6022
PubChem-compound
C00798
KEGG Compound
Propionylcarnitine
C23H38N7O17P3S
Acetyl-CoA
809.1258
C00318
KEGG Compound
4,8-Dimethylnonanoyl-CoA
SubPathwayOutput
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
441253
PubChem-compound
ATP
BioCyc
Mitochondrial carnitine/acylcarnitine carrier protein
559142
ChemSpider
1.0
5742
ChemSpider
1.0
Acetyl-CoA
SubPathwayOutput
952
ChemSpider
Aldehyde dehydrogenase, mitochondrial
HMDB0002057
HMDB
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@@H](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
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
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
HMDB0006202
HMDB
ReactionCatalysis1099
ACTIVATION
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
CARNITINE
BioCyc
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
Propionylcarnitine
Carnitine O-palmitoyltransferase 2, mitochondrial
Long-chain-fatty-acid--CoA ligase 1
328-50-7
CAS
HMDB0000254
HMDB
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)CCCC(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
HMDB0000250
HMDB
Beta Oxidation
SubPathway
SMILES
OP(O)(=O)OP(O)(O)=O
HMDB0001341
HMDB
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
HMDB0001359
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
HMDB0003419
HMDB
1.0
Pristanal
1.0
O43772
UniProt
C10H19NO4
Propionylcarnitine
217.13141
C10H16N5O13P3
Adenosine triphosphate
506.99576
HMDB0001377
HMDB
SMILES
C[N+](C)(C)C[C@H](O)CC([O-])=O
Pristanoyl-CoA
1.0
Pyrophosphate
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
1.0
2-hydroxyacyl-CoA lyase 1
58-64-0
CAS
C18H35NO4
4,8 Dimethylnonanoyl carnitine
329.25662
HMDB0000062
HMDB
HMDB0001275
HMDB
1.0
1.0
644102
PubChem-compound
444493
PubChem-compound
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
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
HMDB0001295
HMDB
4,8 Dimethylnonanoyl carnitine
1.0
Oxidation of Branched-Chain Fatty Acids
C02060
KEGG Compound
1.0
53481003
PubChem-compound
4.0
1.0
Pristanoyl-CoA
HMDB0000538
HMDB
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
HMDB0000795
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
274
ChemSpider
1.0
C5H6O5
Oxoglutaric acid
146.02153
1.0
ReactionCatalysis1101
ACTIVATION
3040-38-8
CAS
ReactionCatalysis1114
ACTIVATION
HMDB0000201
HMDB
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
HMDB0000208
HMDB
SMILES
CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C
1.0
CPD-6641
BioCyc
4,8 Dimethylnonanoyl carnitine
HMDB0001423
HMDB
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
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
1110
PubChem-compound
H4O7P2
Pyrophosphate
177.94322
5957
PubChem-compound
HMDB0001206
HMDB
L-Carnitine
1.0
C4H6O4
Succinic acid
118.02661
C40H72N7O17P3S
Pristanoyl-CoA
1047.3918
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
HMDB0000801
HMDB
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
CHEBI:15422
ChEBI
Pristanic acid
Coenzyme A
6557
ChemSpider
1.0
Homo sapiens
HMDB0000824
HMDB
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
ReactionCatalysis1070
ACTIVATION
390025
ChemSpider
CHEBI:16526
ChEBI
1.0
TransportCatalysis44
ACTIVATION
TransportCatalysis43
ACTIVATION
CHEBI:16761
ChEBI
7782-44-7
CAS
TransportCatalysis42
ACTIVATION
TransportCatalysis41
ACTIVATION
GO:0005778
GENE ONTOLOGY
HMDB0011604
HMDB
TransportCatalysis40
ACTIVATION
GO:0005777
GENE ONTOLOGY
4,8 Dimethylnonanoyl carnitine
SMP0000030
SMPDB
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
SMILES
CCC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C
280
PubChem-compound
P33897
UniProt
HMDB0001958
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
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
1.2.1.3
false
1.2.1.3
Coenzyme A + Pristanal → Pristanoyl-CoA
LEFT_TO_RIGHT
CHEBI:28542
ChEBI
CHEBI:15475
ChEBI
1.0
SMILES
CC(C)CCCC(C)CCCC(C)CCCC(C)C(O)=O
Peroxisomal membrane protein PEX14
HMDB0001967
HMDB
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