KEGG   PATHWAY: hsa00120Help
Entry
hsa00120                    Pathway                                
Name
Primary bile acid biosynthesis - Homo sapiens (human)
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
BRITE hierarchy
Pathway map
Primary bile acid biosynthesis
hsa00120

All organismsOrtholog table
Module
Bile acid biosynthesis, cholesterol => cholate [PATH:hsa00120]
Conjugated bile acid biosynthesis, cholate => taurocholate/glycocholate [PATH:hsa00120]
Disease
H00151  
Cerebrotendinous xanthomatosis
H00407  
Peroxisomal beta-oxidation enzyme deficiency
H00624  
Familial cholestasis
H00628  
Congenital bile acid synthesis defect (CBAS)
H00874  
Leukoencephalopathy with dystonia and motor neuropathy
Organism
Homo sapiens (human) [GN:hsa]
Gene
10858  
CYP46A1; cytochrome P450, family 46, subfamily A, polypeptide 1 [KO:K07440] [EC:1.14.13.98]
51302  
CYP39A1; cytochrome P450, family 39, subfamily A, polypeptide 1 [KO:K07439] [EC:1.14.13.99]
80270  
HSD3B7; hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 7 [KO:K12408] [EC:1.1.1.181]
9023  
CH25H; cholesterol 25-hydroxylase [KO:K10223] [EC:1.14.99.38]
9420  
CYP7B1; cytochrome P450, family 7, subfamily B, polypeptide 1 [KO:K07430] [EC:1.14.13.100]
1581  
CYP7A1; cytochrome P450, family 7, subfamily A, polypeptide 1 [KO:K00489] [EC:1.14.13.17]
1593  
CYP27A1; cytochrome P450, family 27, subfamily A, polypeptide 1 [KO:K00488] [EC:1.14.13.15]
1582  
CYP8B1; cytochrome P450, family 8, subfamily B, polypeptide 1 [KO:K07431] [EC:1.14.13.95]
6718  
AKR1D1; aldo-keto reductase family 1, member D1 [KO:K00251] [EC:1.3.1.3]
1109  
AKR1C4; aldo-keto reductase family 1, member C4 [KO:K00037 K00089 K00092 K00212] [EC:1.1.1.50 1.1.1.213 1.1.1.225 1.3.1.20]
10998  
SLC27A5; solute carrier family 27 (fatty acid transporter), member 5 [KO:K08748] [EC:6.2.1.7]
23600  
AMACR; alpha-methylacyl-CoA racemase [KO:K01796] [EC:5.1.99.4]
8309  
ACOX2; acyl-CoA oxidase 2, branched chain [KO:K10214] [EC:1.17.99.3]
3295  
HSD17B4; hydroxysteroid (17-beta) dehydrogenase 4 [KO:K12405] [EC:4.2.1.107 1.1.1.35]
6342  
SCP2; sterol carrier protein 2 [KO:K08764] [EC:2.3.1.176]
10005  
ACOT8; acyl-CoA thioesterase 8 [KO:K11992] [EC:3.1.2.27]
570  
BAAT; bile acid CoA: amino acid N-acyltransferase (glycine N-choloyltransferase) [KO:K00659] [EC:3.1.2.2 2.3.1.65]
Compound
C00037  
Glycine
C00187  
Cholesterol
C00245  
Taurine
C00695  
Cholic acid
C01301  
3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-al
C01794  
Choloyl-CoA
C01921  
Glycocholate
C02528  
Chenodeoxycholate
C03594  
7alpha-Hydroxycholesterol
C04554  
3alpha,7alpha-Dihydroxy-5beta-cholestanate
C04722  
3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestanoate
C05122  
Taurocholate
C05337  
Chenodeoxycholoyl-CoA
C05444  
3alpha,7alpha,26-Trihydroxy-5beta-cholestane
C05445  
3alpha,7alpha-Dihydroxy-5beta-cholestan-26-al
C05446  
3alpha,7alpha,12alpha,26-Tetrahydroxy-5beta-cholestane
C05447  
3alpha,7alpha-Dihydroxy-5beta-cholest-24-enoyl-CoA
C05448  
3alpha,7alpha,24-Trihydroxy-5beta-cholestanoyl-CoA
C05449  
3alpha,7alpha-Dihydroxy-5beta-24-oxocholestanoyl-CoA
C05450  
3alpha,7alpha,12alpha,24-Tetrahydroxy-5beta-cholestanoyl-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  
3alpha,7alpha,12alpha-Trihydroxy-5beta-cholest-24-enoyl-CoA
C05465  
Taurochenodeoxycholate
C05466  
Glycochenodeoxycholate
C05467  
3alpha,7alpha,12alpha-Trihydroxy-5beta-24-oxocholestanoyl-CoA
C05468  
5beta-Cyprinolsulfate
C06341  
7alpha,27-Dihydroxycholesterol
C13550  
Cerebrosterol
C15518  
(24S)-Cholest-5-ene-3beta,7alpha,24-triol
C15519  
25-Hydroxycholesterol
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
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
Annu Rev Biochem 72:137-74 (2003)
Reference
  Authors
Norlin M, Wikvall K
  Title
Enzymes in the conversion of cholesterol into bile acids.
  Journal
Curr Mol Med 7:199-218 (2007)
Reference
  Authors
Vlahcevic ZR, Pandak WM, Stravitz RT
  Title
Regulation of bile acid biosynthesis.
  Journal
Gastroenterol Clin North Am 28:1-25, v (1999)
Reference
  Authors
Pellicoro A, Faber KN
  Title
Review article: The function and regulation of proteins involved in bile salt biosynthesis and transport.
  Journal
Aliment Pharmacol Ther 26 Suppl 2:149-60 (2007)
Reference
  Authors
Hofmann AF, Hagey LR
  Title
Bile acids: chemistry, pathochemistry, biology, pathobiology, and therapeutics.
  Journal
Cell Mol Life Sci 65:2461-83 (2008)
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
J Clin Invest 106:1175-84 (2000)
Reference
  Authors
Ridlon JM, Kang DJ, Hylemon PB
  Title
Bile salt biotransformations by human intestinal bacteria.
  Journal
J Lipid Res 47:241-59 (2006)
Reference
PMID:9292932
  Authors
Bortolini O, Medici A, Poli S
  Title
Biotransformations on steroid nucleus of bile acids.
  Journal
Steroids 62:564-77 (1997)
KO pathway
 
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