| Entry |
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| Name |
Primary bile acid biosynthesis
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| 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.
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| Class |
Metabolism; Lipid metabolism
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| Pathway map |
| Primary bile acid biosynthesis |
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| Module |
| Bile acid biosynthesis, cholesterol => cholate [PATH: map00120] | | Conjugated bile acid biosynthesis, cholate => taurocholate/glycocholate [PATH: map00120] |
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| Disease |
| Cerebrotendinous xanthomatosis | | Peroxisomal beta-oxidation enzyme deficiency | | Familial cholestasis | | Congenital bile acid synthesis defect (CBAS) | | Leukoencephalopathy with dystonia and motor neuropathy |
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| Reference |
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| Authors |
Russell DW |
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| Title |
The enzymes, regulation, and genetics of bile acid synthesis. |
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| Journal |
Annu Rev Biochem 72:137-74 (2003) |
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| Reference |
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| Authors |
Norlin M, Wikvall K |
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| Title |
Enzymes in the conversion of cholesterol into bile acids. |
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| Journal |
Curr Mol Med 7:199-218 (2007) |
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| Reference |
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| Authors |
Vlahcevic ZR, Pandak WM, Stravitz RT |
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| Title |
Regulation of bile acid biosynthesis. |
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| Journal |
Gastroenterol Clin North Am 28:1-25, v (1999) |
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| Reference |
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| Authors |
Pellicoro A, Faber KN |
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| Title |
Review article: The function and regulation of proteins involved in bile salt biosynthesis and transport. |
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| Journal |
Aliment Pharmacol Ther 26 Suppl 2:149-60 (2007) |
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| Reference |
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| Authors |
Hofmann AF, Hagey LR |
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| Title |
Bile acids: chemistry, pathochemistry, biology, pathobiology, and therapeutics. |
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| Journal |
Cell Mol Life Sci 65:2461-83 (2008) |
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| Reference |
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| Authors |
Schwarz M, Wright AC, Davis DL, Nazer H, Bjorkhem I, Russell DW |
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| Title |
The bile acid synthetic gene 3beta-hydroxy-Delta(5)-C(27)-steroid oxidoreductase is mutated in progressive intrahepatic cholestasis. |
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| Journal |
J Clin Invest 106:1175-84 (2000) |
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| Reference |
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| Authors |
Ridlon JM, Kang DJ, Hylemon PB |
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| Title |
Bile salt biotransformations by human intestinal bacteria. |
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| Journal |
J Lipid Res 47:241-59 (2006) |
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| Reference |
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| Authors |
Bortolini O, Medici A, Poli S |
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| Title |
Biotransformations on steroid nucleus of bile acids. |
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| Journal |
Steroids 62:564-77 (1997) |
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| KO pathway |
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