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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Branched-chain amino acids

Mitochondrial catabolism of valine, leucine and isoleucine into acetyl-, acetoacetyl-, propionyl- and succinyl-linked products. 27 reactions, from Reactome R-HSA-70895.

  1. a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate leu, ile, or val + alpha-ketoglutarate [BCAT2]KIC, KMVA, KIV + L-Glu → 2OG + Leu, Ile, Val
  2. a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate leu, ile, or val + alpha-ketoglutarate [BCAT1]KIC,KMVA,KIV + L-Glu → Leu, Ile, Val + 2OG
  3. beta-methylglutaconyl-CoA + ADP + orthophosphate beta-methylcrotonyl-CoA + ATP + HCO3- (MCCA)bMG-CoA + ADP + Pi + H+ → bMC-CoA + ATP + HCO3-
  4. alpha-methylacetoacetyl-CoA + NADH + H+ alpha-methyl-beta-hydroxybutyryl-CoA + NAD+2MACA-CoA + NADH + H+ → aMbHBUT-CoA + NAD+
  5. methylmalonyl semialdehyde + NADH + H+ beta-hydroxyisobutyrate + NAD+2M3OPROA + NADH + H+ → NAD+ + bHIBA
  6. BCKDK phosphorylates BCKDHBCKDH + 2 ATP → p-BCKDH + 2 ADP + H+
  7. PPM1K dephosphorylates p-BCKDHp-BCKDH + 2 H2O → BCKDH + 2 Pi
  8. leu, ile, or val + alpha-ketoglutarate a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT1]Leu, Ile, Val + 2OG → KIC,KMVA,KIV + L-Glu
  9. leu, ile, or val + alpha-ketoglutarate a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2]Leu, Ile, Val + 2OG → KIC, KMVA, KIV + L-Glu
  10. isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2ISV-CoA + FAD + H+ → bMC-CoA + FADH2
  11. beta-methylcrotonyl-CoA + ATP + HCO3- beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA)bMC-CoA + ATP + HCO3- → bMG-CoA + ADP + Pi + H+
  12. beta-methylglutaconyl-CoA + H2O beta-hydroxy-beta-methylglutaryl-CoAbMG-CoA + H2O → bHMG-CoA
  13. alpha-methylbutyryl-CoA + FAD => tiglyl-CoA + FADH22MBUT-CoA + FAD + H+ → tiglyl-CoA + FADH2
  14. tiglyl-CoA + H2O alpha-methyl-beta-hydroxybutyryl-CoAtiglyl-CoA + H2O → aMbHBUT-CoA
  15. alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ alpha-methylacetoacetyl-CoA + NADH + H+aMbHBUT-CoA + NAD+ → 2MACA-CoA + NADH + H+
  16. alpha-methylacetoacetyl-CoA + CoA => propionyl-CoA + acetyl-CoA2MACA-CoA + CoA-SH → PROP-CoA + Ac-CoA
  17. isobutyryl-CoA + FAD => methacrylyl-CoA + FADH2ISB-CoA + FAD + H+ → MACR-CoA + FADH2
  18. ECHS1 hydrates methacrylyl-CoAMACR-CoA + H2O → bHIB-CoA
  19. beta-hydroxyisobutyryl-CoA + H2O => beta-hydroxyisobutyrate + CoAbHIB-CoA + H2O → bHIBA + CoA-SH + H+
  20. beta-hydroxyisobutyrate + NAD+ methylmalonyl semialdehyde + NADH + H+bHIBA + NAD+ → 2M3OPROA + NADH + H+
  21. methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+2M3OPROA + NAD+ + CoA-SH + H2O → PROP-CoA + NADH + H+ + HCO3-
  22. SLC25A44 transports Leu, Ile and Val from cytosol to mitochondrial matrixLeu, Ile, Val → Leu, Ile, Val
  23. BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIVLipo-K105-DBT + KIC, KMVA, KIV + H+ → CO2 + BCAA-lipoH2-K105-DBT
  24. DBT transfers BCAA to CoABCAA-lipoH2-K105-DBT + CoA-SH → BCAA-CoAs + LipoH2-K105-DBT
  25. DLD dimer dehydrogenates dihydrolipoylNAD+ + LipoH2-K105-DBT → H+ + NADH + Lipo-K105-DBT
  26. CRAT transfers ISB to CARCAR + ISB-CoA → CoA-SH + ISB-CAR
  27. GLYAT transfers ISB to glycineISB-CoA + Gly → CoA-SH + ISB-Gly + H+