Mitochondrial beta-oxidation (WP5241)

Homo sapiens

Human mitochondrial beta-oxidation

Authors

Conroy lipids , Egon Willighagen , Denise Slenter , and Eric Weitz

Activity

last edited

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Cited In

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Organisms

Homo sapiens

Communities

Annotations

Pathway Ontology

lipid metabolic pathway classic metabolic pathway fatty acid beta degradation pathway

Participants

Label Type Compact URI Comment
Stearoyl-CoA Metabolite lipidmaps:LMFA07050369
2E-Octadecenoyl-CoA Metabolite lipidmaps:LMFA07050385
3S-hydroxystearoyl-CoA Metabolite lipidmaps:LMFA07050159
3-oxostearoyl-CoA Metabolite lipidmaps:LMFA07050248
Palmitoyl-CoA Metabolite lipidmaps:LMFA07050360
2E-hexadecenoyl-CoA Metabolite lipidmaps:LMFA07050144
3S-hydroxypalmitoyl-CoA Metabolite lipidmaps:LMFA07050161
3-oxopalmitoyl-CoA Metabolite lipidmaps:LMFA07050250
myristoyl-CoA Metabolite lipidmaps:LMFA07050352
2E-tetradecenoyl-CoA Metabolite lipidmaps:LMFA07050396
3S-Hydroxymyristoyl-CoA Metabolite lipidmaps:LMFA07050257
3-oxo-myristoyl-CoA Metabolite lipidmaps:LMFA07050256
Lauroyl-CoA Metabolite lipidmaps:LMFA07050340
2E-Dodecenoyl-CoA Metabolite lipidmaps:LMFA07050176
3S-hydroxylauroyl-CoA Metabolite lipidmaps:LMFA07050158
3-oxo-lauroyl-CoA Metabolite lipidmaps:LMFA07050013
Decanoyl-CoA Metabolite lipidmaps:LMFA07050313
2E-Decenoyl-CoA Metabolite lipidmaps:LMFA07050391
3S-hydroxy-decanoyl-CoA Metabolite lipidmaps:LMFA07050403
3-oxo-decanoyl-CoA Metabolite lipidmaps:LMFA07050239
Octanoyl-CoA Metabolite lipidmaps:LMFA07050355
2E-octanoyl-CoA Metabolite lipidmaps:LMFA07050394
3S-hydroxy-octanoyl-CoA Metabolite lipidmaps:LMFA07050160
3-oxo-octanoyl-CoA Metabolite lipidmaps:LMFA07050249
Hexanoyl-CoA Metabolite lipidmaps:LMFA07050328
2E-hexenoyl-CoA Metabolite lipidmaps:LMFA07050393
3S-hydroxyhexanoyl-CoA Metabolite lipidmaps:LMFA07050156
3-Oxohexanoyl-CoA Metabolite lipidmaps:LMFA07050242
Butyryl-CoA Metabolite lipidmaps:LMFA07050292
2E-butanoyl-CoA Metabolite lipidmaps:LMFA07050307
3S-hydroxybutanoyl-CoA Metabolite lipidmaps:LMFA07050153
3-oxo-butanoyl-CoA Metabolite lipidmaps:LMFA07050030
Acetyl-CoA Metabolite lipidmaps:LMFA07050281
oleoyl-CoA Metabolite lipidmaps:LMFA07050356
2E,9Z-octadecadienoyl-CoA Metabolite lipidmaps:LMFA07050414
3S-Hydroxy-oleoyl-CoA Metabolite lipidmaps:LMFA07050415
3-oxo-oleoyl-CoA Metabolite lipidmaps:LMFA07050416
7Z-hexadecenoyl-CoA Metabolite lipidmaps:LMFA07050417
2E,7Z-hexadacadienoyl-CoA Metabolite lipidmaps:LMFA07050418
3S-Hydroxy-7Z-hexadecenoyl-CoA Metabolite lipidmaps:LMFA07050419
3-oxo-7Z-hexadecenoyl-CoA Metabolite lipidmaps:LMFA07050420
5Z-tetradecenoyl-CoA Metabolite lipidmaps:LMFA07050305
2E,5Z-tetradecadienoyl-CoA Metabolite lipidmaps:LMFA07050421
3S-hydroxy-5Z-tetradecadienoyl-CoA Metabolite lipidmaps:LMFA07050422
3-oxo-5Z-tetradecenoyl-CoA Metabolite lipidmaps:LMFA07050423
3Z-dodecenoyl-CoA Metabolite lipidmaps:LMFA07050302
Linoleoyl-CoA Metabolite lipidmaps:LMFA07050343
(2E,9Z,12Z)-octadecatrienoyl-CoA Metabolite lipidmaps:LMFA07050404
3S-hydroxy-9Z,12Z-octadecadienoyl-CoA Metabolite lipidmaps:LMFA07050405
(9Z,12Z)-3-oxolinoleoyl-CoA Metabolite lipidmaps:LMFA07050136
7Z,10Z-hexadecadienoyl-CoA Metabolite lipidmaps:LMFA07050406
2E,7Z,10Z-hexadecatrienoyl-CoA Metabolite lipidmaps:LMFA07050429
3S-hydroxy-7Z,10Z-hexadecadienoyl-CoA Metabolite lipidmaps:LMFA07050439
3-oxo-7Z,10Z-hexadecadienoyl-CoA Metabolite lipidmaps:LMFA07050440
5Z,8Z-tetradecadienoyl-CoA Metabolite lipidmaps:LMFA07050296
2E,5Z,8Z-tetradecatrienoyl-CoA Metabolite lipidmaps:LMFA07050468
3S-hydroxy-5Z,8Z-tetradecadienoyl-CoA Metabolite lipidmaps:LMFA07050469
3-oxo-5Z,8Z-tetradecadienoyl-CoA Metabolite lipidmaps:LMFA07050470
3Z,6Z-dodecadienoyl-CoA Metabolite lipidmaps:LMFA07050295
2E,6Z-dodecadienoyl-CoA Metabolite lipidmaps:LMFA07050111
3S-Hydroxy-6Z-dodecenoyl-CoA Metabolite lipidmaps:LMFA07050471
3-oxo-6Z-dodecenoyl-CoA Metabolite lipidmaps:LMFA07050472
4Z-decenoyl-CoA Metabolite lipidmaps:LMFA07050301
2E,4Z-decadienoyl-CoA Metabolite lipidmaps:LMFA07050110
3E-decenoyl-CoA Metabolite lipidmaps:LMFA07050386
Arachidonyl-CoA Metabolite lipidmaps:LMFA07050288
2E,5Z,8Z,11Z,14Z-eicosapentaenoyl-CoA Metabolite lipidmaps:LMFA07050410
3S-Hydroxy-arachidonoyl-CoA Metabolite lipidmaps:LMFA07050411
3-oxo-arachidonoyl-CoA Metabolite lipidmaps:LMFA07050412
3Z,6Z,9Z,12Z-octadecatetraenoyl-CoA Metabolite lipidmaps:LMFA07050413
4Z,7Z,10Z-hexadecatrienoyl-CoA Metabolite lipidmaps:LMFA07050438
2E,4Z,7Z,10Z-hexadecatetraenoyl-CoA Metabolite lipidmaps:LMFA07050484
3E,7Z,10Z-hexadecatrienoyl-CoA Metabolite lipidmaps:LMFA07050485
9Z,12Z,15Z-octadecatrienoyl-CoA Metabolite lipidmaps:LMFA07050286
2E,9Z,12Z,15Z-octadecatetraenoyl-CoA Metabolite lipidmaps:LMFA07050424
3S-hydroxy-9Z,12Z,15Z-octadecatrienoyl-CoA Metabolite lipidmaps:LMFA07050425
3-oxo-9Z,12Z,15Z-octadecatrienoyl-CoA Metabolite lipidmaps:LMFA07050426
7Z,10Z,13Z-hexadecatrienoyl-CoA Metabolite lipidmaps:LMFA07050427
2E,7Z,10Z,13Z-hexadecatetraenoyl-CoA Metabolite lipidmaps:LMFA07050428
3S-Hydroxy-7Z,10Z,13Z-hexadecatrienoyl-CoA Metabolite lipidmaps:LMFA07050455
3oxo-7Z,10Z,13Z-hexadecatrienoyl-CoA Metabolite lipidmaps:LMFA07050456
5Z,8Z,11Z-tetradecatrienoyl-CoA Metabolite lipidmaps:LMFA07050457
2E,5Z,8Z,11Z-tetradecatetraenoyl-CoA Metabolite lipidmaps:LMFA07050458
3S-Hydroxy-5Z,8Z,11Z-tetradecatrienoyl-CoA Metabolite lipidmaps:LMFA07050459
3-oxo-5Z,8Z,11Z-tetradecatrienoyl-CoA Metabolite lipidmaps:LMFA07050460
3Z,6Z,9Z-dodecatrienoyl-CoA Metabolite lipidmaps:LMFA07050461
2E,6Z,9Z-dodecatrienoyl-CoA Metabolite lipidmaps:LMFA07050462
3S-Hydroxy-dodecadienoyl-CoA Metabolite lipidmaps:LMFA07050463
3-oxo-dodecadienoyl-CoA Metabolite lipidmaps:LMFA07050464
4Z,7Z-decadienoyl-CoA Metabolite lipidmaps:LMFA07050465
2E,4Z,7Z-decatrienoyl-CoA Metabolite lipidmaps:LMFA07050466
3E,7Z-decadienoyl-CoA Metabolite lipidmaps:LMFA07050467
2E,7Z-decadienoyl-CoA Metabolite lipidmaps:LMFA07050437
3S-Hydroxy-7Z-decenoyl-CoA Metabolite lipidmaps:LMFA07050436
3-oxo-7Z-decenoyl-CoA Metabolite lipidmaps:LMFA07050435
5Z-octenoyl-CoA Metabolite lipidmaps:LMFA07050434
2E,5Z-octadienoyl-CoA Metabolite lipidmaps:LMFA07050433
3S-Hydroxy-5Z-octenoyl-CoA Metabolite lipidmaps:LMFA07050432
3-oxo-5Z-octenoyl-CoA Metabolite lipidmaps:LMFA07050431
3Z-hexenoyl-CoA Metabolite lipidmaps:LMFA07050430
gamma-linolenoyl-CoA Metabolite lipidmaps:LMFA07050322
2E,6Z,9Z,12Z-octadecatetraenoyl-CoA Metabolite lipidmaps:LMFA07050407
3S-hydroxy-gamma-linoleoyl-CoA Metabolite lipidmaps:LMFA07050408
3-oxo-gamma-linoleoyl-CoA Metabolite lipidmaps:LMFA07050409
9-palmitoleoyl-CoA Metabolite lipidmaps:LMFA07050359
2E-9Z-hexadecadienoyl-CoA Metabolite lipidmaps:LMFA07050473
3S-Hydroxy-9Z-hexadecenoyl-CoA Metabolite lipidmaps:LMFA07050474
3-oxo-9Z-hexadecenoyl-CoA Metabolite lipidmaps:LMFA07050475
7Z-tetradecenoyl-CoA Metabolite lipidmaps:LMFA07050476
2E,7Z-tetradecadienoyl-CoA Metabolite lipidmaps:LMFA07050477
3S-hydroxy-7Z-tetradecadienoyl-CoA Metabolite lipidmaps:LMFA07050478
3-oxo-7Z-tetradecadienoyl-CoA Metabolite lipidmaps:LMFA07050479
5Z-dodecenoyl-CoA Metabolite lipidmaps:LMFA07050480
2E,5Z-dodecadienoyl-CoA Metabolite lipidmaps:LMFA07050375
3S-Hydroxy-5Z-dodecenoyl-CoA Metabolite lipidmaps:LMFA07050481
3-oxo-5Z-dodecenoyl-CoA Metabolite lipidmaps:LMFA07050482
3Z-decenoyl-CoA Metabolite lipidmaps:LMFA07050483
docosahexaenoyl-CoA Metabolite lipidmaps:LMFA07050121
2E,4E,7Z,10Z,13Z,16Z,19Z-Docosaheptaenoyl-CoA Metabolite lipidmaps:LMFA07050171
3E,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl-CoA Metabolite lipidmaps:LMFA07050442
2E,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl-CoA Metabolite lipidmaps:LMFA07050443
3S-hydroxy-7Z,10Z,13Z,16Z,19Z-docosapentaenoyl-CoA Metabolite lipidmaps:LMFA07050444
3-oxo-7Z,10Z,13Z,16Z,19Z-docosapentaenoyl-CoA Metabolite lipidmaps:LMFA07050130
5Z,8Z,11Z,14Z,17Z-eicosapentaenoyl-CoA Metabolite lipidmaps:LMFA07050122
2E,5Z,8Z,11Z,14Z,17Z-eicosahexaenoyl-CoA Metabolite lipidmaps:LMFA07050445
3S-Hydroxy-5Z,8Z,11Z,14Z,17Z-eicosapentaenoyl-CoA Metabolite lipidmaps:LMFA07050446
3-oxo-Hydroxy-5Z,8Z,11Z,14Z,17Z-eicosapentaenoyl-CoA Metabolite lipidmaps:LMFA07050447
3Z,6Z,9Z,12Z,15Z-octadecapentaenoyl-CoA Metabolite lipidmaps:LMFA07050448
2E,6Z,9Z,12Z,15Z-octadecapentaenoyl-CoA Metabolite lipidmaps:LMFA07050449
3S-Hydroxy-6Z,9Z,12Z,15Z-octadecatetraenoyl-CoA Metabolite lipidmaps:LMFA07050450
3-oxo-6Z,9Z,12Z,15Z-octadecatetraenoyl-CoA Metabolite lipidmaps:LMFA07050451
4Z,7Z,10Z,13Z-hexadecatetraenoyl-CoA Metabolite lipidmaps:LMFA07050452
2E,4Z,7Z,10Z,13Z-hexadecapentaenoyl-CoA Metabolite lipidmaps:LMFA07050453
3E,7Z,10Z,13Z-hexadecatetraenoyl-CoA Metabolite lipidmaps:LMFA07050454
Lauric acid Metabolite lipidmaps:LMFA01010012
Myristic acid Metabolite lipidmaps:LMFA01010014
Palmitic acid Metabolite lipidmaps:LMFA01010001
9Z-palmitoleic acid Metabolite lipidmaps:LMFA01030056
Stearic acid Metabolite lipidmaps:LMFA01010018
Oleic acid Metabolite lipidmaps:LMFA01030002
Linoleic acid Metabolite lipidmaps:LMFA01030120
alpha-Linolenic acid Metabolite lipidmaps:LMFA01030152
gamma-Linolenic acid Metabolite lipidmaps:LMFA01030141
Arachidonic acid Metabolite lipidmaps:LMFA01030001
5Z,8Z,11Z,14Z,17Z-eicosapentaenoic acid Metabolite lipidmaps:LMFA01030759
Docosahexaenoic acid Metabolite lipidmaps:LMFA01030185
EHHADH GeneProduct uniprot:Q08426
ACSL3 GeneProduct uniprot:O95573
ECHS1 GeneProduct uniprot:P30084
DECR1 GeneProduct uniprot:Q16698
ACADL GeneProduct uniprot:P28330
ACSL3 GeneProduct uniprot:O95573
ECI1 GeneProduct uniprot:P42126
ACAA2 GeneProduct uniprot:P42765
HSD17B10 GeneProduct uniprot:Q99714
ACADSB GeneProduct uniprot:P45954
ACADSB GeneProduct uniprot:P45954
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
ECHS1 GeneProduct uniprot:P30084
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
HSD17B10 GeneProduct uniprot:Q99714
ACAA2 GeneProduct uniprot:P42765
ACAA2 GeneProduct uniprot:P42765
ECI1 GeneProduct uniprot:P42126
ECI1 GeneProduct uniprot:P42126
ECI1 GeneProduct uniprot:P42126
ECI1 GeneProduct uniprot:P42126
ACAA2 GeneProduct uniprot:P42765
ACAA2 GeneProduct uniprot:P42765
ACADSB GeneProduct uniprot:P45954
ACAA2 GeneProduct uniprot:P42765
ACADSB GeneProduct uniprot:P45954
ACAA2 GeneProduct uniprot:P42765
ACADSB GeneProduct uniprot:P45954
ACAA2 GeneProduct uniprot:P42765
ACADSB GeneProduct uniprot:P45954
ACAA2 GeneProduct uniprot:P42765
ACAA2 GeneProduct uniprot:P42765
ACADSB GeneProduct uniprot:P45954
ACAA2 GeneProduct uniprot:P42765
ACADSB GeneProduct uniprot:P45954
ACAA2 GeneProduct uniprot:P42765
ACADSB GeneProduct uniprot:P45954
ACAA2 GeneProduct uniprot:P42765
ECI1 GeneProduct uniprot:P42126
ACAA2 GeneProduct uniprot:P42765
ACADSB GeneProduct uniprot:P45954
ACAA2 GeneProduct uniprot:P42765
Long-chain-fatty-acid--CoA ligase Protein brenda:6.2.1.3
Long-chain-fatty-acid--CoA ligase Protein brenda:6.2.1.3
Long-chain-fatty-acid--CoA ligase Protein brenda:6.2.1.3
Long-chain-fatty-acid--CoA ligase Protein brenda:6.2.1.3
Long-chain-fatty-acid--CoA ligase Protein brenda:6.2.1.3
Long-chain-fatty-acid--CoA ligase Protein brenda:6.2.1.3
Long-chain-fatty-acid--CoA ligase Protein brenda:6.2.1.3

References

  1. Isolation and expression of a cDNA encoding the precursor for a novel member (ACADSB) of the acyl-CoA dehydrogenase gene family. Rozen R, Vockley J, Zhou L, Milos R, Willard J, Fu K, et al. Genomics. 1994 Nov 15;24(2):280–7. PubMed Europe PMC Scholia
  2. Delta 3, delta 2-enoyl-CoA isomerase is the protein that copurifies with human glutathione S-transferases from S-hexylglutathione affinity matrices. Takahashi Y, Hirata Y, Burstein Y, Listowsky I. Biochem J. 1994 Dec 15;304 ( Pt 3)(Pt 3):849–52. PubMed Europe PMC Scholia
  3. Human short-chain L-3-hydroxyacyl-CoA dehydrogenase: cloning and characterization of the coding sequence. Vredendaal PJ, van den Berg IE, Malingré HE, Stroobants AK, Olde Weghuis DE, Berger R. Biochem Biophys Res Commun. 1996 Jun 25;223(3):718–23. PubMed Europe PMC Scholia
  4. A human brain L-3-hydroxyacyl-coenzyme A dehydrogenase is identical to an amyloid beta-peptide-binding protein involved in Alzheimer’s disease. He XY, Schulz H, Yang SY. J Biol Chem. 1998 Apr 24;273(17):10741–6. PubMed Europe PMC Scholia
  5. Structure and reactivity of human mitochondrial 2,4-dienoyl-CoA reductase: enzyme-ligand interactions in a distinctive short-chain reductase active site. Alphey MS, Yu W, Byres E, Li D, Hunter WN. J Biol Chem. 2005 Jan 28;280(4):3068–77. PubMed Europe PMC Scholia
  6. The crystal structure of human mitochondrial 3-ketoacyl-CoA thiolase (T1): insight into the reaction mechanism of its thiolase and thioesterase activities. Kiema TR, Harijan RK, Strozyk M, Fukao T, Alexson SEH, Wierenga RK. Acta Crystallogr D Biol Crystallogr. 2014 Dec 1;70(Pt 12):3212–25. PubMed Europe PMC Scholia
  7. Clinical, biochemical and metabolic characterisation of a mild form of human short-chain enoyl-CoA hydratase deficiency: significance of increased N-acetyl-S-(2-carboxypropyl)cysteine excretion. Yamada K, Aiba K, Kitaura Y, Kondo Y, Nomura N, Nakamura Y, et al. J Med Genet. 2015 Oct;52(10):691–8. PubMed Europe PMC Scholia