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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-04-29 16:51:41 UTC
HMDB IDHMDB0000601
Secondary Accession Numbers
  • HMDB0003873
  • HMDB00601
  • HMDB03873
Metabolite Identification
Common NameCoprocholic acid
DescriptionCoprocholic acid, also called 3α,7α,12α-Trihydroxy-5β-cholestan-26-oic acid, is a bile acid. Bile acids are steroid acids found predominantly in the bile of mammals. The distinction between different bile acids is minute, depending only on the 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 the 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 (PMID: 11316487 , 16037564 , 12576301 , 11907135 ).
Structure
Data?1547234088
SynonymsNot Available
Chemical FormulaC27H46O5
Average Molecular Weight450.6511
Monoisotopic Molecular Weight450.334524582
IUPAC NameNot Available
Traditional NameNot Available
CAS Registry Number547-98-8
SMILESNot Available
InChI Identifier
InChI=1S/C27H46O5/c1-15(6-5-7-16(2)25(31)32)19-8-9-20-24-21(14-23(30)27(19,20)4)26(3)11-10-18(28)12-17(26)13-22(24)29/h15-24,28-30H,5-14H2,1-4H3,(H,31,32)/t15-,16?,17+,18-,19-,20+,21+,22-,23+,24+,26+,27-/m1/s1
InChI KeyCNWPIIOQKZNXBB-VCVMUKOKSA-N
Chemical Taxonomy
ClassificationNot classified
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Biological location:

Source:

Process

Naturally occurring process:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted PropertiesNot Available
Spectra
Spectrum TypeDescriptionSplash KeyView
Biological Properties
Cellular Locations
  • Extracellular
Biospecimen Locations
  • Blood
Tissue Locations
  • Gall Bladder
  • Intestine
  • Kidney
  • Liver
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified<0.1 uMInfant (0-1 year old)Not SpecifiedNormal details
BloodDetected and Quantified0 uMChildren (1 - 13 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.76 uMChildren (1-13 years old)BothInfantile Refsum disease details
BloodDetected and Quantified0 uMNewborn (0-30 days old)Not Specified
Neonatal adrenoleukodystrophy
details
BloodDetected and Quantified0.013 uMNewborn (0-30 days old)Female
Rhizomelic chondrodysplasia punctata
details
BloodDetected and Quantified0.078 uMInfant (0-1 year old)Male
Peroxisomal biogenesis disorder
details
BloodDetected and Quantified1.15 uMNewborn (0-30 days old)Not Specified
Zellweger syndrome
details
BloodDetected and Quantified0-3.812 uMChildren (1-13 years old)Female
Peroxisomal biogenesis disorder
details
BloodDetected and Quantified0.0290-1.0400 uMNewborn (0-30 days old)Female
Peroxisomal biogenesis disorder
details
BloodDetected and Quantified0.719 - 0.828 uMChildren (1 - 13 years old)MaleClassicle Refsum's disease details
BloodDetected and Quantified13.4 uMInfant (0-1 year old)Not SpecifiedD-Bifunctional Protein Deficiency 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 ]
Rhizomelic chondrodysplasia punctata
  1. Baumgartner MR, Poll-The BT, Verhoeven NM, Jakobs C, Espeel M, Roels F, Rabier D, Levade T, Rolland MO, Martinez M, Wanders RJ, Saudubray JM: Clinical approach to inherited peroxisomal disorders: a series of 27 patients. Ann Neurol. 1998 Nov;44(5):720-30. [PubMed:9818927 ]
Peroxisomal biogenesis defect
  1. Baumgartner MR, Poll-The BT, Verhoeven NM, Jakobs C, Espeel M, Roels F, Rabier D, Levade T, Rolland MO, Martinez M, Wanders RJ, Saudubray JM: Clinical approach to inherited peroxisomal disorders: a series of 27 patients. Ann Neurol. 1998 Nov;44(5):720-30. [PubMed:9818927 ]
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 ]
D-Bifunctional protein deficiency
  1. Rizzo C, Boenzi S, Wanders RJ, Duran M, Caruso U, Dionisi-Vici C: Characteristic acylcarnitine profiles in inherited defects of peroxisome biogenesis: a novel tool for screening diagnosis using tandem mass spectrometry. Pediatr Res. 2003 Jun;53(6):1013-8. doi: 10.1203/01.PDR.0000064902.59052.0F. Epub 2003 Mar 19. [PubMed:12646728 ]
Associated OMIM IDs
  • 266510 (Infantile Refsum's disease)
  • 215100 (Rhizomelic chondrodysplasia punctata)
  • 214100 (Peroxisomal biogenesis defect)
  • 266500 (Refsum's disease)
  • 261515 (D-Bifunctional protein deficiency)
External LinksNot Available
References
Synthesis ReferenceBatta A K; Mirchandani R; Salen G; Shefer S Synthesis of 3 alpha, 7 alpha-dihydroxy-5 beta-cholestan-26-oic acid from 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestan-26-oic acid: configuration in the bile of Alligator mississippiensis. Steroids (1992), 57(4), 162-6.
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 ]
  2. St-Pierre MV, Kullak-Ublick GA, Hagenbuch B, Meier PJ: Transport of bile acids in hepatic and non-hepatic tissues. J Exp Biol. 2001 May;204(Pt 10):1673-86. [PubMed:11316487 ]
  3. Claudel T, Staels B, Kuipers F: The Farnesoid X receptor: a molecular link between bile acid and lipid and glucose metabolism. Arterioscler Thromb Vasc Biol. 2005 Oct;25(10):2020-30. Epub 2005 Jul 21. [PubMed:16037564 ]
  4. Chiang JY: Bile acid regulation of hepatic physiology: III. Bile acids and nuclear receptors. Am J Physiol Gastrointest Liver Physiol. 2003 Mar;284(3):G349-56. [PubMed:12576301 ]
  5. Davis RA, Miyake JH, Hui TY, Spann NJ: Regulation of cholesterol-7alpha-hydroxylase: BAREly missing a SHP. J Lipid Res. 2002 Apr;43(4):533-43. [PubMed:11907135 ]

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

Enzymes

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:
CYP27A1
Uniprot ID:
Q02318
Molecular weight:
60234.28
Reactions
5-b-Cholestane-3a ,7a ,12a-triol + NADPH + Oxygen → Coprocholic acid + NADP + Waterdetails
3a,7a,12a-Trihydroxy-5b-cholestan-26-al + NADPH + Oxygen + Hydrogen Ion → Coprocholic 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:
SLC27A5
Uniprot ID:
Q9Y2P5
Molecular weight:
75384.375
Reactions
Adenosine triphosphate + Coprocholic acid + Coenzyme A → Adenosine monophosphate + Pyrophosphate + (25R)-3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoAdetails
Adenosine triphosphate + Coprocholic acid + Coenzyme A → Adenosine monophosphate + Pyrophosphate + 3a,7a,12a-Trihydroxy-5b-cholestanoyl-CoAdetails
Coprocholic 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:
FABP6
Uniprot ID:
P51161
Molecular weight:
14371.2
References
  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 ]

Transporters

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:
SLCO1B3
Uniprot ID:
Q9NPD5
Molecular weight:
77402.2
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:
SLCO1B1
Uniprot ID:
Q9Y6L6
Molecular weight:
76448.0
References
  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:
ABCC3
Uniprot ID:
O15438
Molecular weight:
169341.1
General function:
Involved in ATP binding
Specific function:
Involved in the ATP-dependent secretion of bile salts into the canaliculus of hepatocytes
Gene Name:
ABCB11
Uniprot ID:
O95342
Molecular weight:
146405.8
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:
SLC10A2
Uniprot ID:
Q12908
Molecular weight:
37697.4
References
  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:
SLC10A1
Uniprot ID:
Q14973
Molecular weight:
38118.64
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:
SLCO1A2
Uniprot ID:
P46721
Molecular weight:
74144.1
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:
SLCO4A1
Uniprot ID:
Q96BD0
Molecular weight:
77192.5

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