PATHWAY: rn00120 Help
Entry Name Primary bile acid biosynthesis
Description Bile acids are steroid carboxylic acids derived from cholesterol in vertebrates. The primary bile acids, cholic acid and chenodeoxycholic acid, are synthesized in the liver and conjugated with taurine or glycine before secretion via bile into the intestine. The conversion from cholesterol to cholic and chenodeoxycholic acids involves four steps: 1) the initiation of synthesis by 7alpha-hydroxylation of sterol precursors, 2) further modifications to the ring structures, 3) side-chain oxidation and shortening (cleavage) by three carbons, and 4) conjugation of the bile acid with taurine or glycine.
Class Metabolism; Lipid metabolism
Pathway map rn00120 Primary bile acid biosynthesis
Module M00104 Bile acid biosynthesis, cholesterol => cholate/chenodeoxycholate [PATH:rn00120 ]
M00106 Conjugated bile acid biosynthesis, cholate => taurocholate/glycocholate [PATH:rn00120 ]
M00862 beta-Oxidation, peroxisome, tri/dihydroxycholestanoyl-CoA => choloyl/chenodeoxycholoyl-CoA [PATH:rn00120 ]
Reaction R01463 cholesterol,NADPH-hemoprotein reductase:oxygen oxidoreductase (7alpha-hydroxylating)
R02794 cholate:CoA ligase (AMP-forming)
R02797 taurocholate amidohydrolase
R03718 choloyl-CoA:glycine N-choloyltransferase
R03719 3alpha,7alpha,12alpha-trihydroxy-5beta-cholanoyl-CoA:propanoyl-CoA C-acyltransferase; choloyl-CoA:propanoyl-CoA C-acyltransferase
R03720 choloyl-CoA:glycine N-choloyltransferase
R03974 chenodeoxycholoyl-CoA hydrolase
R03975 glycochenodeoxycholate amidohydrolase
R03977 chenodeoxycholoyltaurine amidohydrolase
R04263 7alpha-hydroxycholesterol:NAD+ 3-oxidoreductase
R04809 (24R,25R)-3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA hydro-lyase [(24E)-3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA-forming]
R04810 (24R,25R)-3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA:NAD+ oxidoreductase
R04811 propanoyl-CoA:acetyl-CoA C-acyltransferase; 3alpha,7alpha-dihydroxy-5beta-cholanoyl-CoA:propanoyl-CoA; C-acyltransferase
R04812 (24R,25R)-3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA:NAD+ oxidoreductase
R04813 (24R,25R)-3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA hydro-lyase [(24E)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA-forming]
R04817 7alpha-hydroxy-5beta-cholestan-3-one:NADP+ delta4-oxidoreductase
R04818 3alpha,7alpha-dihydroxy-5beta-cholestane:NAD+ oxidoreductase (B-specific)
R04819 3alpha,7alpha-dihydroxy-5beta-cholestane:NADP+ oxidoreductase (B-specific)
R04823 7alpha,12alpha-dihydroxy-5beta-cholestan-3-one:NADP+ delta4-oxidoreductase
R04824 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestane:NAD+ oxidoreductase (B-specific)
R04825 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestane:NADP+ oxidoreductase (B-specific)
R04826 7alpha-hydroxycholest-4-en-3-one,[reduced NADPH---hemoprotein reductase]:oxygen oxidoreductase (12alpha-hydroxylating)
R05835 glycocholate amidohydrolase
R07204 5beta-cholestane-3alpha,7alpha-diol,[reduced NADPH---hemoprotein reductase]:oxygen oxidoreductase (12alpha-hydroxylating)
R07207 cholesterol,NADPH-hemoprotein reductase:oxygen oxidoreductase (24-hydroxylating)
R07208 (24S)-cholest-5-ene-3beta,24-diol,NADPH-hemoprotein reductase:oxygen oxidoreductase (7alpha-hydroxylating)
R07209 cholest-5-ene-3beta,25-diol,[NADPH---hemoprotein reductase]:oxygen oxidoreductase (7alpha-hydroxylating)
R07218 cholesterol,hydrogen-donor:oxygen oxidoreductase (25-hydroxylating)
R07372 cholest-5-ene-3beta,26-diol,[NADPH---hemoprotein reductase]:oxygen oxidoreductase (7alpha-hydroxylating)
R08505 cholesterol 26-hydroxylase
R08721 (24S)-cholest-5-ene-3beta,7alpha,24-triol:NAD+ 3-oxidoreductase
R08723 cholest-5-ene-3beta,7alpha,25-triol:NAD+ 3-oxidoreductase
R08724 7alpha,27-dihydroxycholesterol:NAD+ 3-oxidoreductase
R08727 3beta-hydroxy-5-cholestenoate,NADPH:oxygen oxidoreductase (7alpha-hydroxylating)
R08728 3beta,7alpha-dihydroxy-5-cholestenoate:NAD+ 3-oxidoreductase
R08733 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)
R08734 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoA 25-epimerase
R08735 (25S)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoA:acceptor 24,25-oxidoreductase
R08738 3alpha,7alpha-dihydroxy-5beta-cholestanate:CoA ligase (AMP-forming)
R08739 3alpha,7alpha-dihydroxy-5beta-cholestanoyl-CoA 25-epimerase
R08740 (25S)-3alpha,7alpha-dihydroxy-5beta-cholestanoyl-CoA:acceptor 24,25-oxidoreductase
R08743 chenodeoxycholate:CoA ligase (AMP-forming)
R08744 3alpha,7alpha-dihydroxy-5beta-cholanoyl-CoA:glycine N-choloyltransferase; chenodeoxycholoyl-CoA:glycine N-chenodeoxycholoyltransferase
R08745 3alpha,7alpha-dihydroxy-5beta-cholanoyl-CoA:taurine N-choloyltransferase; chenodeoxycholoyl-CoA:taurine N-chenodeoxycholoyltransferase
Compound C01301 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-al
C03594 7alpha-Hydroxycholesterol
C04554 3alpha,7alpha-Dihydroxy-5beta-cholestanate
C04722 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestanoate
C05444 3alpha,7alpha,26-Trihydroxy-5beta-cholestane
C05445 3alpha,7alpha-Dihydroxy-5beta-cholestan-26-al
C05446 3alpha,7alpha,12alpha,26-Tetrahydroxy-5beta-cholestane
C05447 (24E)-3alpha,7alpha-Dihydroxy-5beta-cholest-24-enoyl-CoA
C05448 (24R,25R)-3alpha,7alpha,24-Trihydroxy-5beta-cholestanoyl-CoA
C05449 3alpha,7alpha-Dihydroxy-5beta-24-oxocholestanoyl-CoA
C05451 7alpha-Hydroxy-5beta-cholestan-3-one
C05452 3alpha,7alpha-Dihydroxy-5beta-cholestane
C05453 7alpha,12alpha-Dihydroxy-5beta-cholestan-3-one
C05454 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestane
C05455 7alpha-Hydroxycholest-4-en-3-one
C05460 (24E)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholest-24-enoyl-CoA
C05467 3alpha,7alpha,12alpha-Trihydroxy-5beta-24-oxocholestanoyl-CoA
C06341 7alpha,27-Dihydroxycholesterol
C15518 (24S)-Cholest-5-ene-3beta,7alpha,24-triol
C15520 Cholest-5-ene-3beta,7alpha,25-triol
C15610 Cholest-5-ene-3beta,26-diol
C15613 (25R)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA
C15614 (24R,25R)-3alpha,7alpha,12alpha,24-Tetrahydroxy-5beta-cholestanoyl-CoA
C17331 7alpha,24-Dihydroxy-4-cholesten-3-one
C17332 7alpha,25-Dihydroxy-4-cholesten-3-one
C17333 3beta-Hydroxy-5-cholestenoate
C17335 3beta,7alpha-Dihydroxy-5-cholestenoate
C17336 7alpha,26-Dihydroxy-4-cholesten-3-one
C17337 7alpha-Hydroxy-3-oxo-4-cholestenoate
C17339 4-Cholesten-7alpha,12alpha-diol-3-one
C17343 (25S)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA
C17345 (25R)-3alpha,7alpha-Dihydroxy-5beta-cholestanoyl-CoA
C17346 (25S)-3alpha,7alpha-Dihydroxy-5beta-cholestanoyl-CoA
Reference Authors Russell DW
Title The enzymes, regulation, and genetics of bile acid synthesis.
Journal Reference Authors Norlin M, Wikvall K
Title Enzymes in the conversion of cholesterol into bile acids.
Journal Reference Authors Vlahcevic ZR, Pandak WM, Stravitz RT
Title Regulation of bile acid biosynthesis.
Journal Reference Authors Pellicoro A, Faber KN
Title Review article: The function and regulation of proteins involved in bile salt biosynthesis and transport.
Journal Reference Authors Hofmann AF, Hagey LR
Title Bile acids: chemistry, pathochemistry, biology, pathobiology, and therapeutics.
Journal Reference Authors Schwarz M, Wright AC, Davis DL, Nazer H, Bjorkhem I, Russell DW
Title The bile acid synthetic gene 3beta-hydroxy-Delta(5)-C(27)-steroid oxidoreductase is mutated in progressive intrahepatic cholestasis.
Journal Reference Authors Ridlon JM, Kang DJ, Hylemon PB
Title Bile salt biotransformations by human intestinal bacteria.
Journal Reference Authors Bortolini O, Medici A, Poli S
Title Biotransformations on steroid nucleus of bile acids.
Journal Related KO pathway
