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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Porphyrin metabolism

Mitochondrial and cytosolic construction of heme followed by controlled degradation of the tetrapyrrole ring. 42 reactions, from Reactome R-HSA-189445.

  1. UGT1A4 transfers GlcA from UDP-GlcA to BMG to form BDGBMG + UDP-GlcA → BDG + UDP
  2. UGT1A4 transfers GlcA from UDP-GlcA to BIL to form BMGBIL + UDP-GlcA → BMG + UDP
  3. BIL translocates from the cytosol to the ER lumenBIL → BIL
  4. BLVRA:Zn2+, BLVRB reduce BV to BILBV + NADPH → BIL + NADP+
  5. HMOX1 dimer, HMOX2 cleave hemeferroheme + 3 NADPH + 3 O2 → 3 NADP+ + 3 H2O + Fe2+ + CO + BV
  6. 4 PBGs bind to form HMB4 PBG + H2O → HMBL + 4 NH4+
  7. CPO transforms COPRO3 to PPGEN9COPRO3 + O2 + H+ → PPGEN9 + 2 CO2 + H2O
  8. PPO oxidises PPGEN9 to PRIN9PPGEN9 + 3 O2 → PRIN9 + 3 H2O2
  9. UROD decarboxylates URO3 to COPRO3URO3 + 4 H+ → COPRO3 + 4 CO2
  10. ALAD condenses 2 dALAs to form PBG2 dALA → PBG + 2 H2O + H+
  11. ALAS condenses SUCC-CoA and Gly to form dALASUCC-CoA + Gly + H+ → dALA + CoA-SH + CO2
  12. Mitochondrial dALA translocates to cytosoldALA → dALA
  13. Mitochondrial intermembrane PRIN9 translocates to mitochondrial matrixPRIN9 → PRIN9
  14. FECH binds Fe2+ to PRIN9 to form hemePRIN9 + Fe2+ → 2 H+ + heme b
  15. Cytosolic COPRO3 translocates to mitochondrial intermembrane spaceCOPRO3 → COPRO3
  16. UROS transforms HMB to URO3HMBL → URO3 + H2O
  17. ALAD binds to Pb2+8x(ALAD:Zn2+) + 4 Pb2+ → 8xALAD:Pb2+:Zn2+ + 4 Zn2+
  18. HMBL spontaneously transforms to URO1HMBL → H2O + URO1
  19. UROD decarboxylates URO1 to COPRO1URO1 + 4 H+ → COPRO1 + 4 CO2
  20. COX10 transforms heme to heme OFPP + H2O + heme b → PPi + heme O
  21. COX15 transforms heme O to heme A2 hydrogen acceptor + H2O + heme O → 2 hydrogen donor + heme A
  22. ABCC2 transports BMG,BDG from cytosol to extracellular regionBMG,BDG + ATP + H2O → BMG,BDG + ADP + Pi
  23. FLVCR1-1 transports heme from cytosol to extracellular regionferroheme → heme
  24. ABCG2 tetramer transports heme from cytosol to extracellular regionferroheme + ATP + H2O → heme + ADP + Pi + H+
  25. UGT1A1 tetramer transfers GlcA from UDP-GlcA to BMG to form BDGBMG + UDP-GlcA → BDG + UDP
  26. UGT1A1 transfers GlcA from UDP-GlcA to BIL to form BMGBIL + UDP-GlcA → BMG + UDP
  27. SLCO1B1 transports BIL from extracellular region (blood) to cytosol (hepatocyte)BIL → BIL
  28. ABCC1 transports BIL from cytosol to extracellular region (blood)BIL → BIL
  29. FLVCR1-2 transports heme from mitochondrial matrix to cytosolheme → ferroheme
  30. ABCG2 tetramer transports BMG,BDG from cytosol to extracellular regionBMG,BDG + ATP + H2O → BMG,BDG + ADP + Pi
  31. ALB binds extracellular hemeALB + heme → ALB:heme
  32. BIL:ALB dissociatesBIL:ALB → BIL + ALB
  33. BIL binds ALBBIL + ALB → BIL:ALB
  34. BMG, BDG translocates from ER lumen to cytosolBMG,BDG → BMG,BDG
  35. An unknown oxidase oxidises D-UBGN to UBND-UBGN + an oxidized electron acceptor → UBN + a reduced electron acceptor
  36. SLCO2B1-3 transports BIL from extracellular region (blood) to cytosol (hepatocyte)BIL → BIL
  37. An unknown reductase reduces D-UBGN to STBND-UBGN + a reduced electron acceptor → STBN + an oxidized electron acceptor
  38. An unknown BILR reduces BIL to D-UBGNBIL + a reduced electron acceptor → D-UBGN + an oxidized electron acceptor
  39. SLCO1B3 transports BIL from extracellular region (blood) to cytosol (hepatocyte)BIL → BIL
  40. Bacterial GUSB hydrolyses BDG to BILBDG + 3 NADPH + 2 H2O → 3 NADP+ + 2 GlcA + BIL
  41. BIL dissociates from GSTA1, FABP1BIL:GSTA1, FABP1 → BIL + GSTA1, FABP1
  42. BIL binds GSTA1, FABP1BIL + GSTA1, FABP1 → BIL:GSTA1, FABP1