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
PATHWAY_MAP hsa00120  Primary bile acid biosynthesis
MODULE      hsa_M00104  Bile acid biosynthesis, cholesterol => cholate/chenodeoxycholate [PATH:hsa00120]
            hsa_M00106  Conjugated bile acid biosynthesis, cholate => taurocholate/glycocholate [PATH:hsa00120]
            hsa_M00862  beta-Oxidation, peroxisome, tri/dihydroxycholestanoyl-CoA => choloyl/chenodeoxycholoyl-CoA [PATH:hsa00120]
NETWORK     nt06021  beta-Oxidation in peroxisome
            nt06022  Bile acid biosynthesis
  ELEMENT   N00779  beta-Oxidation, peroxisome, bile acid
            N00805  Bile acid biosynthesis
ORGANISM    Homo sapiens (human) [GN:hsa]
GENE        10858  CYP46A1; cytochrome P450 family 46 subfamily A member 1 [KO:K07440] [EC:1.14.14.25]
            51302  CYP39A1; cytochrome P450 family 39 subfamily A member 1 [KO:K07439] [EC:1.14.14.26]
            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 member 1 [KO:K07430] [EC:1.14.14.29]
            1581  CYP7A1; cytochrome P450 family 7 subfamily A member 1 [KO:K00489] [EC:1.14.14.23]
            1593  CYP27A1; cytochrome P450 family 27 subfamily A member 1 [KO:K00488] [EC:1.14.15.15]
            1582  CYP8B1; cytochrome P450 family 8 subfamily B member 1 [KO:K07431] [EC:1.14.14.139]
            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] [EC:1.1.1.50 1.1.1.357 1.1.1.225]
            10998  SLC27A5; solute carrier family 27 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 [KO:K10214] [EC:1.17.99.3]
            3295  HSD17B4; hydroxysteroid 17-beta dehydrogenase 4 [KO:K12405] [EC:1.1.1.- 4.2.1.107 4.2.1.119]
            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 [KO:K00659] [EC:2.3.1.65 3.1.2.2]
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  (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
            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
            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   PMID:12543708
  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   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)
            DOI:10.2174/156652407780059168
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)
            DOI:10.1016/S0889-8553(05)70041-8
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)
            DOI:10.1111/j.1365-2036.2007.03522.x
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)
            DOI:10.1007/s00018-008-7568-6
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)
            DOI:10.1172/JCI10902
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)
            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
REL_PATHWAY hsa00100  Steroid biosynthesis
KO_PATHWAY  ko00120
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