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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Bile acids and salts

Synthesis, conjugation, transport and recycling of amphipathic cholesterol metabolites. 87 reactions, from Reactome R-HSA-194068.

  1. Cytosolic cholate and chenodeoxycholate are conjugated with Coenzyme A (SLC27A5 BACS)CCA,CDCA + ATP + CoA-SH → choloyl-CoA; chenodeoxycholoyl-CoA + AMP + PPi + H2O
  2. Cytosolic chenodeoxycholoyl-CoA or choloyl-CoA are conjugated with glycine or taurinecholoyl-CoA; chenodeoxycholoyl-CoA + Gly, TAU → bile salts + CoA-SH
  3. THCA is translocated from the mitochondrial matrix to the cytosolTHCA → THCA
  4. 27-hydroxycholesterol is 7alpha-hydroxylated27HCHOL + NADPH + H+ + O2 → Cholest-5-ene-3beta,7alpha,27-triol + H2O + NADP+
  5. Cholesterol is hydroxylated to 25-hydroxycholesterolCHOL + NADPH + H+ + O2 → H2O + NADP+ + 25OH-CHOL
  6. CYP27A1 27-hydroxylates 5bCHOL3a,7a,12a-triol5bCHOL3a,7a,12a-triol + NADPH + H+ + O2 → 5bCHOL3a,7a,12a,27-tetrol + H2O + NADP+
  7. 5beta-cholestan-3alpha, 7alpha, 12alpha-triol is translocated from the cytosol to the mitochondrial matrix5beta-cholestan-3alpha,7alpha,12alpha-triol → 5bCHOL3a,7a,12a-triol
  8. 4-cholesten-7alpha-ol-3-one is reduced to 5beta-cholestan-7alpha-ol-3-one4CHOL7aOLONE + NADPH + H+ → 5beta-cholestan-7alpha-ol-3-one + NADP+
  9. 5Beta-cholesten-7alpha, 12alpha-diol-3-one is reduced to 5beta-cholestan-3alpha, 7alpha, 12alpha-triol5beta-cholesten-7alpha, 12alpha-diol-3-one + NADPH + H+ → 5beta-cholestan-3alpha,7alpha,12alpha-triol + NADP+
  10. 5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al5bCHOL3a,7a,12a,27-tetrol + NADP+ → 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al + NADPH + H+
  11. CYP7A1 7-hydroxylates CHOLCHOL + NADPH + H+ + O2 → H2O + NADP+ + 7 alpha-hydroxycholesterol
  12. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate (THCA)3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-27-al + NADPH + H+ + O2 → THCA + NADP+ + H2O
  13. Isomerization of 25(R) THCA-CoA to 25(S) THCA-CoA25(R) THCA-CoA → 25(S) THCA-CoA
  14. CYP46A1 24-hydroxylates CHOLCHOL + NADPH + H+ + O2 → 24OH-CHOL + NADP+ + H2O
  15. CYP7B1 7-hydroxylates 25OH-CHOL25OH-CHOL + NADPH + H+ + O2 → CHOL3b,7a,25TRIOL + H2O + NADP+
  16. 4-cholesten-7alpha, 12alpha-diol-3-one is reduced to 5beta-cholesten-7alpha, 12alpha-diol-3-one4CHOL7a,12aDONE + NADPH + H+ → 5beta-cholesten-7alpha, 12alpha-diol-3-one + NADP+
  17. 7alpha-hydroxycholesterol is oxidized and isomerized to 4-cholesten-7alpha-ol-3-one7 alpha-hydroxycholesterol + NAD+ → 4CHOL7aOLONE + NADH + H+
  18. CYP27A1 27-hydroxylates CHOLCHOL + NADPH + H+ + O2 → 27OH-CHOL + H2O + NADP+
  19. THCA is conjugated with Coenzyme A (SLC27A5 BACS)THCA + ATP + CoA-SH → 25(R) THCA-CoA + AMP + PPi
  20. CYP8B1 12-hydroxylates 4CHOL7aOLONE4CHOL7aOLONE + NADPH + H+ + O2 → 4CHOL7a,12aDONE + NADP+ + H2O
  21. 5beta-cholestan-7alpha-ol-3-one is reduced to 5beta-cholestan-3alpha, 7alpha-diol5beta-cholestan-7alpha-ol-3-one + NADPH + H+ → 5beta-cholestan-3alpha,7alpha-diol + NADP+
  22. CYP39A1 7-hydroxylates 24OH-CHOL24OH-CHOL + O2 + hydrogen donor → CHOL7a,24(S)DIOL + H2O + hydrogen acceptor + H+
  23. Choloyl CoA reacts with glycine or taurine to form glycocholate or taurocholatecholoyl-CoA + Gly, TAU → cholate bile salts + CoA-SH + H+
  24. Bile salts are translocated from the peroxisomal matrix to the cytosolbile salts → bile salts
  25. 25(R) THCA-CoA is translocated from the cytosol to the peroxisome25(R) THCA-CoA + ATP + H2O → 25(R) THCA-CoA + ADP + H+ + Pi
  26. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA (THCA-CoA) is hydrated to (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoATHCA-CoA + H2O → (24R, 25R) 3a,7a,12a,24-tetrahydroxy-5b-cholestanoyl-CoA
  27. 25(S) THCA-CoA is dehydrogenated to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA (THCA-CoA)25(S) THCA-CoA + O2 → THCA-CoA + H2O2
  28. Thiolysis of 3alpha,7alpha,12alpha-trihydroxy-5beta-cholan-24-one-CoA yields choloyl-CoA (3alpha,7alpha,12alpha-trihydroxy-5beta-cholan-24-one-CoA) and propionyl CoA3a,7a,12a-trihydroxy-5b-cholest-24-one-CoA + CoA-SH → choloyl-CoA + propionyl CoA
  29. ABCB11 transports bile salts from cytosol to extracellular regionbile salts + ATP + H2O → bile salts + ADP + Pi + H+
  30. 25(S) DHCA-CoA is dehydrogenated to 25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA25(S) DHCA-CoA + O2 → 25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA + H2O2
  31. Hydrolysis of choloyl-CoA to cholate and CoASHH2O + choloyl-CoA → CCA + CoA-SH + H+
  32. 5beta-cholestan-3alpha, 7alpha-diol is hydroxylated to 5beta-cholestan-3alpha, 7alpha, 26-triol5beta-cholestan-3alpha, 7alpha-diol + NADPH + H+ + O2 → 5beta-cholestan-3alpha,7alpha,26-triol + H2O + NADP+
  33. Cholate is translocated from the peroxisomal matrix to the cytosolCCA → CCA
  34. THCA is conjugated with Coenzyme A (SLC27A2 VLCS)THCA + ATP + CoA-SH → 25(R) THCA-CoA + AMP + PPi
  35. DHCA is conjugated with Coenzyme A (SLC27A5 BACS)ATP + CoA-SH + DHCA → 25(R) DHCA-CoA + AMP + PPi
  36. DHCA is conjugated with Coenzyme A (SLC27A2 VLCS)DHCA + ATP + CoA-SH → 25(R) DHCA-CoA + AMP + PPi
  37. Isomerization of 25(R) DHCA-CoA to 25(S) DHCA-CoA25(R) DHCA-CoA → 25(S) DHCA-CoA
  38. (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoA(24R, 25R) 3a,7a,12a,24-tetrahydroxy-5b-cholestanoyl-CoA + NAD+ → 3a,7a,12a-trihydroxy-5b-cholest-24-one-CoA + NADH + H+
  39. 3alpha, 7alpha-dihydroxy-5beta-cholestan-26-al is oxidized to 3alpha, 7alpha-dihydroxy-5beta-cholestanoate (DHCA)3alpha,7alpha-dihydroxy-5beta-cholestan-26-al + NADPH + H+ + O2 → DHCA + NADP+ + H2O
  40. 25(R) DHCA-CoA is translocated from the cytosol to the peroxisome25(R) DHCA-CoA + ATP + H2O → 25(R) DHCA-CoA + ADP + Pi + H+
  41. Chenodeoxycholoyl CoA reacts with glycine or taurine to form glycochenodeoxycholate or taurochenodeoxycholatechenodeoxycholoyl-CoA + Gly, TAU → CoA-SH + chenodeoxycholate bile salts + H+
  42. 5beta-cholestan-3alpha, 7alpha, 26-triol is oxidized to 3alpha, 7alpha-dihydroxy-5beta-cholestan-26-al5beta-cholestan-3alpha,7alpha,26-triol + NADP+ → 3alpha,7alpha-dihydroxy-5beta-cholestan-26-al + H+ + NADPH
  43. (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA is oxidized to 3alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoA3,7,24THCA-CoA + NAD+ → 3a,7a-dihydroxy-5b-cholest-24-one-CoA + NADH + H+
  44. DHCA is translocated from the mitochondrial matrix to the cytosolDHCA → DHCA
  45. Thiolysis of 3alpha,7alpha-dihydroxy-5beta-cholan-24-one-CoA yields chenodeoxycholoyl-CoA (3alpha,7alpha-dihydroxy-5beta-cholan-24-one-CoA) and propionyl CoA3a,7a-dihydroxy-5b-cholest-24-one-CoA + CoA-SH → chenodeoxycholoyl-CoA + propionyl CoA
  46. 25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA is hydrated to (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA + H2O → 3,7,24THCA-CoA
  47. 5beta-cholestan-3alpha, 7alpha-diol is translocated from the cytosol to the mitochondrial matrix5beta-cholestan-3alpha,7alpha-diol → 5beta-cholestan-3alpha, 7alpha-diol
  48. 3,7,24THCA is conjugated with Coenzyme A (SLC27A5 BACS)3,7,24THCA + ATP + CoA-SH → 3,7,24THCA-CoA + AMP + PPi + H2O
  49. 5beta-cholestan-3alpha,7alpha,24(S)-triol is translocated from the cytosol to the mitochondrial matrix5beta-cholestan-3alpha,7alpha,24(S)-triol → 5β-CHOL3α,7α,24(s)-triol
  50. 5beta-cholestan-3alpha,7alpha,24(S),27-tetrol is oxidized to 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al5bCHOL3a,7a,24(s),27-tetrol + O2 + NADPH + H+ → 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al + H2O + NADP+
  51. Isomerization of 3,7,24THCA-CoA to (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA3,7,24THCA-CoA → 3,7,24THCA-CoA
  52. 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al is oxidized to 3alpha,7alpha,24(S)-trihydroxy-5beta-cholestanoate (3,7,24THCA)3alpha,7alpha,24(S)-trihydroxy-5beta-cholestan-27-al + NADPH + H+ + O2 → 3,7,24THCA + NADP+ + H2O
  53. 3,7,24THCA is conjugated with Coenzyme A (SLC27A2 VLCS)3,7,24THCA + ATP + CoA-SH → 3,7,24THCA-CoA + AMP + PPi + H2O
  54. 4-cholesten-7alpha,24(S)-diol-3-one is reduced to 5beta-cholestan-7alpha,24(S)-diol-3-one4CHOL7a,24(S)DONE + NADPH + H+ → 5beta-cholestan-7alpha,24(S)-diol-3-one + NADP+
  55. 3,7,24THCA-CoA is translocated from the cytosol to the peroxisome3,7,24THCA-CoA + ATP + H2O → 3,7,24THCA-CoA + ADP + H+ + Pi
  56. 5beta-cholestan-7alpha,24(S)-diol-3-one is reduced to 5beta-cholestan-3alpha,7alpha,24(S)-triol5beta-cholestan-7alpha,24(S)-diol-3-one + NADPH + H+ → 5beta-cholestan-3alpha,7alpha,24(S)-triol + NADP+
  57. Influx of 24-hydroxycholesterol24OH-CHOL → 24OH-CHOL
  58. Efflux of 24-hydroxycholesterol24OH-CHOL → 24OH-CHOL
  59. 3,7,24THCA is translocated from the mitochondrial matrix to the cytosol3,7,24THCA → 3,7,24THCA
  60. Cholest-5-ene-3beta,7alpha,24(S)-triol is oxidized and isomerized to 4-cholesten-7alpha,24(S)-diol-3-oneCHOL7a,24(S)DIOL + NAD+ → 4CHOL7a,24(S)DONE + NADH + H+
  61. CYP27A1 27-hydroxylates 5β-CHOL3α,7α,24(s)-triol5β-CHOL3α,7α,24(s)-triol + NADPH + H+ + O2 → 5bCHOL3a,7a,24(s),27-tetrol + H2O + NADP+
  62. 5Beta-cholestan-7alpha,12alpha,27-triol-3-one is reduced to 5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol5beta-cholestan-7alpha,12alpha,27-triol-3-one + NADPH + H+ → 5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol + NADP+
  63. Influx of 27-hydroxycholesterol27OH-CHOL → 27HCHOL
  64. 5beta-cholestan-3alpha,7alpha,27-triol is translocated from the cytosol to the mitochondrial matrix5beta-cholestan-3alpha,7alpha,26-triol → 5beta-cholestan-3alpha,7alpha,26-triol
  65. Efflux of 27-hydroxycholesterol27OH-CHOL → 27OH-CHOL
  66. Cholest-5-ene-3beta,7alpha,27-triol is oxidized and isomerized to 4-cholesten-7alpha,27-diol-3-oneCholest-5-ene-3beta,7alpha,27-triol + NAD+ → 4CHOL7a,27DONE + NADH + H+
  67. 4-cholesten-7alpha,12alpha,27-triol-3-one is reduced to 5beta-cholestan-7alpha,12alpha,27-triol-3-one4CHOL7a,12a,27TONE + NADPH + H+ → 5beta-cholestan-7alpha,12alpha,27-triol-3-one + NADP+
  68. 4-cholesten-7alpha,27-diol-3-one is reduced to 5beta-cholestan-7alpha,27-diol-3-one4CHOL7a,27DONE + NADPH + H+ → 5beta-cholestan-7alpha,27-diol-3-one + NADP+
  69. 5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol is translocated from the cytosol to the mitochondrial matrix5beta-cholestan-3alpha,7alpha,12alpha,27-tetrol → 5bCHOL3a,7a,12a,27-tetrol
  70. 5beta-cholestan-7alpha,27-diol-3-one is reduced to 5beta-cholestan-3alpha,7alpha,27-triol5beta-cholestan-7alpha,27-diol-3-one + NADPH + H+ → 5beta-cholestan-3alpha,7alpha,26-triol + NADP+
  71. CYP8B1 12-hydroxylates 4CHOL7a,27DONE4CHOL7a,27DONE + NADPH + H+ + O2 → 4CHOL7a,12a,27TONE + NADP+ + H2O
  72. SLCO1B3 transports ALB:(GCCA, TCCA) from extracellular region to cytosolGCCA, TCCA → GCCA, TCCA
  73. SLCO1B1 transports ALB:(GCCA,TCCA) from extracellular region to cytosolGCCA, TCCA → GCCA, TCCA
  74. SLC10A1 co-transport bile salts and Na+ from extracellular region to cytosol2 Na+ + bile salts and acids → 2 Na+ + bile salts and acids
  75. Transport (influx) of bile salts and acids by OATP-Abile salts and acids → bile salts and acids
  76. ABCC3 transports bile salts from cytosol to extracellular regionbile salts and acids + ATP → ADP + Pi + bile salts and acids
  77. SLC10A2 transports bile salts and acids and Na+ from extracellular region to cytosolNa+ + bile salts and acids → bile salts and acids + Na+
  78. NR1H4 binds DCA, CDCA, LCHADCA,CDCA,LCHA + NR1H4 → NR1H4:DCA,CDCA,LCHA
  79. NR1H4:DCA,CDCA,LCHA binds RXRA and NCOA1,2NR1H4:DCA,CDCA,LCHA + NCOA1,NCOA2,RXRA → NCOA1,2:RXRA:NR1H4:DCA,CDCA,LCHA
  80. OSBPs transport 25OH-CHOL from ER membrane to plasma membrane25OH-CHOL → 25OH-CHOL
  81. OSBP exchanges 25OH-CHOL with PI4P from ER membrane to Golgi membrane25OH-CHOL + PI4P → 25OH-CHOL + PI4P
  82. STARD5 binds DCA, LCASTARD5 + DCA, LCA → STARD5:DCA/LCA
  83. Bile salts and acids bind ALBALB + bile salts and acids → ALB:bile salts and acids
  84. Bile salts and acids dissociate from ALBALB:bile salts and acids → ALB + bile salts and acids
  85. SLC51A:SLC51B transports bile salts from cytosol to extracellular regionbile salts and acids → bile salts and acids
  86. Bile salts dissociate from FABP6bile salts and acids:FABP6 → bile salts and acids + FABP6
  87. Bile salts bind FABP6bile salts and acids + FABP6 → bile salts and acids:FABP6