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Entry
map00120                    Pathway                                

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
BRITE hierarchy
Pathway map
map00120  Primary bile acid biosynthesis
map00120

Ortholog table
Module
M00104  Bile acid biosynthesis, cholesterol => cholate/chenodeoxycholate [PATH:map00120]
M00106  Conjugated bile acid biosynthesis, cholate => taurocholate/glycocholate [PATH:map00120]
M00862  beta-Oxidation, peroxisome, tri/dihydroxycholestanoyl-CoA => choloyl/chenodeoxycholoyl-CoA [PATH:map00120]
Disease
H00151  Cerebrotendinous xanthomatosis
H00407  Peroxisomal beta-oxidation enzyme deficiency
H00628  Congenital bile acid synthesis defect
H00874  Leukoencephalopathy with dystonia and motor neuropathy
H02095  Perrault syndrome
H02098  D-bifunctional protein deficiency
H02099  Alpha-methylacyl-CoA racemase deficiency
Reference
  Authors
Russell DW
  Title
The enzymes, regulation, and genetics of bile acid synthesis.
  Journal
Annu Rev Biochem 72:137-74 (2003)
DOI:10.1146/annurev.biochem.72.121801.161712
Reference
  Authors
Norlin M, Wikvall K
  Title
Enzymes in the conversion of cholesterol into bile acids.
  Journal
Curr Mol Med 7:199-218 (2007)
DOI:10.2174/156652407780059168
Reference
  Authors
Vlahcevic ZR, Pandak WM, Stravitz RT
  Title
Regulation of bile acid biosynthesis.
  Journal
Gastroenterol Clin North Am 28:1-25, v (1999)
DOI:10.1016/S0889-8553(05)70041-8
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)
DOI:10.1111/j.1365-2036.2007.03522.x
Reference
  Authors
Hofmann AF, Hagey LR
  Title
Bile acids: chemistry, pathochemistry, biology, pathobiology, and therapeutics.
  Journal
Cell Mol Life Sci 65:2461-83 (2008)
DOI:10.1007/s00018-008-7568-6
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)
DOI:10.1172/JCI10902
Reference
  Authors
Ridlon JM, Kang DJ, Hylemon PB
  Title
Bile salt biotransformations by human intestinal bacteria.
  Journal
J Lipid Res 47:241-59 (2006)
DOI:10.1194/jlr.R500013-JLR200
Reference
PMID:9292932
  Authors
Bortolini O, Medici A, Poli S
  Title
Biotransformations on steroid nucleus of bile acids.
  Journal
Steroids 62:564-77 (1997)
DOI:10.1016/S0039-128X(97)00043-3
Related
pathway
map00100  Steroid biosynthesis
map00121  Secondary bile acid biosynthesis
KO pathway
ko00120   

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Pathway (3)   
   KEGG MODULE (3)   
Disease (7)   
   KEGG DISEASE (7)   
Chemical substance (47)   
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Chemical reaction (73)   
   KEGG ENZYME (19)   
   KEGG REACTION (54)   
Gene (21952)   
   KEGG ORTHOLOGY (18)   
   RefGene (21934)   
Literature (8)   
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