Mitochondrial beta-oxidation (WP5241)
Homo sapiens
Human mitochondrial beta-oxidation
Authors
Conroy lipids , Egon Willighagen , Denise Slenter , and Eric WeitzActivity
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Organisms
Homo sapiensCommunities
Annotations
Pathway Ontology
lipid metabolic pathway classic metabolic pathway fatty acid beta degradation pathwayLabel | 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 | |
ECI1 | GeneProduct | uniprot:P42126 | |
ACAA2 | GeneProduct | uniprot:P42765 | |
HSD17B10 | GeneProduct | uniprot:Q99714 | |
ACADSB | GeneProduct | uniprot:P45954 | |
Long-chain-fatty-acid--CoA ligase | Protein | brenda:6.2.1.3 |
References
- 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
- 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
- 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
- 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
- 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
- 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
- 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