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Record Information
StatusDetected and Quantified
Creation Date2006-08-13 06:26:12 UTC
Update Date2018-03-20 18:27:30 UTC
Secondary Accession Numbers
  • HMDB03873
Metabolite Identification
Common Name3a,7a,12a-Trihydroxy-5b-cholestanoic acid
Description3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestanoic acid is a bile acid. Bile acids are steroid acids found predominantly in bile of mammals. The distinction between different bile acids is minute, depends only on presence or absence of hydroxyl groups on positions 3, 7, and 12. Bile acids are physiological detergents that facilitate excretion, absorption, and transport of fats and sterols in the intestine and liver. Bile acids are also steroidal amphipathic molecules derived from the catabolism of cholesterol. They modulate bile flow and lipid secretion, are essential for the absorption of dietary fats and vitamins, and have been implicated in the regulation of all the key enzymes involved in cholesterol homeostasis. Bile acids recirculate through the liver, bile ducts, small intestine and portal vein to form an enterohepatic circuit. They exist as anions at physiological pH and, consequently, require a carrier for transport across the membranes of the enterohepatic tissues. The unique detergent properties of bile acids are essential for the digestion and intestinal absorption of hydrophobic nutrients. Bile acids have potent toxic properties (e.g., membrane disruption) and there are a plethora of mechanisms to limit their accumulation in blood and tissues. 3a,7a,12a-Trihydroxy-5b-cholestanoic acid is found to be associated with infantile Refsum disease, which is an inborn error of metabolism.
(3alpha,5beta,7alpha,12alpha,25R)-3,7,12-Trihydroxycholestan-26-Oic acidChEBI
(3a,5b,7a,12a,25R)-3,7,12-Trihydroxycholestan-26-Oic acidGenerator
(3α,5β,7α,12α,25R)-3,7,12-trihydroxycholestan-26-Oic acidGenerator
(25R)-3-alpha,7-alpha,12-alpha-Trihydroxy-5-beta-cholestan-26-Oic acidHMDB
3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestanoic acidHMDB
Chemical FormulaC27H46O5
Average Molecular Weight450.6511
Monoisotopic Molecular Weight450.334524582
IUPAC Name(2R,6R)-2-methyl-6-[(1S,2S,5R,7S,9R,10R,11S,14R,15R,16S)-5,9,16-trihydroxy-2,15-dimethyltetracyclo[²,⁷.0¹¹,¹⁵]heptadecan-14-yl]heptanoic acid
Traditional Name(2R,6R)-2-methyl-6-[(1S,2S,5R,7S,9R,10R,11S,14R,15R,16S)-5,9,16-trihydroxy-2,15-dimethyltetracyclo[²,⁷.0¹¹,¹⁵]heptadecan-14-yl]heptanoic acid
CAS Registry Number547-98-8
InChI Identifier
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as trihydroxy bile acids, alcohols and derivatives. These are prenol lipids structurally characterized by a bile acid or alcohol which bears three hydroxyl groups.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassSteroids and steroid derivatives
Sub ClassBile acids, alcohols and derivatives
Direct ParentTrihydroxy bile acids, alcohols and derivatives
Alternative Parents
  • Trihydroxy bile acid, alcohol, or derivatives
  • Steroid acid
  • 3-hydroxysteroid
  • 12-hydroxysteroid
  • 7-hydroxysteroid
  • 3-alpha-hydroxysteroid
  • Hydroxysteroid
  • Medium-chain fatty acid
  • Hydroxy fatty acid
  • Fatty acyl
  • Fatty acid
  • Cyclic alcohol
  • Secondary alcohol
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Polyol
  • Organooxygen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organic oxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic homopolycyclic compound
Molecular FrameworkAliphatic homopolycyclic compounds
External Descriptors
  • 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oic acid (CHEBI:48043 )
  • C27 bile acids, alcohols, and derivatives (LMST04030151 )
Physiological effect

Health effect:

  Health condition:


Naturally occurring process:

  Biological process:

    Biochemical pathway:

    Cellular process:

    Chemical reaction:

    Biochemical process:


Biological location:

  Biofluid and excreta:



  Tissue and substructures:

  Cell and elements:

Route of exposure:




Industrial application:

Biological role:

  Molecular messenger:

Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility0.022 g/LALOGPS
pKa (Strongest Acidic)4.62ChemAxon
pKa (Strongest Basic)-0.16ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area97.99 ŲChemAxon
Rotatable Bond Count6ChemAxon
Refractivity124.57 m³·mol⁻¹ChemAxon
Polarizability53.21 ųChemAxon
Number of Rings4ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0a4i-0353900000-c3bc744526d90b133841View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positivesplash10-0udi-1341349000-6c72d8109d5d5eae56d4View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00lr-0001900000-adf6831ff2e0a9c5ca12View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-015i-0018900000-a785d8993c7e11e2a341View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4r-1309300000-695e056a38badf6cf6e5View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0002-0000900000-e61f4254ed98eb0cde09View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-001s-0003900000-c0a96180a836b10b4d77View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-059i-9008700000-6d14292657023708cb78View in MoNA
Biological Properties
Cellular Locations
  • Extracellular
Biofluid Locations
  • Blood
Tissue LocationNot Available
27-Hydroxylase DeficiencyThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Bile Acid BiosynthesisThumbThumb?image type=greyscaleThumb?image type=simpleMap00120
Cerebrotendinous Xanthomatosis (CTX)ThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Congenital Bile Acid Synthesis Defect Type IIThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Congenital Bile Acid Synthesis Defect Type IIIThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Normal Concentrations
BloodDetected and Quantified0 uMChildren (1 - 13 years old)BothNormal details
Abnormal Concentrations
BloodDetected and Quantified0.76 uMChildren (1-13 years old)BothInfantile Refsum disease details
Associated Disorders and Diseases
Disease References
Infantile Refsum's disease
  1. Poll-The BT, Saudubray JM, Ogier H, Schutgens RB, Wanders RJ, Schrakamp G, van den Bosch H, Trijbels JM, Poulos A, Moser HW, et al.: Infantile Refsum's disease: biochemical findings suggesting multiple peroxisomal dysfunction. J Inherit Metab Dis. 1986;9(2):169-74. [PubMed:2427795 ]
Associated OMIM IDs
  • 266510 (Infantile Refsum's disease)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB023241
KNApSAcK IDNot Available
Chemspider ID21865494
KEGG Compound IDC04722
BioCyc IDCPD-1836
BiGG ID44407
Wikipedia LinkNot Available
PubChem Compound23657849
PDB IDNot Available
ChEBI ID48043
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Mihalik SJ, Steinberg SJ, Pei Z, Park J, Kim DG, Heinzer AK, Dacremont G, Wanders RJ, Cuebas DA, Smith KD, Watkins PA: Participation of two members of the very long-chain acyl-CoA synthetase family in bile acid synthesis and recycling. J Biol Chem. 2002 Jul 5;277(27):24771-9. Epub 2002 Apr 29. [PubMed:11980911 ]

Only showing the first 10 proteins. There are 11 proteins in total.


General function:
Involved in monooxygenase activity
Specific function:
Catalyzes the first step in the oxidation of the side chain of sterol intermediates; the 27-hydroxylation of 5-beta-cholestane-3-alpha,7-alpha,12-alpha-triol. Has also a vitamin D3-25-hydroxylase activity.
Gene Name:
Uniprot ID:
Molecular weight:
5-b-Cholestane-3a ,7a ,12a-triol + NADPH + Oxygen → 3a,7a,12a-Trihydroxy-5b-cholestanoic acid + NADP + Waterdetails
3a,7a,12a-Trihydroxy-5b-cholestan-26-al + NADPH + Oxygen + Hydrogen Ion → 3a,7a,12a-Trihydroxy-5b-cholestanoic acid + NADP + Waterdetails
General function:
Involved in catalytic activity
Specific function:
Acyl-CoA synthetase involved in bile acid metabolism. Proposed to catalyze the first step in the conjugation of C24 bile acids (choloneates) to glycine and taurine before excretion into bile canaliculi by activating them to their CoA thioesters. Seems to activate secondary bile acids entering the liver from the enterohepatic circulation. In vitro, also activates 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanate (THCA), the C27 precursor of cholic acid deriving from the de novo synthesis from cholesterol.
Gene Name:
Uniprot ID:
Molecular weight:
Adenosine triphosphate + 3a,7a,12a-Trihydroxy-5b-cholestanoic acid + Coenzyme A → Adenosine monophosphate + Pyrophosphate + (25R)-3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoAdetails
Adenosine triphosphate + 3a,7a,12a-Trihydroxy-5b-cholestanoic acid + Coenzyme A → Adenosine monophosphate + Pyrophosphate + 3a,7a,12a-Trihydroxy-5b-cholestanoyl-CoAdetails
3a,7a,12a-Trihydroxy-5b-cholestanoic acid + Adenosine triphosphate + Coenzyme A → (25R)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA + Adenosine monophosphate + Pyrophosphatedetails
General function:
Involved in binding
Specific function:
Ileal protein which stimulates gastric acid and pepsinogen secretion. Seems to be able to bind to bile salts and bilirubins. Isoform 2 is essential for the survival of colon cancer cells to bile acid-induced apoptosis
Gene Name:
Uniprot ID:
Molecular weight:
  1. Kurz M, Brachvogel V, Matter H, Stengelin S, Thuring H, Kramer W: Insights into the bile acid transportation system: the human ileal lipid-binding protein-cholyltaurine complex and its comparison with homologous structures. Proteins. 2003 Feb 1;50(2):312-28. [PubMed:12486725 ]


General function:
Involved in transporter activity
Specific function:
Mediates the Na(+)-independent transport of organic anions such as 17-beta-glucuronosyl estradiol, taurocholate, triiodothyronine (T3), leukotriene C4, dehydroepiandrosterone sulfate (DHEAS), methotrexate and sulfobromophthalein (BSP)
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in transporter activity
Specific function:
Mediates the Na(+)-independent transport of organic anions such as pravastatin, taurocholate, methotrexate, dehydroepiandrosterone sulfate, 17-beta-glucuronosyl estradiol, estrone sulfate, prostaglandin E2, thromboxane B2, leukotriene C3, leukotriene E4, thyroxine and triiodothyronine. May play an important role in the clearance of bile acids and organic anions from the liver
Gene Name:
Uniprot ID:
Molecular weight:
  1. Michalski C, Cui Y, Nies AT, Nuessler AK, Neuhaus P, Zanger UM, Klein K, Eichelbaum M, Keppler D, Konig J: A naturally occurring mutation in the SLC21A6 gene causing impaired membrane localization of the hepatocyte uptake transporter. J Biol Chem. 2002 Nov 8;277(45):43058-63. Epub 2002 Aug 23. [PubMed:12196548 ]
General function:
Involved in ATP binding
Specific function:
May act as an inducible transporter in the biliary and intestinal excretion of organic anions. Acts as an alternative route for the export of bile acids and glucuronides from cholestatic hepatocytes
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in ATP binding
Specific function:
Involved in the ATP-dependent secretion of bile salts into the canaliculus of hepatocytes
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in bile acid:sodium symporter activity
Specific function:
Plays a critical role in the sodium-dependent reabsorption of bile acids from the lumen of the small intestine. Plays a key role in cholesterol metabolism
Gene Name:
Uniprot ID:
Molecular weight:
  1. Kramer W, Girbig F, Glombik H, Corsiero D, Stengelin S, Weyland C: Identification of a ligand-binding site in the Na+/bile acid cotransporting protein from rabbit ileum. J Biol Chem. 2001 Sep 21;276(38):36020-7. Epub 2001 Jul 10. [PubMed:11447228 ]
General function:
Involved in bile acid:sodium symporter activity
Specific function:
The hepatic sodium/bile acid uptake system exhibits broad substrate specificity and transports various non-bile acid organic compounds as well. It is strictly dependent on the extracellular presence of sodium.
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in transporter activity
Specific function:
Mediates the Na(+)-independent transport of organic anions such as sulfobromophthalein (BSP) and conjugated (taurocholate) and unconjugated (cholate) bile acids
Gene Name:
Uniprot ID:
Molecular weight:
General function:
Involved in transporter activity
Specific function:
Mediates the Na(+)-independent transport of organic anions such as the thyroid hormones T3 (triiodo-L-thyronine), T4 (thyroxine) and rT3, and of estrone-3-sulfate and taurocholate
Gene Name:
Uniprot ID:
Molecular weight:

Only showing the first 10 proteins. There are 11 proteins in total.