonanoyl-CoA Acetyl-CoA Pristanoyl-CoA Propionylcarnitine Propionylcarnitine L- Acetylcarnitine L- Acetylcarnitine 4,8 Dimethylnonanoyl carnitine 4,8 Dimethylnonanoyl carnitine Phytanoyl-CoA Phytanoyl-CoA Propionylcarnitine L- Acetylcarnitine 4,8 Dimethylnonanoyl carnitine ATP Pristanic acid CoA ADP PP i Pristanal CoA 2-Hydroxyphytanoyl-CoA Formyl-CoA Oxoglutaric acid O 2 Succinic acid CO 2 L-Carnitine CoA L-Carnitine CoA CoA Phytanic acid ATP CoA ADP PP i Propionyl-CoA L-Carnitine CoA Acetyl-CoA CoA L-Carnitine CoA Magnesium Magnesium Thiamine pyrophosphate Iron Ascorbic acid Magnesium Beta Oxidation Beta Oxidation Beta Oxidation Beta Oxidation Peroxisome Mitochondria Mitochondrial Inner Membrane Mitochondrial Outer Membrane Intracellular Space Extracellular Space
HMDB00254 HMDB 6022 PubChem-compound C00798 KEGG Compound Propionylcarnitine C23H38N7O17P3S Acetyl-CoA 809.1258 C00318 KEGG Compound 4,8-Dimethylnonanoyl-CoA SubPathwayOutput HMDB00250 HMDB HMDB01341 HMDB CHEBI:49189 ChEBI Phytanoyl-CoA dioxygenase, peroxisomal Pristanal 50 ChemSpider 2.3.1.7 false 2.3.1.7 L-Carnitine + Propionyl-CoA &harr; Coenzyme A + Propionylcarnitine REVERSIBLE 1.0 HMDB01359 HMDB Oxidation of Branched Chain Fatty Acids 441253 PubChem-compound ATP BioCyc Mitochondrial carnitine/acylcarnitine carrier protein 559142 ChemSpider http://identifiers.org/smpdb/SMP00030 SMPDB 1.0 HMDB01206 HMDB 5742 ChemSpider 1.0 Acetyl-CoA SubPathwayOutput 952 ChemSpider Aldehyde dehydrogenase, mitochondrial PW000155 PathWhiz 1.0 C00100 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 2.3.1.21 false 2.3.1.21 4,8-Dimethylnonanoyl-CoA + L-Carnitine &harr; 4,8 Dimethylnonanoyl carnitine + Coenzyme A REVERSIBLE 2.3.1.7 false 2.3.1.7 Acetyl-CoA + L-Carnitine &harr; Coenzyme A + L-Acetylcarnitine REVERSIBLE 439164 PubChem-compound L-Acetylcarnitine PPI BioCyc Aldehyde dehydrogenase, mitochondrial SubPathwayInteraction236 SubPathwayReaction SubPathway236Reaction HMDB01423 HMDB C24H40N7O17P3S Propionyl-CoA 823.1414 2-hydroxyacyl-CoA lyase 1 Cytosol Formyl-CoA Carnitine O-acetyltransferase 388310 ChemSpider SUC BioCyc Transport71 false Pristanoyl-CoA (&rarr;) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Peroxisome LEFT_TO_RIGHT CHEBI:51340 ChEBI Transport73 false Propionylcarnitine (&rarr;) Transport: Homo sapiens, Cell, Peroxisome to Homo sapiens, Cell, Cytosol LEFT_TO_RIGHT Propionyl-CoA HMDB00208 HMDB 1.0 C00008 KEGG Compound C41H74N7O18P3S 2-Hydroxyphytanoyl-CoA 1077.4023 388444 ChemSpider C00007 KEGG Compound Q92968 UniProt 1.0 C19H38O2 Pristanic acid 298.28717 PROPIONYL-COA BioCyc CHEBI:18361 ChEBI C00002 KEGG Compound C20H40O2 Phytanic acid 312.30283 Pristanoyl-CoA 13131-49-2 CAS Adenosine diphosphate ReactionCatalysis1099 ACTIVATION HMDB00795 HMDB 13628282 ChemSpider 441263 PubChem-compound C00011 KEGG Compound C00010 KEGG Compound CHEBI:73024 ChEBI C00013 KEGG Compound 1 PubChem-compound 204120-61-6 CAS 26840 PubChem-compound 1.0 Phytanic acid BiologicalState59 Homo sapiens, Cell, Peroxisome Membrane CARNITINE BioCyc HMDB00201 HMDB FORMYL-COA BioCyc Transport79 false 4,8 Dimethylnonanoyl carnitine (&rarr;) Transport: Homo sapiens, Cell, Mitochondrial Intermembrane Space to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT Transport78 false L-Acetylcarnitine (&rarr;) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT 56-65-5 CAS Transport75 false 4,8 Dimethylnonanoyl carnitine (&rarr;) Transport: Homo sapiens, Cell, Peroxisome to Homo sapiens, Cell, Cytosol LEFT_TO_RIGHT C00026 KEGG Compound Transport74 false L-Acetylcarnitine (&rarr;) Transport: Homo sapiens, Cell, Peroxisome to Homo sapiens, Cell, Cytosol LEFT_TO_RIGHT CHEBI:16285 ChEBI Transport77 false Propionylcarnitine (&rarr;) Transport: Homo sapiens, Cell, Mitochondrial Intermembrane Space to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT C00024 KEGG Compound Transport76 false Phytanoyl-CoA (&rarr;) Transport: Homo sapiens, Cell, Peroxisome to Homo sapiens, Cell, Cytosol LEFT_TO_RIGHT 317-66-8 CAS 1.0 C02571 KEGG Compound Propionylcarnitine Phytanoyl-CoA Propionylcarnitine Carnitine O-acetyltransferase 4,8-Dimethylnonanoyl-CoA SubPathwayInput CL:0000000 CELL TYPE ONTOLOGY Propionylcarnitine Carnitine O-palmitoyltransferase 2, mitochondrial Long-chain-fatty-acid--CoA ligase 1 328-50-7 CAS Peroxisome SMILES OC(=O)CCC(O)=O 105373-75-9 CAS 1.0 C22H36N7O17P3S Formyl-CoA 795.1101 172787-73-4 CAS SMILES CC(C)CCCC(C)CCCC(C)CCC(=O)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 Beta Oxidation SubPathway SMILES [O-]P([O-])(=O)OP([O-])([O-])=O 1.0 C00042 KEGG Compound L-Acetylcarnitine SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O 2-HYDROXYPHYTANOYL-COA BioCyc 110458 ChemSpider HMDB00538 HMDB 1.0 C32H56N7O17P3S 4,8-Dimethylnonanoyl-CoA 935.2666 123929 PubChem-compound 1.0 SubPathwayInteraction213 SubPathwayReaction SubPathway213Reaction L-Carnitine SubPathwayInteraction214 SubPathway214Reaction SubPathwayReaction 1.0 4,8 Dimethylnonanoyl carnitine 25001 ChemSpider Propionyl-CoA SubPathwayOutput SubPathwayInteraction215 SubPathway215Reaction SubPathwayReaction 1.0 Pristanal 1.0 HMDB01958 HMDB O43772 UniProt C10H19NO4 Propionylcarnitine 217.13141 C10H16N5O13P3 Adenosine triphosphate 506.99576 SMILES C[N+](C)(C)C[C@H](O)CC([O-])=O Pristanoyl-CoA 1.0 Pyrophosphate HMDB01967 HMDB 1.0 Aldehyde dehydrogenase, mitochondrial 388712 ChemSpider ATP-binding cassette sub-family D 5800 ChemSpider 1.0 (-)o-acetylcarnitine BioCyc CO2 Carbon dioxide 43.98983 1.0 SMILES O=O Carnitine O-palmitoyltransferase 2, mitochondrial O75381 UniProt O14832 UniProt Coenzyme A BiologicalState5 Homo sapiens, Cell, Peroxisome BiologicalState3 Homo sapiens, Cell, Mitochondrion 1.0 2-hydroxyacyl-CoA lyase 1 58-64-0 CAS C18H35NO4 4,8 Dimethylnonanoyl carnitine 329.25662 1.0 1.0 644102 PubChem-compound 444493 PubChem-compound HMDB11604 HMDB 53477801 PubChem-compound 1.0 1.0 ACETYL-COA BioCyc SMILES CC(C)CCC[C@@H](C)CCC[C@@H](C)CCC[C@H](C)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 Propionylcarnitine HMDB00824 HMDB 1.0 Beta Oxidation SubPathway Beta Oxidation SubPathway Beta Oxidation SubPathway 977 PubChem-compound Phytanoyl-CoA C03017 KEGG Compound Peroxisomal membrane protein C19H38O Pristanal 282.29227 4,8 Dimethylnonanoyl carnitine 1.0 C02060 KEGG Compound HMDB00801 HMDB 1.0 53481003 PubChem-compound 4.0 1.0 Pristanoyl-CoA Acetyl-CoA SubPathwayOutput L-Acetylcarnitine SMILES CC(C)CCCC(C)CCCC(C)CCCC(C)C=O Peroxisome Membrane 6816 PubChem-compound 51 PubChem-compound Carbon dioxide ADP BioCyc C7H15NO3 L-Carnitine 161.1052 1.0 C9H17NO4 L-Acetylcarnitine 203.11575 17298-37-2 CAS ReactionCatalysis1104 ACTIVATION GO:0005829 GENE ONTOLOGY 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 274 ChemSpider 1.0 C5H6O5 Oxoglutaric acid 146.02153 1.0 ReactionCatalysis1101 ACTIVATION ReactionCatalysis1114 ACTIVATION 1189-37-3 CAS 3-OXOPRISTANOYL-COA BioCyc 1.0 Carnitine O-acetyltransferase SMILES CC(C)CCCC(C)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 Mitochondrion CHEBI:15522 ChEBI 1.0 ATP-binding cassette sub-family D member 1 2-hydroxyacyl-CoA lyase 1 ATP-binding cassette sub-family D member 2 Peroxisomal membrane protein PEX13 Peroxisomal membrane protein PEX14 541-15-1 CAS SMILES CC(=O)OC(CC([O-])=O)C[N+](C)(C)C 1.0 CPD-6641 BioCyc 4,8 Dimethylnonanoyl carnitine SMILES OC(=O)CCC(=O)C(O)=O 1.0 CHEBI:30915 ChEBI 124-38-9 CAS Adenosine triphosphate Carnitine O-palmitoyltransferase 2, mitochondrial C41H74N7O17P3S Phytanoyl-CoA 1061.4075 1.0 1.0 HMDB06202 HMDB P23786 UniProt 1.0 GO:0005758 GENE ONTOLOGY GO:0005759 GENE ONTOLOGY Long-chain-fatty-acid--CoA ligase 1 CHEBI:15741 ChEBI 1.0 SMILES O=C=O Mitochondrial carnitine/acylcarnitine carrier protein HMDB02057 HMDB 1110 PubChem-compound O7P2 Pyrophosphate 173.91193 5957 PubChem-compound L-Carnitine 1.0 C4H6O4 Succinic acid 118.02661 C40H70N7O18P3S Pristanoyl-CoA 1061.3711 CPD-206 BioCyc 1.0 SMILES CC(C)CCCC(C)CCC(=O)OC(CC([O-])=O)C[N+](C)(C)C P43155 UniProt GO:0005739 GENE ONTOLOGY C07343 KEGG Compound 1.0 C10H15N5O10P2 Adenosine diphosphate 427.02942 GO:0005737 GENE ONTOLOGY 439640 PubChem-compound 1.14.11.18 false 1.14.11.18 Oxoglutaric acid + Oxygen + Phytanoyl-CoA &rarr; 2-Hydroxyphytanoyl-CoA + Carbon dioxide + Succinic acid LEFT_TO_RIGHT 4.1.-.- false 4.1.-.- 2-Hydroxyphytanoyl-CoA &rarr; Formyl-CoA + Pristanal LEFT_TO_RIGHT P33121 UniProt Pristanoyl-CoA SubPathwayInput 6.2.1.3 false 6.2.1.3 Adenosine triphosphate + Coenzyme A + Phytanic acid &rarr; Adenosine diphosphate + Phytanoyl-CoA + Pyrophosphate LEFT_TO_RIGHT ReactionCatalysis432 ACTIVATION ReactionCatalysis433 ACTIVATION 21233922 ChemSpider 4.0 Phytanoyl-CoA dioxygenase, peroxisomal CHEBI:1146900 ChEBI 439313 PubChem-compound 390032 ChemSpider L-Acetylcarnitine 2-Hydroxyphytanoyl-CoA Mitochondrial Matrix 96904 ChemSpider CHEBI:63874 ChEBI Phytanoyl-CoA 14000-31-8 CAS 1.0 CO-A BioCyc P05091 UniProt 1078 ChemSpider 72-89-9 CAS Oxygen C21H36N7O16P3S Coenzyme A 767.11523 10917 PubChem-compound Cytoplasm 10455 ChemSpider Cell CHEBI:15422 ChEBI Pristanic acid Coenzyme A 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 TransportCatalysis39 ACTIVATION TransportCatalysis38 ACTIVATION TransportCatalysis37 ACTIVATION O2 Oxygen 31.98983 CHEBI:28867 ChEBI CHEBI:15539 ChEBI CHEBI:15538 ChEBI ReactionCatalysis1066 ACTIVATION 870-77-9 CAS ReactionCatalysis1070 ACTIVATION 390025 ChemSpider CHEBI:16526 ChEBI 1.0 TransportCatalysis44 ACTIVATION TransportCatalysis43 ACTIVATION HMDB01295 HMDB CHEBI:16761 ChEBI 7782-44-7 CAS TransportCatalysis42 ACTIVATION TransportCatalysis41 ACTIVATION GO:0005778 GENE ONTOLOGY TransportCatalysis40 ACTIVATION GO:0005777 GENE ONTOLOGY 4,8 Dimethylnonanoyl carnitine 9606 TAXONOMY Coenzyme A 14721-66-5 CAS Succinic acid Oxoglutaric acid C01607 KEGG Compound 2-KETOGLUTARATE BioCyc Q9UBJ2 UniProt 392413 ChemSpider 4,8 Dimethylnonanoyl carnitine 110-15-6 CAS 14671060 PubChem-compound 1.0 Mitochondrial Intermembrane Space 1.0 CHEBI:15379 ChEBI CHEBI:16347 ChEBI 107738 PubChem-compound 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 HMDB00062 HMDB HMDB01275 HMDB SMILES CCC(=O)OC(CC([O-])=O)C[N+](C)(C)C 280 PubChem-compound P33897 UniProt 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 6.2.1.3 false 6.2.1.3 Adenosine triphosphate + Coenzyme A + Pristanic acid &rarr; Adenosine diphosphate + Pristanoyl-CoA + Pyrophosphate LEFT_TO_RIGHT 21243783 ChemSpider 146622-45-9 CAS C07297 KEGG Compound HMDB03419 HMDB 1.2.1.3 false 1.2.1.3 Coenzyme A + Pristanal &rarr; Pristanoyl-CoA LEFT_TO_RIGHT CHEBI:28542 ChEBI HMDB01377 HMDB CHEBI:15475 ChEBI 1.0 SMILES CC(C)CCCC(C)CCCC(C)CCCC(C)C(O)=O Peroxisomal membrane protein PEX14 ATP-binding cassette sub-family D member 1 Q9UJ83 UniProt Peroxisomal membrane protein PEX13 CHEBI:15351 ChEBI SMILES CC(C)CCCC(C)CCCC(C)CCCC(C)CC(O)=O ATP-binding cassette sub-family D member 2 SMILES CC(C)CCC[C@@H](C)CCC[C@@H](C)CCC[C@H](C)C(O)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 1.0 85-61-0 CAS <