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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Mitochondrial β-oxidation

Repeated dehydrogenation, hydration, oxidation and thiolysis that shortens mitochondrial fatty acyl-CoA species. 47 reactions, from Reactome R-HSA-77289.

  1. Isomerization of cis,cis-3,6-Dodecadienoyl-CoA to form trans,cis-Lauro-2,6-dienoyl-CoAcis,cis-3,6-Dodecadienoyl-CoA → trans,cis-Lauro-2,6-dienoyl-CoA
  2. Linoleoyl-CoA is converted to cis,cis-3,6- dodecadienoyl-CoA by three cycles of beta-oxidationLIN-CoA + 3 FAD + 3 H2O + 3 NAD+ + 3 CoA-SH → cis,cis-3,6-Dodecadienoyl-CoA + 3 FADH2 + 3 NADH + 3 H+ + 3 Ac-CoA
  3. dehydrogenation of 4-cis-decenoyl-CoA to form 2-trans-4-cis-decadienoyl-CoA4-cis-decenoyl-CoA + FAD → 2-trans-4-cis-decadienoyl-CoA + FADH2
  4. Trans,cis-lauro-2,6-dienoyl-CoA is converted to cis,cis-3,6- 4-cis-decenoyl-CoA by one cycle of beta-oxidationtrans,cis-Lauro-2,6-dienoyl-CoA + FAD + H2O + NAD+ + CoA-SH → 4-cis-decenoyl-CoA + FADH2 + NADH + Ac-CoA + H+
  5. Reduction of 2-trans-4-cis-decadienoyl-CoA to form 3-trans-decenoyl-CoA2-trans-4-cis-decadienoyl-CoA + NADH + H+ → 3-trans-decenoyl-CoA + NAD+
  6. Isomerization of 3-trans-decenoyl-CoA to form trans-dec-2-enoyl-CoA3-trans-decenoyl-CoA → tdec2-CoA
  7. Cytosolic ACOTs hydrolyse MCFA-CoA, LCFA-CoAMCFA-CoA, LCFA-CoA + H2O → MCFA, LCFA + CoA-SH + H+
  8. ACOT2,9,THEM4,5 hydrolyse MCFA-CoA, LCFA-CoAMCFA-CoA, LCFA-CoA + H2O → MCFA, LCFA + CoA-SH + H+
  9. ACAD10 dehydrogenates S-2MPDA-CoAS-2MPDA-CoA + FAD → S-2MDPDA-CoA + FADH2
  10. ACAD11 dehydrogenates BH-CoABH-CoA + FAD → DHB-CoA + FADH2
  11. ACSF2 ligates CoA-SH to MCFAMCFA + ATP + CoA-SH → MCFA-CoA + AMP + PPi
  12. MUT isomerises L-MM-CoA to SUCC-CoAL-MM-CoA → SUCC-CoA
  13. D-methylmalonyl-CoA L-methylmalonyl-CoAMEMA-CoA → L-MM-CoA
  14. propionyl-CoA + CO2 + ATP D-methylmalonyl-CoA + ADP + orthophosphatePROP-CoA + ATP + HCO3- → MEMA-CoA + ADP + Pi + H+
  15. (S)-3-Hydroxydodecanoyl-CoA+NAD => 3-Oxododecanoyl-CoA+NADH+HNAD+ + (S)-3-Hydroxydodecanoyl-CoA → NADH + H+ + 3-Oxododecanoyl-CoA
  16. 2-trans-Dodecenoyl-CoA+H2O =>( S)-3-Hydroxydodecanoyl-CoAH2O + 2-trans-Dodecenoyl-CoA → (S)-3-Hydroxydodecanoyl-CoA
  17. lauroyl-CoA+FAD => 2-trans-Dodecenoyl-CoA+FADH2FAD + LAU-CoA + H+ → FADH2 + 2-trans-Dodecenoyl-CoA
  18. 3-Oxotetradecanoyl-CoA+CoA-SH => Lauroyl-CoA + acetyl-CoA3-Oxotetradecanoyl-CoA + CoA-SH → LAU-CoA + Ac-CoA
  19. myristoyl-CoA+FAD => trans-tetradec-2-enoyl-CoA+FADH2FAD + MYS-CoA + H+ → FADH2 + trans-Tetradec-2-enoyl-CoA
  20. trans-Tetradec-2-enoyl-CoA+H2O => (S)-3-Hydroxytetradecanoyl-CoAH2O + trans-Tetradec-2-enoyl-CoA → (S)-3-Hydroxytetradecanoyl-CoA
  21. (S)-3-Hydroxytetradecanoyl-CoA+NAD => 3-Oxotetradecanoyl-CoA+NADH+HNAD+ + (S)-3-Hydroxytetradecanoyl-CoA → NADH + H+ + 3-Oxotetradecanoyl-CoA
  22. palmitoyl-CoA+FAD => trans-Hexadec-2-enoyl-CoA+FADH2PalmCoA + FAD + H+ → FADH2 + trans-Hexadec-2-enoyl-CoA
  23. trans-Hexadec-2-enoyl-CoA+H2O => (S)-3-Hydroxyhexadecanoyl-CoAtrans-Hexadec-2-enoyl-CoA + H2O → (S)-3-Hydroxyhexadecanoyl-CoA
  24. (S)-3-Hydroxyhexadecanoyl-CoA+NAD => 3-Oxopalmitoyl-CoA+NADH+H(S)-3-Hydroxyhexadecanoyl-CoA + NAD+ → NADH + 3-Oxopalmitoyl-CoA + H+
  25. 3-Oxopalmitoyl-CoA+CoA-SH => myristoyl-CoA+acetyl-CoA3-Oxopalmitoyl-CoA + CoA-SH → MYS-CoA + Ac-CoA
  26. 3-Oxododecanoyl-CoA+CoA-SH => Decanoyl-CoA + acetyl-CoA3-Oxododecanoyl-CoA + CoA-SH → DEC-CoA + Ac-CoA
  27. (S)-Hydroxybutanoyl-CoA+NAD => Acetoacetyl-CoA+NADH+HNAD+ + 3HB-CoA → NADH + H+ + ACA-CoA
  28. Crotonoyl-CoA+H2O => (S)-3-Hydroxybutanoyl-CoAH2O + Crotonoyl-CoA → 3HB-CoA
  29. Butanoyl-CoA+FAD => Crotonoyl-CoA+FADH2FAD + BT-CoA + H+ → FADH2 + Crotonoyl-CoA
  30. 3-Oxohexanoyl-CoA+CoA-SH => Butanoyl-CoA + acetyl-CoA3-Oxohexanoyl-CoA + CoA-SH → Ac-CoA + BT-CoA
  31. (S)-Hydroxyhexanoyl-CoA+NAD => 3-Oxohexanoyl-CoA+NADH+HNAD+ + (S)-Hydroxyhexanoyl-CoA → NADH + H+ + 3-Oxohexanoyl-CoA
  32. trans-Hex-2-enoyl-CoA+H2O (S)-Hydroxyhexanoyl-CoAH2O + trans-Hex-2-enoyl-CoA → (S)-Hydroxyhexanoyl-CoA
  33. Hexanoyl-CoA+FAD => trans-Hex-2-enoyl-CoA+FADH2FAD + HX-CoA + H+ → FADH2 + trans-Hex-2-enoyl-CoA
  34. 3-Oxooctanoyl-CoA+CoA-SH => Hexanoyl-CoA + acetyl-CoA3-Oxooctanoyl-CoA + CoA-SH → HX-CoA + Ac-CoA
  35. (S)-Hydroxyoctanoyl-CoA+NAD => 3-Oxooctanoyl-CoA+NADH+HNAD+ + (S)-Hydroxyoctanoyl-CoA → NADH + H+ + 3-Oxooctanoyl-CoA
  36. trans-Oct-2-enoyl-CoA+H2O => (S)-Hydroxyoctanoyl-CoAH2O + trans-Oct-2-enoyl-CoA → (S)-Hydroxyoctanoyl-CoA
  37. Octanoyl-CoA+FAD => trans-Oct-2-enoyl-CoA+FADH2FAD + Octanoyl-CoA + H+ → FADH2 + trans-Oct-2-enoyl-CoA
  38. 3-Oxodecanoyl-CoA+CoA-SH => Octanoyl-CoA + acetyl-CoA3-Oxodecanoyl-CoA + CoA-SH → Octanoyl-CoA + Ac-CoA
  39. (S)-Hydroxydecanoyl-CoA+NAD => 3-Oxodecanoyl-CoA+NADH+HNAD+ + (S)-Hydroxydecanoyl-CoA → NADH + H+ + 3-Oxodecanoyl-CoA
  40. trans-Dec-2-enoyl-CoA+H2O => (S)-Hydroxydecanoyl-CoAH2O + tdec2-CoA → (S)-Hydroxydecanoyl-CoA
  41. Decanoyl-CoA+FAD => trans-Dec-2-enoyl-CoA+FADH2FAD + DEC-CoA + H+ → FADH2 + tdec2-CoA
  42. DBI, ACBD7 bind MCFA-CoA and LCFA-CoADBI, (ACBD7) + MCFA-CoA, LCFA-CoA → DBI,ACBD7:MCFA-CoA,LCFA-CoA
  43. ACBD6 binds LCFA-CoAACBD6 + LCFA-CoA → ACBD6:LCFA-CoA
  44. ACAA2 tetramer transfers acyl group from Ac-CoA to acyl-CoA forming 3OA-CoA and CoA-SHAc-CoA + acyl-CoA → 3OA-CoA + CoA-SH
  45. ACSM3,ACSM6 ligate CoA to BUTBUT + ATP + CoA-SH → BT-CoA + AMP + PPi
  46. MCAT transfers Mal from Mal-CoA to NDUFAB1Malonyl-CoA + NDUFAB1 → Mal-PPANT-S112-NDUFAB1 + CoA-SH
  47. MECR dimer reduces tdec2-CoA to DEC-CoAtdec2-CoA + NADPH + H+ → DEC-CoA + NADP+