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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:03:02 UTC
HMDB IDHMDB00619
Secondary Accession NumbersNone
Metabolite Identification
Common NameCholic acid
DescriptionCholic acid is a major primary bile acid produced in the liver and usually conjugated with glycine or taurine. It facilitates fat absorption and cholesterol excretion. 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. (PMID: 11316487 , 16037564 , 12576301 , 11907135 ).
Structure
Thumb
Synonyms
ValueSource
CholateKegg
3alpha,7alpha,12alpha-Trihydroxy-5beta-cholanateKegg
3alpha,7alpha,12alpha-Trihydroxy-5beta-cholanic acidKegg
3a,7a,12a-Trihydroxy-5b-cholanateGenerator
3a,7a,12a-Trihydroxy-5b-cholanic acidGenerator
3α,7α,12α-trihydroxy-5β-cholanateGenerator
3α,7α,12α-trihydroxy-5β-cholanic acidGenerator
17b-[1-Methyl-3-carboxypropyl]etiocholane-3a,7a,12a-triolHMDB
3a,7a,12a-Trihydroxy-5b-cholan-24-OateHMDB
3a,7a,12a-Trihydroxy-5b-cholan-24-Oic acidHMDB
3a,7a,12a-Trihydroxy-5b-cholanoateHMDB
3a,7a,12a-Trihydroxy-5b-cholanoic acidHMDB
3a,7a,12a-Trihydroxy-b-cholanateHMDB
3a,7a,12a-Trihydroxy-b-cholanic acidHMDB
3a,7a,12a-Trihydroxy-beta-cholanateHMDB
3a,7a,12a-Trihydroxy-beta-cholanic acidHMDB
3a,7a,12a-TrihydroxycholanateHMDB
3a,7a,12a-Trihydroxycholanic acidHMDB
5b-Cholanic acid-3a,7a,12a-triolHMDB
5b-CholateHMDB
5b-Cholic acidHMDB
CholalateHMDB
Cholalic acidHMDB
CholalinHMDB
ColalinHMDB
Chemical FormulaC24H40O5
Average Molecular Weight408.5714
Monoisotopic Molecular Weight408.28757439
IUPAC Name(4R)-4-[(1S,2S,5R,7S,9R,10R,11S,14R,15R,16S)-5,9,16-trihydroxy-2,15-dimethyltetracyclo[8.7.0.0²,⁷.0¹¹,¹⁵]heptadecan-14-yl]pentanoic acid
Traditional Namecholic acid
CAS Registry Number81-25-4
SMILES
[H][C@@]1(CC[C@@]2([H])[C@]3([H])[C@H](O)C[C@]4([H])C[C@H](O)CC[C@]4(C)[C@@]3([H])C[C@H](O)[C@]12C)[C@H](C)CCC(O)=O
InChI Identifier
InChI=1S/C24H40O5/c1-13(4-7-21(28)29)16-5-6-17-22-18(12-20(27)24(16,17)3)23(2)9-8-15(25)10-14(23)11-19(22)26/h13-20,22,25-27H,4-12H2,1-3H3,(H,28,29)/t13-,14+,15-,16-,17+,18+,19-,20+,22+,23+,24-/m1/s1
InChI KeyInChIKey=BHQCQFFYRZLCQQ-OELDTZBJSA-N
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
Substituents
  • Trihydroxy bile acid, alcohol, or derivatives
  • 7-hydroxysteroid
  • 3-alpha-hydroxysteroid
  • Hydroxysteroid
  • 12-hydroxysteroid
  • 3-hydroxysteroid
  • Cyclic alcohol
  • Secondary alcohol
  • Polyol
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic homopolycyclic compound
Molecular FrameworkAliphatic homopolycyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fat solubilization and Waste products
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point197 - 201 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility0.175 mg/mLNot Available
LogP2.02RODA,A ET AL. (1990)
Predicted Properties
PropertyValueSource
Water Solubility0.074 mg/mLALOGPS
logP2.26ALOGPS
logP2.48ChemAxon
logS-3.7ALOGPS
pKa (Strongest Acidic)4.48ChemAxon
pKa (Strongest Basic)-0.16ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count4ChemAxon
Polar Surface Area97.99 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity110.79 m3·mol-1ChemAxon
Polarizability46.93 Å3ChemAxon
Number of Rings4ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00dl-0009000000-85abf6e916e1cc1c46b6View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00dl-0009000000-476dc71e401cc2327292View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-00nb-2109000000-0181c85f78bb9e29ea53View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0a4r-0009700000-63a8d2c748b3bc631c17View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-052r-1009200000-ba378537551be8134141View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4l-9006000000-caef890bd53da215442eView in MoNA
MSMass Spectrum (Electron Ionization)splash10-0596-9642000000-e425981b8a0ac72ea6eeView in MoNA
1D NMR13C NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Extracellular
Biofluid Locations
  • Bile
  • Blood
  • Feces
  • Urine
Tissue Location
  • Intestine
  • Kidney
  • Liver
  • Placenta
  • Platelet
  • Small Intestine
  • Spleen
Pathways
NameSMPDB LinkKEGG Link
27-Hydroxylase DeficiencySMP00720Not Available
Bile Acid BiosynthesisSMP00035map00120
Cerebrotendinous Xanthomatosis (CTX)SMP00315Not Available
Congenital Bile Acid Synthesis Defect Type IISMP00314Not Available
Congenital Bile Acid Synthesis Defect Type IIISMP00318Not Available
Familial Hypercholanemia (FHCA)SMP00317Not Available
Zellweger SyndromeSMP00316Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BileDetected and Quantified32060 (31830-32280) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.7 +/- 0.3 uMNewborn (0-30 days old)BothNormal details
BloodDetected and Quantified0.72 +/- 0.24 uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Normal
details
FecesDetected and Quantified44.71 +/- 47.79 nmol/g of fecesNot SpecifiedNot Specified
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified48.5 (2.8-124.5) uMAdult (>18 years old)Both
Biliary cirrhosis
details
BloodDetected and Quantified13.8 (1.2-36.5) uMAdult (>18 years old)Both
Primary biliary cirrhosis
details
BloodDetected and Quantified15.6 +/- 3.6 uMNewborn (0-30 days old)BothExtrahepatic biliary atresia (EHBA) details
BloodDetected and Quantified1.71 +/- 0.29 uMAdult (>18 years old)Both
Cystic fibrosis
details
BloodDetected and Quantified1.52 +/- 0.26 uMChildren (1-13 years old)BothCystic fibrosis details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Recurrent Clostridium difficile infection
details
FecesDetected but not QuantifiedNot ApplicableNot SpecifiedNot Specified
Recurrent Clostridium difficile infection
details
UrineDetected and Quantified2.2 (0.13-6.6) umol/mmol creatinineAdult (>18 years old)Both
Biliary cirrhosis
details
UrineDetected and Quantified1.9 (0.11-7.2) umol/mmol creatinineAdult (>18 years old)Both
Biliary cirrhosis
details
UrineDetected and Quantified1.969 +/- 0.612 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified0.075 +/- 0.090 umol/mmol creatinineAdult (>18 years old)BothBiliary atresia details
Associated Disorders and Diseases
Disease References
Biliary atresia
  1. Gustafsson J, Alvelius G, Bjorkhem I, Nemeth A: Bile acid metabolism in extrahepatic biliary atresia: lithocholic acid in stored dried blood collected at neonatal screening. Ups J Med Sci. 2006;111(1):131-6. [16553252 ]
  2. Nittono H, Obinata K, Nakatsu N, Watanabe T, Niijima S, Sasaki H, Arisaka O, Kato H, Yabuta K, Miyano T: Sulfated and nonsulfated bile acids in urine of patients with biliary atresia: analysis of bile acids by high-performance liquid chromatography. J Pediatr Gastroenterol Nutr. 1986 Jan;5(1):23-9. [3944741 ]
Cirrhosis
  1. Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [2621422 ]
Cystic fibrosis
  1. Smith JL, Lewindon PJ, Hoskins AC, Pereira TN, Setchell KD, O'Connell NC, Shepherd RW, Ramm GA: Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis. Hepatology. 2004 Jun;39(6):1673-82. [15185309 ]
Primary biliary cirrhosis
  1. Batta AK, Arora R, Salen G, Tint GS, Eskreis D, Katz S: Characterization of serum and urinary bile acids in patients with primary biliary cirrhosis by gas-liquid chromatography-mass spectrometry: effect of ursodeoxycholic acid treatment. J Lipid Res. 1989 Dec;30(12):1953-62. [2621422 ]
Associated OMIM IDs
DrugBank IDDB02659
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB012810
KNApSAcK IDNot Available
Chemspider ID192176
KEGG Compound IDC00695
BioCyc IDNot Available
BiGG ID35720
Wikipedia LinkCholic acid
NuGOwiki LinkHMDB00619
Metagene LinkHMDB00619
METLIN ID206
PubChem Compound221493
PDB IDCHD
ChEBI ID16359
References
Synthesis ReferenceCalimente, Daniel. Synthesis and characterization of new naphthalimide and cholic acid-containing complexing agents. (1998), 104 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Tadano T, Kanoh M, Matsumoto M, Sakamoto K, Kamano T: Studies of serum and feces bile acids determination by gas chromatography-mass spectrometry. Rinsho Byori. 2006 Feb;54(2):103-10. [16548228 ]
  2. Smith JL, Lewindon PJ, Hoskins AC, Pereira TN, Setchell KD, O'Connell NC, Shepherd RW, Ramm GA: Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosis. Hepatology. 2004 Jun;39(6):1673-82. [15185309 ]
  3. Kuramoto T, Furukawa Y, Nishina T, Sugimoto T, Mahara R, Tohma M, Kihira K, Hoshita T: Identification of short side chain bile acids in urine of patients with cerebrotendinous xanthomatosis. J Lipid Res. 1990 Oct;31(10):1895-902. [2079611 ]
  4. Wildgrube HJ, Stang H, Winkler M, Mauritz G: [Value of serum levels of conjugated cholic acid in the diagnosis of liver disease (author's transl)] Dtsch Med Wochenschr. 1982 Aug 20;107(33):1235-7. [7106004 ]
  5. Rodrigues CM, Marin JJ, Brites D: Bile acid patterns in meconium are influenced by cholestasis of pregnancy and not altered by ursodeoxycholic acid treatment. Gut. 1999 Sep;45(3):446-52. [10446117 ]
  6. Einarsson K, Reihner E, Ewerth S, Bjorkhem I: Serum concentrations of unconjugated and conjugated cholic acid in portal venous and systemic venous blood of fasting man. Scand J Clin Lab Invest. 1989 Feb;49(1):83-91. [2727621 ]
  7. Briz O, Macias RI, Serrano MA, Gonzalez-Gallego J, Bayon JE, Marin JJ: Excretion of foetal bilirubin by the rat placenta-maternal liver tandem. Placenta. 2003 May;24(5):462-72. [12744922 ]
  8. Gustafsson J, Alvelius G, Bjorkhem I, Nemeth A: Bile acid metabolism in extrahepatic biliary atresia: lithocholic acid in stored dried blood collected at neonatal screening. Ups J Med Sci. 2006;111(1):131-6. [16553252 ]
  9. Salen G, Shefer S, Tint GS, Nicolau G, Dayal B, Batta AK: Biosynthesis of bile acids in cerebrotendinous xanthomatosis. Relationship of bile acid pool sizes and synthesis rates to hydroxylations at C-12, C-25, and C-26. J Clin Invest. 1985 Aug;76(2):744-51. [4031069 ]
  10. Brites D, Poeiras J, Rodrigues C: [Intrahepatic cholestasis in pregnancy. Its etiopathogenesis, prognosis and therapy] Acta Med Port. 1994 Mar;7(3):181-8. [8209706 ]
  11. Van Den Berg JW, Van Blankenstein M, Bosman-Jacobs EP, Frenkel M, Horchner P, Ooost-Harwig OI, Wilson JH: Solid phase radioimmunoassay for determination of conjugated cholic acid in serum. Clin Chim Acta. 1976 Dec 1;73(2):277-83. [1000848 ]
  12. Pomare EW, Low-Beer TS: Proceedings: Effect of administration of a colonic metabolite of cholic acid on cholesterol levels in bile and blood. Gut. 1974 Oct;15(10):830. [4434946 ]
  13. Einarsson K, Bergstrom M, Eklof R, Nord CE, Bjorkhem I: Comparison of the proportion of unconjugated to total serum cholic acid and the [14C]-xylose breath test in patients with suspected small intestinal bacterial overgrowth. Scand J Clin Lab Invest. 1992 Sep;52(5):425-30. [1514020 ]
  14. Ewerth S, Bjorkhem I, Einarsson K, Ost L: Lymphatic transport of bile acids in man. J Lipid Res. 1982 Nov;23(8):1183-6. [7175375 ]
  15. LaRusso NF, Hoffman NE, Hofmann AF, Korman MG: Validity and sensitivity of an intravenous bile acid tolerance test in patients with liver disease. N Engl J Med. 1975 Jun 5;292(23):1209-14. [1128572 ]
  16. Lillienau J, Schteingart CD, Hofmann AF: Physicochemical and physiological properties of cholylsarcosine. A potential replacement detergent for bile acid deficiency states in the small intestine. J Clin Invest. 1992 Feb;89(2):420-31. [1371123 ]
  17. 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. [11316487 ]
  18. 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. [16037564 ]
  19. 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. [12576301 ]
  20. 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. [11907135 ]

Enzymes

General function:
Involved in phospholipase A2 activity
Specific function:
PA2 catalyzes the calcium-dependent hydrolysis of the 2-acyl groups in 3-sn-phosphoglycerides.
Gene Name:
PLA2G1B
Uniprot ID:
P04054
Molecular weight:
16359.535
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Lipid transport and metabolism
Specific function:
Involved in the detoxification of xenobiotics and in the activation of ester and amide prodrugs. Hydrolyzes aromatic and aliphatic esters, but has no catalytic activity toward amides or a fatty acyl-CoA ester. Hydrolyzes the methyl ester group of cocaine to form benzoylecgonine. Catalyzes the transesterification of cocaine to form cocaethylene. Displays fatty acid ethyl ester synthase activity, catalyzing the ethyl esterification of oleic acid to ethyloleate.
Gene Name:
CES1
Uniprot ID:
P23141
Molecular weight:
62520.62
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in zinc ion binding
Specific function:
Not Available
Gene Name:
ADH1C
Uniprot ID:
P00326
Molecular weight:
39867.27
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in acyl-CoA thioesterase activity
Specific function:
Acyl-CoA thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH. May mediate Nef-induced down-regulation of CD4. Major thioesterase in peroxisomes. Competes with BAAT (Bile acid CoA: amino acid N-acyltransferase) for bile acid-CoA substrate (such as chenodeoxycholoyl-CoA). Shows a preference for medium-length fatty acyl-CoAs (By similarity). May be involved in the metabolic regulation of peroxisome proliferation.
Gene Name:
ACOT8
Uniprot ID:
O14734
Molecular weight:
35914.02
Reactions
Choloyl-CoA + Water → Cholic acid + Coenzyme Adetails
General function:
Involved in ferrochelatase activity
Specific function:
Catalyzes the ferrous insertion into protoporphyrin IX.
Gene Name:
FECH
Uniprot ID:
P22830
Molecular weight:
47861.77
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [17016423 ]
  3. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in iron ion binding
Specific function:
Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B.
Gene Name:
MT-CO1
Uniprot ID:
P00395
Molecular weight:
57040.91
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in cytochrome-c oxidase activity
Specific function:
This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport
Gene Name:
COX7A1
Uniprot ID:
P24310
Molecular weight:
9117.4
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
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 + Cholic acid + Coenzyme A → Adenosine monophosphate + Pyrophosphate + Choloyl-CoAdetails
General function:
Involved in cytochrome-c oxidase activity
Specific function:
This is the heme A-containing chain of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport
Gene Name:
COX5A
Uniprot ID:
P20674
Molecular weight:
16762.0
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in cytochrome-c oxidase activity
Specific function:
This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport
Gene Name:
COX6A2
Uniprot ID:
Q02221
Molecular weight:
10815.3
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in cytochrome-c oxidase activity
Specific function:
This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport
Gene Name:
COX7B
Uniprot ID:
P24311
Molecular weight:
9160.5
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in cytochrome-c oxidase activity
Specific function:
This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport
Gene Name:
COX7C
Uniprot ID:
P15954
Molecular weight:
7245.4
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in cytochrome-c oxidase activity
Specific function:
This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport
Gene Name:
COX5B
Uniprot ID:
P10606
Molecular weight:
13695.6
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in cytochrome-c oxidase activity
Specific function:
Connects the two COX monomers into the physiological dimeric form
Gene Name:
COX6B1
Uniprot ID:
P14854
Molecular weight:
10192.3
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in cytochrome-c oxidase activity
Specific function:
This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport
Gene Name:
COX4I1
Uniprot ID:
P13073
Molecular weight:
19576.6
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in cytochrome-c oxidase activity
Specific function:
This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport
Gene Name:
COX8A
Uniprot ID:
P10176
Molecular weight:
7579.0
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in cytochrome-c oxidase activity
Specific function:
This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport
Gene Name:
COX6C
Uniprot ID:
P09669
Molecular weight:
8781.4
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in cytochrome-c oxidase activity
Specific function:
Subunits I, II and III form the functional core of the enzyme complex
Gene Name:
MT-CO3
Uniprot ID:
P00414
Molecular weight:
29950.6
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in copper ion binding
Specific function:
Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1- 3 form the functional core of the enzyme complex. Subunit 2 transfers the electrons from cytochrome c via its binuclear copper A center to the bimetallic center of the catalytic subunit 1
Gene Name:
MT-CO2
Uniprot ID:
P00403
Molecular weight:
25564.7
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
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. [12486725 ]
  2. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]
General function:
Involved in ferrochelatase activity
Specific function:
Catalyzes the ferrous insertion into protoporphyrin IX
Gene Name:
DKFZp686P18130
Uniprot ID:
Q7KZA3
Molecular weight:
47131.9
References
  1. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]

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
References
  1. Cui Y, Konig J, Leier I, Buchholz U, Keppler D: Hepatic uptake of bilirubin and its conjugates by the human organic anion transporter SLC21A6. J Biol Chem. 2001 Mar 30;276(13):9626-30. Epub 2000 Dec 27. [11134001 ]
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. [12196548 ]
  2. Hartmann G, Cheung AK, Piquette-Miller M: Inflammatory cytokines, but not bile acids, regulate expression of murine hepatic anion transporters in endotoxemia. J Pharmacol Exp Ther. 2002 Oct;303(1):273-81. [12235261 ]
  3. Konig J, Cui Y, Nies AT, Keppler D: A novel human organic anion transporting polypeptide localized to the basolateral hepatocyte membrane. Am J Physiol Gastrointest Liver Physiol. 2000 Jan;278(1):G156-64. [10644574 ]
  4. Cui Y, Konig J, Leier I, Buchholz U, Keppler D: Hepatic uptake of bilirubin and its conjugates by the human organic anion transporter SLC21A6. J Biol Chem. 2001 Mar 30;276(13):9626-30. Epub 2000 Dec 27. [11134001 ]
General function:
Involved in ATP binding
Specific function:
Mediates hepatobiliary excretion of numerous organic anions. May function as a cellular cisplatin transporter
Gene Name:
ABCC2
Uniprot ID:
Q92887
Molecular weight:
174205.6
References
  1. Fickert P, Zollner G, Fuchsbichler A, Stumptner C, Pojer C, Zenz R, Lammert F, Stieger B, Meier PJ, Zatloukal K, Denk H, Trauner M: Effects of ursodeoxycholic and cholic acid feeding on hepatocellular transporter expression in mouse liver. Gastroenterology. 2001 Jul;121(1):170-83. [11438506 ]
General function:
Involved in ATP binding
Specific function:
Mediates export of organic anions and drugs from the cytoplasm. Mediates ATP-dependent transport of glutathione and glutathione conjugates, leukotriene C4, estradiol-17-beta-o- glucuronide, methotrexate, antiviral drugs and other xenobiotics. Confers resistance to anticancer drugs. Hydrolyzes ATP with low efficiency
Gene Name:
ABCC1
Uniprot ID:
P33527
Molecular weight:
171589.5
References
  1. Hartmann G, Cheung AK, Piquette-Miller M: Inflammatory cytokines, but not bile acids, regulate expression of murine hepatic anion transporters in endotoxemia. J Pharmacol Exp Ther. 2002 Oct;303(1):273-81. [12235261 ]
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
References
  1. Hirohashi T, Suzuki H, Takikawa H, Sugiyama Y: ATP-dependent transport of bile salts by rat multidrug resistance-associated protein 3 (Mrp3). J Biol Chem. 2000 Jan 28;275(4):2905-10. [10644759 ]
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
References
  1. Hartmann G, Cheung AK, Piquette-Miller M: Inflammatory cytokines, but not bile acids, regulate expression of murine hepatic anion transporters in endotoxemia. J Pharmacol Exp Ther. 2002 Oct;303(1):273-81. [12235261 ]
  2. Green RM, Hoda F, Ward KL: Molecular cloning and characterization of the murine bile salt export pump. Gene. 2000 Jan 4;241(1):117-23. [10607905 ]
  3. Mita S, Suzuki H, Akita H, Stieger B, Meier PJ, Hofmann AF, Sugiyama Y: Vectorial transport of bile salts across MDCK cells expressing both rat Na+-taurocholate cotransporting polypeptide and rat bile salt export pump. Am J Physiol Gastrointest Liver Physiol. 2005 Jan;288(1):G159-67. Epub 2004 Aug 5. [15297262 ]
General function:
Involved in ATP binding
Specific function:
Energy-dependent efflux pump responsible for decreased drug accumulation in multidrug-resistant cells
Gene Name:
ABCB1
Uniprot ID:
P08183
Molecular weight:
141477.3
References
  1. Fickert P, Zollner G, Fuchsbichler A, Stumptner C, Pojer C, Zenz R, Lammert F, Stieger B, Meier PJ, Zatloukal K, Denk H, Trauner M: Effects of ursodeoxycholic and cholic acid feeding on hepatocellular transporter expression in mouse liver. Gastroenterology. 2001 Jul;121(1):170-83. [11438506 ]
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. [11447228 ]
  2. Fickert P, Zollner G, Fuchsbichler A, Stumptner C, Pojer C, Zenz R, Lammert F, Stieger B, Meier PJ, Zatloukal K, Denk H, Trauner M: Effects of ursodeoxycholic and cholic acid feeding on hepatocellular transporter expression in mouse liver. Gastroenterology. 2001 Jul;121(1):170-83. [11438506 ]
  3. Craddock AL, Love MW, Daniel RW, Kirby LC, Walters HC, Wong MH, Dawson PA: Expression and transport properties of the human ileal and renal sodium-dependent bile acid transporter. Am J Physiol. 1998 Jan;274(1 Pt 1):G157-69. [9458785 ]
  4. Saeki T, Matoba K, Furukawa H, Kirifuji K, Kanamoto R, Iwami K: Characterization, cDNA cloning, and functional expression of mouse ileal sodium-dependent bile acid transporter. J Biochem. 1999 Apr;125(4):846-51. [10101301 ]
  5. Saeki T, Takahashi N, Kanamoto R, Iwami K: Characterization of cloned mouse Na+/taurocholate cotransporting polypeptide by transient expression in COS-7 cells. Biosci Biotechnol Biochem. 2002 May;66(5):1116-8. [12092825 ]
  6. Walters HC, Craddock AL, Fusegawa H, Willingham MC, Dawson PA: Expression, transport properties, and chromosomal location of organic anion transporter subtype 3. Am J Physiol Gastrointest Liver Physiol. 2000 Dec;279(6):G1188-200. [11093941 ]
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
References
  1. Hagenbuch B, Stieger B, Foguet M, Lubbert H, Meier PJ: Functional expression cloning and characterization of the hepatocyte Na+/bile acid cotransport system. Proc Natl Acad Sci U S A. 1991 Dec 1;88(23):10629-33. [1961729 ]
  2. Schroeder A, Eckhardt U, Stieger B, Tynes R, Schteingart CD, Hofmann AF, Meier PJ, Hagenbuch B: Substrate specificity of the rat liver Na(+)-bile salt cotransporter in Xenopus laevis oocytes and in CHO cells. Am J Physiol. 1998 Feb;274(2 Pt 1):G370-5. [9486191 ]
  3. Hata S, Wang P, Eftychiou N, Ananthanarayanan M, Batta A, Salen G, Pang KS, Wolkoff AW: Substrate specificities of rat oatp1 and ntcp: implications for hepatic organic anion uptake. Am J Physiol Gastrointest Liver Physiol. 2003 Nov;285(5):G829-39. Epub 2003 Jul 3. [12842829 ]
  4. Mita S, Suzuki H, Akita H, Stieger B, Meier PJ, Hofmann AF, Sugiyama Y: Vectorial transport of bile salts across MDCK cells expressing both rat Na+-taurocholate cotransporting polypeptide and rat bile salt export pump. Am J Physiol Gastrointest Liver Physiol. 2005 Jan;288(1):G159-67. Epub 2004 Aug 5. [15297262 ]
  5. Platte HD, Honscha W, Schuh K, Petzinger E: Functional characterization of the hepatic sodium-dependent taurocholate transporter stably transfected into an immortalized liver-derived cell line and V79 fibroblasts. Eur J Cell Biol. 1996 May;70(1):54-60. [8738419 ]
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
References
  1. Fickert P, Zollner G, Fuchsbichler A, Stumptner C, Pojer C, Zenz R, Lammert F, Stieger B, Meier PJ, Zatloukal K, Denk H, Trauner M: Effects of ursodeoxycholic and cholic acid feeding on hepatocellular transporter expression in mouse liver. Gastroenterology. 2001 Jul;121(1):170-83. [11438506 ]
  2. Kullak-Ublick GA, Hagenbuch B, Stieger B, Schteingart CD, Hofmann AF, Wolkoff AW, Meier PJ: Molecular and functional characterization of an organic anion transporting polypeptide cloned from human liver. Gastroenterology. 1995 Oct;109(4):1274-82. [7557095 ]
  3. Kullak-Ublick GA, Hagenbuch B, Stieger B, Wolkoff AW, Meier PJ: Functional characterization of the basolateral rat liver organic anion transporting polypeptide. Hepatology. 1994 Aug;20(2):411-6. [8045503 ]
  4. Kanai N, Lu R, Bao Y, Wolkoff AW, Schuster VL: Transient expression of oatp organic anion transporter in mammalian cells: identification of candidate substrates. Am J Physiol. 1996 Feb;270(2 Pt 2):F319-25. [8779893 ]
  5. Hata S, Wang P, Eftychiou N, Ananthanarayanan M, Batta A, Salen G, Pang KS, Wolkoff AW: Substrate specificities of rat oatp1 and ntcp: implications for hepatic organic anion uptake. Am J Physiol Gastrointest Liver Physiol. 2003 Nov;285(5):G829-39. Epub 2003 Jul 3. [12842829 ]
  6. Jacquemin E, Hagenbuch B, Stieger B, Wolkoff AW, Meier PJ: Expression cloning of a rat liver Na(+)-independent organic anion transporter. Proc Natl Acad Sci U S A. 1994 Jan 4;91(1):133-7. [8278353 ]
  7. Kouzuki H, Suzuki H, Ito K, Ohashi R, Sugiyama Y: Contribution of organic anion transporting polypeptide to uptake of its possible substrates into rat hepatocytes. J Pharmacol Exp Ther. 1999 Feb;288(2):627-34. [9918568 ]
  8. Eckhardt U, Schroeder A, Stieger B, Hochli M, Landmann L, Tynes R, Meier PJ, Hagenbuch B: Polyspecific substrate uptake by the hepatic organic anion transporter Oatp1 in stably transfected CHO cells. Am J Physiol. 1999 Apr;276(4 Pt 1):G1037-42. [10198348 ]
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
General function:
Involved in ATP binding
Specific function:
May be an organic anion pump relevant to cellular detoxification
Gene Name:
ABCC4
Uniprot ID:
O15439
Molecular weight:
149525.3
References
  1. Rius M, Nies AT, Hummel-Eisenbeiss J, Jedlitschky G, Keppler D: Cotransport of reduced glutathione with bile salts by MRP4 (ABCC4) localized to the basolateral hepatocyte membrane. Hepatology. 2003 Aug;38(2):374-84. [12883481 ]
  2. Bai J, Lai L, Yeo HC, Goh BC, Tan TM: Multidrug resistance protein 4 (MRP4/ABCC4) mediates efflux of bimane-glutathione. Int J Biochem Cell Biol. 2004 Feb;36(2):247-57. [14643890 ]
General function:
Involved in transmembrane transport
Specific function:
Mediates sodium-independent multispecific organic anion transport. Transport of prostaglandin E2, prostaglandin F2, tetracycline, bumetanide, estrone sulfate, glutarate, dehydroepiandrosterone sulfate, allopurinol, 5-fluorouracil, paclitaxel, L-ascorbic acid, salicylate, ethotrexate, and alpha- ketoglutarate
Gene Name:
SLC22A7
Uniprot ID:
Q9Y694
Molecular weight:
60025.0
References
  1. Sekine T, Cha SH, Tsuda M, Apiwattanakul N, Nakajima N, Kanai Y, Endou H: Identification of multispecific organic anion transporter 2 expressed predominantly in the liver. FEBS Lett. 1998 Jun 12;429(2):179-82. [9650585 ]