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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Cofactor metabolism

Assembly and handling of non-vitamin and vitamin-derived cofactor systems. 34 reactions, from Reactome R-HSA-8978934.

  1. GCH1 reduces GTP to dihydroneopterin triphosphateGTP + H2O → DHNTP + HCOOH
  2. GCHFR binds to GCH1 and negatively regulates its activityGCH1 decamer + 2 GCHFR pentamer → 2GCHFR:GCH1
  3. DHNTP is dephosphorylated by PTPS to PTHPDHNTP → PTHP + PPP
  4. PTHP is reduced to BH4 by sepiapterin reductase (SPR)PTHP + 2 NADPH → BH4 + 2 NADP+
  5. PTPS is phosphorylated by cGMP-dependant protein kinase II6 ATP + PTPS hexamer → p-PTPS hexamer + 6 ADP
  6. The cofactor BH4 is required for electron transfer in the eNOS catalytic cyclep-S1177-eNOS:CaM:HSP90:p-AKT1 + BH4 → p-S1177-eNOS:CaM:HSP90:p-AKT1:BH4
  7. Salvage - BH2 is reduced to BH4 by DHFRq-BH2 + H+ + NADPH → BH4 + NADP+
  8. BH2 binding can lead to eNOS uncouplingp-S1177-eNOS:CaM:HSP90:p-AKT1:BH4 + q-BH2 → p-S1177-eNOS:CaM:HSP90:p-AKT1:BH2 + BH4
  9. BH4 is oxidised to the BH3 radical during the eNOS catalytic cycleBH4 → BH3. + e-
  10. Sepiapterin reductase (SPR) is phosphorylated by Ca2+/calmodulin-dependent protein kinase IISPR dimer + 2 ATP → 2 ADP + p-SPR dimer
  11. Ascorbate can reduce the BH3 radical back to BH4BH3. + AscH- → BH4 + Asc.-
  12. The BH3 radical can decay to dihydrobiopterin (BH2)BH3. → q-BH2
  13. Peroxynitrite can oxidise BH4 to the BH3 radicalBH4 + Peroxynitrite → BH3.
  14. Salvage - Sepiapterin is reduced to q-BH2NADPH + sepiapterin + H+ → q-BH2 + NADP+
  15. Ferrous iron reduces the BH3 radical back to BH4Fe2+ + BH3. → BH4 + Fe3+
  16. COQ3 methylates DeMQ10H2DeMQ10H2 + AdoMet → CoQH2 + AdoHcy + H+
  17. COQ6 hydroxylates DHBDHB + O2 + H+ + (2Fe-2S)(1+) → DHDB + H2O + (2Fe-2S)2+
  18. COQ5 methylates MDMQ10H2MDMQ10H2 + AdoMet → DMQ10H2 + AdoHcy + H+
  19. Unknown enzyme hydroxylates DMPhOHDMPhOH + O2 + NADPH + H+ → MDMQ10H2 + NADP+ + H2O
  20. COQ2 ligates all-E-10PrP2 to PHBPHB + all-E-10PrP2 → DHB + PPi
  21. COQ3 methylates DHDBDHDB + AdoMet → MHDB + AdoHcy + H+
  22. COQ7:COQ9 octamer hydroxylates DMQ10H2DMQ10H2 + O2 + H+ + NADH → DeMQ10H2 + H2O + NAD+
  23. COQ4 decarboxylates MHDBMHDB + H+ → DMPhOH + CO2
  24. PDSS1,2 ligates FPP to IPPPFPP + 7 IPPP → all-E-10PrP2 + 7 PPi
  25. isocitrate + NADP+ => 2-oxoglutarate + CO2 + NADPH + H+ISCIT + NADP+ → 2OG + CO2 + NADPH + H+
  26. isocitrate + NADP+ => 2-oxoglutarate + CO2 + NADPH + H+ISCIT + NADP+ → 2OG + CO2 + NADPH + H+
  27. Exchange of isocitrate and 2-oxoglutarate across the peroxisomal membrane2OG + ISCIT → 2OG + ISCIT
  28. ACO1:4Fe-4S isomerises CIT to ISCITCIT → ISCIT
  29. Unknown sepiapterin synthase transforms PTHP to sepiapterinPTHP → sepiapterin
  30. mito-STARD7 transports Q10 to outer mitochondrial membraneCoQH2 → CoQH2
  31. STARD7 transports Q10 to plasma membraneCoQH2 → CoQH2
  32. HPDL dioxygenates HPPAHPP + O2 → 4-HMA + CO2
  33. Unknown enzyme(s) process 4-HMA to 4-Hbz4-HMA → 4-Hbz
  34. Unknown dehydrogenase oxidizes 4-HBz4-Hbz + H2O + NAD+ → PHB + NADH + H+