Page last updated: 2024-10-06

Disorders of bile acid synthesis and biliary transport

Proteins (18)

ProteinSynonymsTaxonomy
Very long-chain acyl-CoA synthetaseVLACS; VLCS; EC 6.2.1.-; Arachidonate--CoA ligase; 6.2.1.15; Fatty acid transport protein 2; FATP-2; Fatty-acid-coenzyme A ligase, very long-chain 1; Long-chain-fatty-acid--CoA ligase; 6.2.1.3; Phytanate--CoA ligase; 6.2.1.24; Solute carrier family 27 memHomo sapiens (human)
Bile acyl-CoA synthetaseBACS; EC 6.2.1.7; Bile acid-CoA ligase; BA-CoA ligase; BAL; Cholate--CoA ligase; Fatty acid transport protein 5; FATP-5; Fatty-acid-coenzyme A ligase, very long-chain 3; Solute carrier family 27 member 5; Very long-chain acyl-CoA synthetase homolog 2; VLCHomo sapiens (human)
ATP-binding cassette sub-family C member 3EC 7.6.2.-; EC 7.6.2.2; EC 7.6.2.3; Canalicular multispecific organic anion transporter 2; Multi-specific organic anion transporter D; MOAT-D; Multidrug resistance-associated protein 3Homo sapiens (human)
ATP-dependent translocase ABCB1ATP-binding cassette sub-family B member 1; Multidrug resistance protein 1; 7.6.2.2; P-glycoprotein 1; Phospholipid transporter ABCB1; 7.6.2.1Homo sapiens (human)
Aldo-keto reductase family 1 member C4EC 1.1.1.-; EC 1.1.1.209; EC 1.1.1.210; EC 1.1.1.51; EC 1.1.1.53; EC 1.1.1.62; 3-alpha-hydroxysteroid dehydrogenase type I; 3-alpha-HSD1; 1.1.1.357; 3alpha-hydroxysteroid 3-dehydrogenase; Chlordecone reductase; CDR; 1.1.1.225; Dihydrodiol dehydrogenase 4;Homo sapiens (human)
Canalicular multispecific organic anion transporter 1ATP-binding cassette sub-family C member 2; Canalicular multidrug resistance protein; Multidrug resistance-associated protein 2; 7.6.2.2Homo sapiens (human)
Solute carrier organic anion transporter family member 1B1Liver-specific organic anion transporter 1; LST-1; OATP-C; Sodium-independent organic anion-transporting polypeptide 2; OATP-2; Solute carrier family 21 member 6Homo sapiens (human)
Multidrug resistance-associated protein 4ATP-binding cassette sub-family C member 4; MRP/cMOAT-related ABC transporter; Multi-specific organic anion transporter B; MOAT-BHomo sapiens (human)
GastrotropinGT; Fatty acid-binding protein 6; Ileal lipid-binding protein; ILBP; Intestinal 15 kDa protein; I-15P; Intestinal bile acid-binding protein; I-BABPHomo sapiens (human)
Bile salt export pumpEC 7.6.2.-; ATP-binding cassette sub-family B member 11Homo sapiens (human)
Ileal sodium/bile acid cotransporterApical sodium-dependent bile acid transporter; ASBT; Ileal Na(+)/bile acid cotransporter; Ileal sodium-dependent bile acid transporter; IBAT; ISBT; Na(+)-dependent ileal bile acid transporter; Sodium/taurocholate cotransporting polypeptide, ileal; Solute Homo sapiens (human)
Sodium/bile acid cotransporterCell growth-inhibiting gene 29 protein; Na(+)/bile acid cotransporter; Na(+)/taurocholate transport protein; Sodium/taurocholate cotransporting polypeptide; Solute carrier family 10 member 1Homo sapiens (human)
Organic solute transporter subunit alphaOST-alpha; Solute carrier family 51 subunit alphaHomo sapiens (human)
Solute carrier organic anion transporter family member 1B3Liver-specific organic anion transporter 2; LST-2; Organic anion transporter 8; Organic anion-transporting polypeptide 8; OATP-8; Solute carrier family 21 member 8Homo sapiens (human)
Conjugated bile acid hydrolaseCBAH; EC 3.5.1.-; Bile salt hydrolase; BSH; CBAH-1; Choloylglycine hydrolase; 3.5.1.24Clostridium perfringens str. 13
Bile acid-CoA:amino acid N-acyltransferaseBACAT; BAT; EC 2.3.1.65; Bile acid-CoA thioesterase; Choloyl-CoA hydrolase; 3.1.2.27; Glycine N-choloyltransferase; Long-chain fatty-acyl-CoA hydrolase; 3.1.2.2Homo sapiens (human)
ATP-binding cassette sub-family D member 370 kDa peroxisomal membrane protein; PMP70Homo sapiens (human)
Organic solute transporter subunit betaOST-beta; Solute carrier family 51 subunit betaHomo sapiens (human)

Compounds (19)

CompoundDescription
CholesterolThe principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils.
3,7,12-trihydroxycholestan-26-oic acidRN given refers to (3alpha,5beta,7alpha,12alpha)-isomer
7 alpha-hydroxy-4-cholesten-3-onestructure
glycochenodeoxycholic acidA bile salt formed in the liver from chenodeoxycholate and glycine, usually as the sodium salt. It acts as a detergent to solubilize fats for absorption and is itself absorbed. It is a cholagogue and choleretic.
3,7,12-trihydroxycoprostaneintermediate in bile acid synthesis in liver; enhances rate of porphyrin synthesis in cultured liver cells by induction of delta-aminolevulinic acid synthetase; RN given refers to (3alpha,5beta,7alpha,12alpha)-isomer; structure
cholestane-3,7,12,26-tetrolprecursor of cholic acid biosyn in man; RN given refers to (3alpha,5beta,7alpha,12alpha)-isomer
dihydroxycoprostaneintermediate in bile acid synthesis in the liver; enhance rate of prophyrin synthesis in cultured liver cells by induction of delta-aminolevulinic acid synthetase; RN given refers to (3alpha,5beta,7alpha)-isomer; structure
3,7-dihydroxy-5-cholestenoic acidfound in patients with liver disease
7 alpha,12 alpha-dihydroxy-5-cholesten-3-oneinermediate in bile acid biosynthesis
cholestane-3,7,26-triol
3,7,12-trihydroxycholestan-26-alstructure in first source
Taurocholic AcidThe product of conjugation of cholic acid with taurine. Its sodium salt is the chief ingredient of the bile of carnivorous animals. It acts as a detergent to solubilize fats for absorption and is itself absorbed. It is used as a cholagogue and cholerectic.
glycocholic acidThe glycine conjugate of CHOLIC ACID. It acts as a detergent to solubilize fats for absorption and is itself absorbed.
cholest-5-en-3 beta,7 alpha-diolsubstrate for hydroxysteroid dehydrogenase; RN given refers to (3beta,7alpha)-isomer
3-hydroxy-5-cholestenoic acidfound in infants with coprostanicacidemia; RN given refers to (3beta)-isomer
7 alpha,26-dihydroxy-4-cholesten-3-one
taurochenodeoxycholic acidA bile salt formed in the liver by conjugation of chenodeoxycholate with taurine, usually as the sodium salt. It acts as detergent to solubilize fats in the small intestine and is itself absorbed. It is used as a cholagogue and choleretic.
cholyl-coenzyme Astructure in first source
cholest-5-ene-3 beta,26-diolisolated from human brain