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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Citric acid cycle

The mitochondrial reaction cycle that oxidizes acetyl units while exchanging intermediates with many biosynthetic routes. 23 reactions, from Reactome R-HSA-71403.

  1. NNT dimer transfers proton from NADPH to NAD+NAD+ + NADPH + H+ → H+ + NADH + NADP+
  2. IDH2 dimer decarboxylates isocitrateISCIT + NADP+ → 2OG + CO2 + NADPH
  3. IDH3 complex decarboxylates isocitrateISCIT + NAD+ → 2OG + CO2 + NADH
  4. ACO2 isomerizes citrateCIT → ISCIT
  5. CS acetylates OA to citrateAc-CoA + H2O + OA → CIT + CoA-SH + H+
  6. MDH2 dimer dehydrogenates MALMAL + NAD+ → OA + NADH + H+
  7. FH tetramer hydrates fumarate to L-malateFUMA + H2O → MAL
  8. SDH complex dehydrogenates succinateSUCCA + CoQ → FUMA + CoQH2
  9. SUCLG1/A2 cleaves succinyl-CoAADP + Pi + SUCC-CoA → ATP + SUCCA + CoA-SH
  10. OGDH dimer decarboxylates 2-OG2OG + H+ + Lipo-K110-DLST → CO2 + SuccinyllipoH2-K110-DLST
  11. SUCLG1/G2 cleaves succinyl-CoAGDP + Pi + SUCC-CoA → GTP + SUCCA + CoA-SH
  12. DLD dimer dehydrogenates dihydrolipoylNAD+ + LipoH2-K110-DLST → H+ + NADH + Lipo-K110-DLST
  13. DLST transfers succinyl to CoACoA-SH + SuccinyllipoH2-K110-DLST → SUCC-CoA + LipoH2-K110-DLST
  14. CSKMT methylates Citrate SynthaseMethylated(n) CS dimer + 2 S-adenosyl-L-methionine → Methylated(n+1) CS dimer + 2 H+ + 2 S-adenosyl-L-homocysteine
  15. ROS,RNS oxidize ACO2:4Fe-4SROS,RNS + ACO2:4Fe-4S → ACO2:3Fe-4S + Fe2+
  16. Frataxin transfers Fe2+ to ACO2ACO2:3Fe-4S + 2 Iron:FXN → ACO2:4Fe-4S + FXN(81-210) + Fe2+
  17. ACAT1 tetramer acetylates IDH2 dimer2 Ac-CoA + IDH2 dimer → 2 CoA-SH + 2 H+ + AcK-IDH2 dimer
  18. SIRT3 deacetylates AcK-IDH2 dimer2 NAD+ + AcK-IDH2 dimer → 2 NAM + 2 2'-O-acetyl-ADP-ribose + IDH2 dimer
  19. FAD attaches to SDHASDHA + FAD → FAD-SDHA
  20. SDHAF4 binds to FAD-SDHAFAD-SDHA + SDHAF4 → FAD-SDHA:SDHAF4
  21. Transfer of Fe-S clusters to SDHBFXN:NFS1:ISD11:ISCU:2Fe-2S Cluster + 2 ISCA1:ISCA2:4Fe-4S + SDHB + SDHAF1 + SDHAF3 → FXN:ISD11:NFS1:ISCU + 2 ISCA1:ISCA2 + Fe2+ + SDHB:2Fe-2S:3Fe-4S:4Fe-4S:SDHAF1:SDHAF3
  22. SDHA binds to SDHBFAD-SDHA:SDHAF4 + SDHB:2Fe-2S:3Fe-4S:4Fe-4S:SDHAF1:SDHAF3 → SDHAF4 + FAD-SDHA:SDHB:2Fe-2S:3Fe-4S:4Fe-4S:SDHFA1:SDHFA3
  23. SDHA:SDHB binds to SDHC:SDHDFAD-SDHA:SDHB:2Fe-2S:3Fe-4S:4Fe-4S:SDHFA1:SDHFA3 + SDHC:SDHD:heme b → SDH complex + SDHAF1 + SDHAF3