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3-alpha- hydroxysteroid dehydrogenase 3-oxo-5-beta- steroid 4-dehydrogenase Sterol 26-hydroxylase, mitochondrial Sterol 26-hydroxylase, mitochondrial Sterol 26-hydroxylase, mitochondrial Bile acyl-CoA synthetase Alpha- methylacyl- CoA racemase Peroxisomal acyl-coenzyme A oxidase 2 Peroxisomal multifunctional enzyme type 2 Non-specific lipid-transfer protein Bile acid-CoA:amino acid N- acyltransferase Bile acid-CoA:amino acid N- acyltransferase Bile acyl-CoA synthetase 3 beta- hydroxysteroid dehydrogenase type 7 Prostacyclin synthase Cholesterol 7-alpha- monooxygenase Lysosomal acid lipase/cholesteryl ester hydrolase 3-oxo-5-beta- steroid 4-dehydrogenase Sterol 26-hydroxylase, mitochondrial 3-alpha- hydroxysteroid dehydrogenase Sterol 26-hydroxylase, mitochondrial Sterol 26-hydroxylase, mitochondrial Bile acyl-CoA synthetase Alpha- methylacyl- CoA racemase Peroxisomal acyl-coenzyme A oxidase 2 Peroxisomal multifunctional enzyme type 2 Bile acid-CoA:amino acid N- acyltransferase Bile acid-CoA:amino acid N- acyltransferase Bile acyl-CoA synthetase Cytochrome P450 3A9 25- hydroxycholesterol 7-alpha- hydroxylase 25- hydroxycholesterol 7-alpha- hydroxylase 25- hydroxycholesterol 7-alpha- hydroxylase Cholesterol 7-alpha- monooxygenase 25- hydroxycholesterol 7-alpha- hydroxylase Sterol 26-hydroxylase, mitochondrial 25- hydroxycholesterol 7-alpha- hydroxylase 3 beta- hydroxysteroid dehydrogenase type 7 Peroxisomal multifunctional enzyme type 2 Non-specific lipid-transfer protein Cholesterol 25-hydroxylase 3 beta- hydroxysteroid dehydrogenase type 7 Peroxisomal multifunctional enzyme type 2 Cholesterol Cholesterol Cholesterol Taurochenodesoxycholic acid Chenodeoxycholic acid glycine conjugate Accumulation Chenodeoxycholic acid Lithocholyltaurine Lithocholic acid glycine conjugate Lithocholic acid Taurocholic acid Glycocholic acid Accumulation Cholic acid Taurodeoxycholic acid Deoxycholic acid glycine conjugate Deoxycholic acid Cholesterol CE(22:2(13Z,16Z)) CE(22:2(13Z,16Z)) 7a-Hydroxy-5b-cholestan-3-one NAD 3a,7a-Dihydroxy-5b-cholestane NADP 7a-Hydroxy-cholestene-3-one NADPH NADP NADPH O2 3 α,7 α,26-Trihydroxy-5β-cholestane NADP H2O 3a,7a-Dihydroxy-5b- cholestan-26-al 3α,7α-Dihydroxycoprostanic acid ATP CoA 3a,7a-Dihydroxy-5b- cholestanoyl-CoA PPi AMP 3a,7a-Dihydroxy-5b- cholestanoyl-CoA H2O 3a,7a-Dihydroxy-5b-cholest- 24-enoyl-CoA 3 α,7 α,24-Trihydroxy-5β- cholestanoyl-CoA Chenodeoxycholoyl-CoA H2O Propionyl-CoA Chenodeoxycholoyl-CoA CoA Taurine Glycine Glycocholic acid ATP Accumulation Cholic acid AMP PPi 7a-Hydroxycholesterol NAD NADH NADPH O2 7a,12a-Dihydroxy-cholestene- 3-one NADP H2O NADPH O2 NADP H2O H2O Palmitic acid NADPH 7a,12a-Dihydroxy-5b- cholestan-3-one NADP 5-b-Cholestane-3a ,7a ,12a-triol NADPH O2 27-Deoxy-5b-cyprinol NADP H2O NADP NADPH 5b-Cyprinol sulfate NADPH O2 3a,7a,12a-Trihydroxy-5b- cholestan-26-al NADP H2O 3α,7α,12α-Trihydroxy-5β- cholestanoic acid ATP CoA 3a,7a,12a-Trihydroxy-5b-24- oxocholestanoyl-CoA ADP PPi 3a,7a,12a-Trihydroxy-5b-24- oxocholestanoyl-CoA H2O 3a,7a,12a-Trihydroxy-5b- cholest-24-enoyl-CoA 3a,7a,12a,24-Tetrahydroxy- 5b-cholestanoyl-CoA NAD 3a,7a,12a-Trihydroxy-5b-24- oxocholestanoyl-CoA NADH Choloyl-CoA Taurine CoA Glycine CoA AMP PPi ATP CoA NADPH O2 24-Hydroxycholesterol NADP H2O NADPH O2 (24R)-Cholest-5-ene-3-β,7- α,24-triol NADP H2O 25-Hydroxycholesterol NADPH O2 7-a,25-Dihydroxycholesterol NADP H2O NADPH O2 27-Hydroxycholesterol NADP H2O NADPH O2 7-a,27-Dihydroxycholesterol NADP H2O NADPH O2 NADP H2O NADPH O2 3 β-Hydroxy-5-cholestenoate NADP H2O NADPH O2 3β,7α-Dihydroxy-5- cholestenoate NADP H2O NAD 7α-Hydroxy-3-oxo-4- cholestenoate NADH H2O CoA Propionyl-CoA O2 H2O NADP 7 α,26-Dihydroxy-4-cholesten- 3-one NADPH H2O Heme Heme Heme FAD Heme Heme Heme Heme Heme FAD Heme Heme Heme Heme Heme Heme Heme Heme Iron Acceptor Reduced acceptor AH2 A Steroid Biosynthesis Steroid Biosynthesis Steroid Biosynthesis Extracellular space Intracellular space Endoplasmic Reticurum Endoplasmic Reticurum Endoplamic Reticurum Endoplasmic Reticurum Mitochondria Liver Mitochondria Blood Vesicle Lysosome
Peroxisome Peroxisome Akr1c9 Akr1d1 Cyp27a1 Cyp27a1 Cyp27a1 Slc27a5 Amacr Acox2 Hsd17b4 Scp2 Baat Baat Slc27a5 Hsd3b7 Ptgis Cyp7a1 Lipa Akr1d1 Cyp27a1 Akr1c9 Cyp27a1 Cyp27a1 Slc27a5 Amacr Acox2 Hsd17b4 Baat Baat Slc27a5 Cyp3a9 Cyp7b1 Cyp7b1 Cyp7b1 Cyp7a1 Cyp7b1 Cyp27a1 Cyp7b1 Hsd3b7 Hsd17b4 Scp2 Ch25h Hsd3b7 Hsd17b4 Cholesterol Cholesterol Cholesterol Taurochenodesoxycholic acid Chenodeoxycholic acid glycine conjugate Accumulation Chenodeoxycholic acid Lithocholyltaurine Lithocholic acid glycine conjugate Lithocholic acid Taurocholic acid Glycocholic acid Accumulation Cholic acid Taurodeoxycholic acid Deoxycholic acid glycine conjugate Deoxycholic acid Cholesterol CE(22:2(13Z,16Z)) CE(22:2(13Z,16Z)) 7a-Hydroxy- 5b-cholestan- 3-one NAD 3a,7a- Dihydroxy-5b- cholestane NADP 7a-Hydroxy- cholestene-3- one NADPH NADP NADPH Oxygen 3 α,7 α,26- Trihydroxy- 5β-cholestane NADP Water 3a,7a- Dihydroxy-5b- cholestan-26- al 3α,7α- Dihydroxycoprostanic acid Adenosine triphosphate Coenzyme A 3a,7a- Dihydroxy-5b- cholestanoyl- CoA Pyrophosphate 3'-AMP 3a,7a- Dihydroxy-5b- cholestanoyl- CoA Water 3a,7a- Dihydroxy-5b- cholest-24- enoyl-CoA 3 α,7 α,24- Trihydroxy- 5β- cholestanoyl- CoA Chenodeoxycholoyl- CoA Water Propionyl-CoA Chenodeoxycholoyl- CoA Coenzyme A Taurine Glycine Glycocholic acid Adenosine triphosphate Accumulation Cholic acid 3'-AMP Pyrophosphate 7a- Hydroxycholesterol NAD NADH NADPH Oxygen 7a,12a- Dihydroxy- cholestene-3- one NADP Water NADPH Oxygen NADP Water Water Palmitic acid NADPH 7a,12a- Dihydroxy-5b- cholestan-3- one NADP 5-b- Cholestane-3a ,7a ,12a-triol NADPH Oxygen 27-Deoxy-5b- cyprinol NADP Water NADP NADPH 5b-Cyprinol sulfate NADPH Oxygen 3a,7a,12a- Trihydroxy- 5b-cholestan- 26-al NADP Water 3α,7α,12α- Trihydroxy- 5β- cholestanoic acid Adenosine triphosphate Coenzyme A 3a,7a,12a- Trihydroxy- 5b-24- oxocholestanoyl- CoA Adenosine diphosphate Pyrophosphate 3a,7a,12a- Trihydroxy- 5b-24- oxocholestanoyl- CoA Water 3a,7a,12a- Trihydroxy- 5b-cholest- 24-enoyl-CoA 3a,7a,12a,24- Tetrahydroxy- 5b- cholestanoyl- CoA NAD 3a,7a,12a- Trihydroxy- 5b-24- oxocholestanoyl- CoA NADH Choloyl-CoA Taurine Coenzyme A Glycine Coenzyme A 3'-AMP Pyrophosphate Adenosine triphosphate Coenzyme A NADPH Oxygen 24- Hydroxycholesterol NADP Water NADPH Oxygen (24R)- Cholest-5- ene-3-β,7- α,24-triol NADP Water 25- Hydroxycholesterol NADPH Oxygen 7-a,25- Dihydroxycholesterol NADP Water NADPH Oxygen 27- Hydroxycholesterol NADP Water NADPH Oxygen 7-a,27- Dihydroxycholesterol NADP Water NADPH Oxygen NADP Water NADPH Oxygen 3 β-Hydroxy-5- cholestenoate NADP Water NADPH Oxygen 3β,7α- Dihydroxy-5- cholestenoate NADP Water NAD 7α-Hydroxy-3- oxo-4- cholestenoate NADH Water Coenzyme A Propionyl-CoA Oxygen Water NADP 7 α,26- Dihydroxy-4- cholesten-3- one NADPH Water Acceptor Reduced acceptor AH2 A Steroid Biosynthesis Steroid Biosynthesis Steroid Biosynthesis Intestinal Microflora Intestinal Microflora
Akr1c9 Akr1d1 Cyp27a1 Cyp27a1 Cyp27a1 Slc27a5 Amacr Acox2 Hsd17b4 Scp2 Baat Baat Slc27a5 Hsd3b7 Ptgis Cyp7a1 Lipa Akr1d1 Cyp27a1 Akr1c9 Cyp27a1 Cyp27a1 Slc27a5 Amacr Acox2 Hsd17b4 Baat Baat Slc27a5 Cyp3a9 Cyp7b1 Cyp7b1 Cyp7b1 Cyp7a1 Cyp7b1 Cyp27a1 Cyp7b1 Hsd3b7 Hsd17b4 Scp2 Ch25h Hsd3b7 Hsd17b4 Lanol Lanol Lanol Taucoa Deoxych Accumulation Cheno Lithoch LitCAGC Lithoch Cholaic Glycoch Accumulation Cholate TDCA Deoxygl Dochoa Lanol 13,16-D 13,16-D 7HCone NAD 3a7aDhc NADP 7aHC3oe NADPH NADP NADPH O2 3α7t5βc NADP H2O 37aHCal 37a-Dca ATP CoA DHC-CoA PPi AMP DHC-CoA H2O d5ceCoA 37αT5CA ChendCA H2O PropCoA ChendCA CoA Taurine Gly Glycoch ATP Accumulation Cholate AMP PPi 7a-HCol NAD NADH NADPH O2 7DC3O NADP H2O NADPH O2 NADP H2O H2O 16:0 NADPH 7DHCone NADP 5C3712T NADPH O2 D5bCypl NADP H2O NADP NADPH 5bCypS NADPH O2 37THCAL NADP H2O Coproch ATP CoA toCOA ADP PPi toCOA H2O aTrCCoA aTC-CoA NAD toCOA NADH ChoylCA Taurine CoA Gly CoA AMP PPi ATP CoA NADPH O2 24-HCol NADP H2O NADPH O2 24RCiol NADP H2O 25-HC NADPH O2 7DHChol NADP H2O NADPH O2 27-HC NADP H2O NADPH O2 7a27Dih NADP H2O NADPH O2 NADP H2O NADPH O2 3βh5c NADP H2O NADPH O2 3β7αd5c NADP H2O NAD 7αH304C NADH H2O CoA PropCoA O2 H2O NADP Dchnone NADPH H2O Heme Heme Heme FAD Heme Heme Heme Heme Heme FAD Heme Heme Heme Heme Heme Heme Heme Heme Iron Accepto RA AH2 A PW000050 PW000050 PW000050 Extracellular space Intracellular space Endoplasmic Reticurum Endoplasmic Reticurum Endoplamic Reticurum Endoplasmic Reticurum Mitochondria Liver Mitochondria