KEGG PATHWAY: ko00120

PATHWAY: ko00120

Entry

ko00120 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

Pathway map

ko00120

Primary bile acid biosynthesis

Module

M00104	Bile acid biosynthesis, cholesterol => cholate [PATH:ko00120]
M00106	Conjugated bile acid biosynthesis, cholate => taurocholate/glycocholate [PATH:ko00120]

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

Orthology

K07440	cytochrome P450, family 46, subfamily A (cholesterol 24(S)-hydroxylase) [EC:1.14.13.98]
K07439	cytochrome P450, family 39, subfamily A (24-hydroxycholesterol 7alpha-hydroxylase) [EC:1.14.13.99]
K12408	cholest-5-ene-3beta,7alpha-diol 3beta-dehydrogenase [EC:1.1.1.181]
K10223	cholesterol 25-hydroxylase [EC:1.14.99.38]
K07430	cytochrome P450, family 7, subfamily B (oxysterol 7-alpha-hydroxylase) [EC:1.14.13.100]
K00489	cytochrome P450, family 7, subfamily A (cholesterol 7alpha-monooxygenase) [EC:1.14.13.17]
K00488	cytochrome P450, family 27, subfamily A (cholestanetriol 26-monooxygenase) [EC:1.14.13.15]
K07431	cytochrome P450, family 8, subfamily B (sterol 12-alpha-hydroxylase) [EC:1.14.13.95]
K00251	3-oxo-5-beta-steroid 4-dehydrogenase [EC:1.3.1.3]
K00037	3-alpha-hydroxysteroid dehydrogenase [EC:1.1.1.50]
K08748	solute carrier family 27 (fatty acid transporter), member 5 [EC:6.2.1.7]
K01796	alpha-methylacyl-CoA racemase [EC:5.1.99.4]
K10214	3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase [EC:1.17.99.3]
K12405	3-hydroxyacyl-CoA dehydrogenase / 3a,7a,12a-trihydroxy-5b-cholest-24-enoyl-CoA hydratase [EC:4.2.1.107 1.1.1.35]
K08764	sterol carrier protein 2 [EC:2.3.1.176]
K11992	acyl-CoA thioesterase 8 [EC:3.1.2.27]
K00659	bile acid-CoA:amino acid N-acyltransferase [EC:3.1.2.2 2.3.1.65]
K01442	choloylglycine hydrolase [EC:3.5.1.24]

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

PMID:12543708

Authors

Russell DW

Title

The enzymes, regulation, and genetics of bile acid synthesis.

Journal

Annu Rev Biochem 72:137-74 (2003)

Reference

PMID:17346171

Authors

Norlin M, Wikvall K

Title

Enzymes in the conversion of cholesterol into bile acids.

Journal

Curr Mol Med 7:199-218 (2007)

Reference

PMID:10198776

Authors

Vlahcevic ZR, Pandak WM, Stravitz RT

Title

Regulation of bile acid biosynthesis.

Journal

Gastroenterol Clin North Am 28:1-25, v (1999)

Reference

PMID:18081658

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

PMID:18488143

Authors

Hofmann AF, Hagey LR

Title

Bile acids: chemistry, pathochemistry, biology, pathobiology, and therapeutics.

Journal

Cell Mol Life Sci 65:2461-83 (2008)

Reference

PMID:11067870

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

PMID:16299351

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)

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Ontology (1)   
   KEGG BRITE (1)   
Pathway (6)   
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   KEGG MODULE (2)   
   BioSystems (1)   
Disease (5)   
   KEGG DISEASE (5)   
Chemical substance (47)   
   KEGG COMPOUND (47)   
Chemical reaction (20)   
   KEGG ENZYME (20)   
Gene (18)   
   KEGG ORTHOLOGY (18)   
Literature (8)   
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