UniProt functional annotation for O95342



	UniProt functional annotation for O95342

UniProt code: O95342.

Organism: Homo sapiens (Human).

Taxonomy: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo.

Function: Catalyzes the transport of the major hydrophobic bile salts, such as taurine and glycine-conjugated cholic acid across the canalicular membrane of hepatocytes in an ATP-dependent manner, therefore participates to hepatic bile acids homeostasis and consequently to lipid homeostasis through regulation of biliary lipid secretion in a bile salts dependent manner (PubMed:16332456, PubMed:22262466, PubMed:15791618, PubMed:18985798, PubMed:19228692, PubMed:20398791, PubMed:24711118, PubMed:29507376, PubMed:20010382, PubMed:32203132). Transports taurine-conjugated bile salts more rapidly than glycine-conjugated bile salts (PubMed:16332456). Also transports non-bile acid compounds, such as pravastatin and fexofenadine in an ATP-dependent manner and may be involved in their biliary excretion (PubMed:15901796, PubMed:18245269). {ECO:0000269|PubMed:15791618, ECO:0000269|PubMed:15901796, ECO:0000269|PubMed:16332456, ECO:0000269|PubMed:18245269, ECO:0000269|PubMed:18985798, ECO:0000269|PubMed:19228692, ECO:0000269|PubMed:20010382, ECO:0000269|PubMed:20398791, ECO:0000269|PubMed:22262466, ECO:0000269|PubMed:24711118, ECO:0000269|PubMed:29507376, ECO:0000269|PubMed:32203132}.

Catalytic activity: Reaction=ATP + cholate(in) + H2O = ADP + cholate(out) + H(+) + phosphate; Xref=Rhea:RHEA:50048, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:29747, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50049; Evidence={ECO:0000269|PubMed:16332456};

Catalytic activity: Reaction=ATP + H2O + taurocholate(in) = ADP + H(+) + phosphate + taurocholate(out); Xref=Rhea:RHEA:50052, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:36257, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269|PubMed:15791618, ECO:0000269|PubMed:15901796, ECO:0000269|PubMed:16332456, ECO:0000269|PubMed:18985798, ECO:0000269|PubMed:19228692, ECO:0000269|PubMed:20010382, ECO:0000269|PubMed:20398791, ECO:0000269|PubMed:22262466, ECO:0000269|PubMed:24711118, ECO:0000269|PubMed:29507376, ECO:0000269|PubMed:32203132}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50053; Evidence={ECO:0000269|PubMed:15791618, ECO:0000269|PubMed:15901796, ECO:0000269|PubMed:16332456, ECO:0000269|PubMed:18985798, ECO:0000269|PubMed:19228692, ECO:0000269|PubMed:20010382, ECO:0000269|PubMed:20398791, ECO:0000269|PubMed:22262466, ECO:0000269|PubMed:24711118, ECO:0000269|PubMed:29507376, ECO:0000305|PubMed:32203132};

Catalytic activity: Reaction=ATP + glycocholate(in) + H2O = ADP + glycocholate(out) + H(+) + phosphate; Xref=Rhea:RHEA:50056, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:29746, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269|PubMed:15791618, ECO:0000269|PubMed:16332456, ECO:0000269|PubMed:32203132}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50057; Evidence={ECO:0000269|PubMed:15791618, ECO:0000269|PubMed:16332456, ECO:0000305|PubMed:32203132};

Catalytic activity: Reaction=ATP + glycochenodeoxycholate(in) + H2O = ADP + glycochenodeoxycholate(out) + H(+) + phosphate; Xref=Rhea:RHEA:50060, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:36252, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50061; Evidence={ECO:0000269|PubMed:16332456};

Catalytic activity: Reaction=ATP + H2O + taurochenodeoxycholate(in) = ADP + H(+) + phosphate + taurochenodeoxycholate(out); Xref=Rhea:RHEA:50064, ChEBI:CHEBI:9407, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50065; Evidence={ECO:0000269|PubMed:16332456};

Catalytic activity: Reaction=ATP + glycoursodeoxycholate(in) + H2O = ADP + glycoursodeoxycholate(out) + H(+) + phosphate; Xref=Rhea:RHEA:50068, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:132030, ChEBI:CHEBI:456216; Evidence={ECO:0000269|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50069; Evidence={ECO:0000269|PubMed:16332456};

Catalytic activity: Reaction=ATP + H2O + tauroursodeoxycholate(in) = ADP + H(+) + phosphate + tauroursodeoxycholate(out); Xref=Rhea:RHEA:50072, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:132028, ChEBI:CHEBI:456216; Evidence={ECO:0000269|PubMed:16332456, ECO:0000269|PubMed:32203132}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50073; Evidence={ECO:0000269|PubMed:16332456, ECO:0000305|PubMed:32203132};

Catalytic activity: Reaction=ATP + H2O + taurodeoxycholate(in) = ADP + H(+) + phosphate + taurodeoxycholate(out); Xref=Rhea:RHEA:50080, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:36261, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50081; Evidence={ECO:0000269|PubMed:16332456};

Catalytic activity: Reaction=ATP + H2O + taurolithocholate 3-sulfate(in) = ADP + H(+) + phosphate + taurolithocholate 3-sulfate(out); Xref=Rhea:RHEA:50084, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:58301, ChEBI:CHEBI:456216; Evidence={ECO:0000269|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50085; Evidence={ECO:0000269|PubMed:16332456};

Catalytic activity: Reaction=ATP + H2O + pravastatin(in) = ADP + H(+) + phosphate + pravastatin(out); Xref=Rhea:RHEA:63908, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:63660, ChEBI:CHEBI:456216; Evidence={ECO:0000269|PubMed:15901796}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:63909; Evidence={ECO:0000305|PubMed:15901796};

Activity regulation: The uptake of taurocholate is inhibited by taurolithocholate sulfate with an IC(50) of 9 uM (PubMed:16332456). Pravastatin competitively inhibits the transport of taurocholic acid (PubMed:18985798, PubMed:15901796). Cyclosporin A, glibenclamide, rifampicin and troglitazonestrongly competitively inhibit the transport activity of taurocholate (PubMed:18985798, PubMed:32203132). The canalicular transport activity of taurocholate is strongly dependent on canalicular membrane cholesterol content (PubMed:19228692). The uptake of taurocholate is increased by short- and medium-chain fatty acids (PubMed:20398791). Cholesterol increases transport capacity of taurocholate without affecting the affinity for the substrate (PubMed:24711118). {ECO:0000269|PubMed:15901796, ECO:0000269|PubMed:16332456, ECO:0000269|PubMed:18985798, ECO:0000269|PubMed:19228692, ECO:0000269|PubMed:20398791, ECO:0000269|PubMed:24711118, ECO:0000269|PubMed:32203132}.

Biophysicochemical properties: Kinetic parameters: KM=30.4 uM for taurocholate {ECO:0000269|PubMed:17264802}; KM=6.2 uM for taurocholate {ECO:0000269|PubMed:16332456}; KM=21.7 uM for glycocholate {ECO:0000269|PubMed:16332456}; KM=6.6 uM for taurochenodeoxycholate {ECO:0000269|PubMed:16332456}; KM=7.5 uM for glycochenodeoxycholate {ECO:0000269|PubMed:16332456}; KM=9.5 uM for taurolithocholate sulfate {ECO:0000269|PubMed:16332456}; KM=4.61 uM for taurocholate {ECO:0000269|PubMed:15791618}; KM=4.64 uM for taurocholate {ECO:0000269|PubMed:15901796}; KM=124 uM for pravastatin {ECO:0000269|PubMed:15901796}; KM=19.9 uM for taurocholate {ECO:0000269|PubMed:18985798}; Vmax=232 pmol/min/mg enzyme for taurocholate transport {ECO:0000269|PubMed:17264802}; Vmax=2510 pmol/min/mg enzyme for taurocholate transport {ECO:0000269|PubMed:16332456}; Vmax=2310 pmol/min/mg enzyme for taurocholate transport {ECO:0000269|PubMed:15791618}; Vmax=4290 pmol/min/mg enzyme for taurocholate transport {ECO:0000269|PubMed:15901796}; Vmax=1220 pmol/min/mg enzyme for pravastatin transport {ECO:0000269|PubMed:15901796}; Vmax=98.5 pmol/min/mg enzyme for taurocholate transport {ECO:0000269|PubMed:18985798};

Subunit: Interacts with HAX1 (By similarity). Interacts with the adapter protein complex 2 (AP-2) throught AP2A2 or AP2A1; this interaction regulates cell membrane expresion of ABCB11 through its internalization in a clathrin-dependent manner and its subsequent degradation (PubMed:22262466). {ECO:0000250|UniProtKB:O70127, ECO:0000269|PubMed:22262466}.

Subcellular location: Apical cell membrane {ECO:0000269|PubMed:15791618, ECO:0000269|PubMed:22262466}; Multi-pass membrane protein {ECO:0000255}. Recycling endosome membrane {ECO:0000250|UniProtKB:O70127}; Multi-pass membrane protein {ECO:0000250|UniProtKB:O70127}. Endosome {ECO:0000250|UniProtKB:O70127}. Cell membrane {ECO:0000269|PubMed:20010382, ECO:0000269|PubMed:22262466, ECO:0000269|PubMed:29507376, ECO:0000269|PubMed:30431138}; Multi-pass membrane protein {ECO:0000255}. Note=Internalized at the canalicular membrane through interaction with the adapter protein complex 2 (AP-2) (PubMed:22262466). At steady state, localizes in the canalicular membrane but is also present in recycling endosomes. ABCB11 constantly and rapidly exchanges between the two sites through tubulo-vesicles carriers that move along microtubules. Microtubule-dependent trafficking of ABCB11 is enhanced by taurocholate and cAMP and regulated by STK11 through a PKA-mediated pathway. Trafficking of newly synthesized ABCB11 through endosomal compartment to the bile canalicular membrane is accelerated by cAMP but not by taurocholate (By similarity). Cell membrane expression is up-regulated by short- and medium-chain fatty acids (PubMed:20398791). {ECO:0000250|UniProtKB:O70127, ECO:0000269|PubMed:20398791, ECO:0000269|PubMed:22262466}.

Tissue specificity: Expressed predominantly, if not exclusively in the liver, where it was further localized to the canalicular microvilli and to subcanalicular vesicles of the hepatocytes by in situ.

Domain: Multifunctional polypeptide with two homologous halves, each containing a hydrophobic membrane-anchoring domain and an ATP binding cassette (ABC) domain.

Ptm: N-glycosylated. {ECO:0000269|PubMed:15791618, ECO:0000269|PubMed:18829893}.

Ptm: Ubiquitinated; short-chain ubiquitination regulates cell-Surface expression of ABCB11. {ECO:0000269|PubMed:18829893}.

Disease: Cholestasis, progressive familial intrahepatic, 2 (PFIC2) [MIM:601847]: A disorder characterized by early onset of cholestasis that progresses to hepatic fibrosis, cirrhosis, and end-stage liver disease before adulthood. {ECO:0000269|PubMed:10579978, ECO:0000269|PubMed:11815775, ECO:0000269|PubMed:15791618, ECO:0000269|PubMed:18829893, ECO:0000269|PubMed:20010382, ECO:0000269|PubMed:24969679, ECO:0000269|PubMed:29507376, ECO:0000269|PubMed:9806540}. Note=The disease is caused by variants affecting the gene represented in this entry.

Disease: Cholestasis, benign recurrent intrahepatic, 2 (BRIC2) [MIM:605479]: A disorder characterized by intermittent episodes of cholestasis without progression to liver failure. There is initial elevation of serum bile acids, followed by cholestatic jaundice which generally spontaneously resolves after periods of weeks to months. The cholestatic attacks vary in severity and duration. Patients are asymptomatic between episodes, both clinically and biochemically. {ECO:0000269|PubMed:15300568, ECO:0000269|PubMed:16039748, ECO:0000269|PubMed:24711118}. Note=The disease is caused by variants affecting the gene represented in this entry.

Similarity: Belongs to the ABC transporter superfamily. ABCB family. Multidrug resistance exporter (TC 3.A.1.201) subfamily. {ECO:0000305}.

Annotations taken from UniProtKB at the EBI.


Organism:		Homo sapiens (Human).
Taxonomy:		Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo.



Function:		Catalyzes the transport of the major hydrophobic bile salts, such as taurine and glycine-conjugated cholic acid across the canalicular membrane of hepatocytes in an ATP-dependent manner, therefore participates to hepatic bile acids homeostasis and consequently to lipid homeostasis through regulation of biliary lipid secretion in a bile salts dependent manner (PubMed:16332456, PubMed:22262466, PubMed:15791618, PubMed:18985798, PubMed:19228692, PubMed:20398791, PubMed:24711118, PubMed:29507376, PubMed:20010382, PubMed:32203132). Transports taurine-conjugated bile salts more rapidly than glycine-conjugated bile salts (PubMed:16332456). Also transports non-bile acid compounds, such as pravastatin and fexofenadine in an ATP-dependent manner and may be involved in their biliary excretion (PubMed:15901796, PubMed:18245269). {ECO:0000269\|PubMed:15791618, ECO:0000269\|PubMed:15901796, ECO:0000269\|PubMed:16332456, ECO:0000269\|PubMed:18245269, ECO:0000269\|PubMed:18985798, ECO:0000269\|PubMed:19228692, ECO:0000269\|PubMed:20010382, ECO:0000269\|PubMed:20398791, ECO:0000269\|PubMed:22262466, ECO:0000269\|PubMed:24711118, ECO:0000269\|PubMed:29507376, ECO:0000269\|PubMed:32203132}.


Catalytic activity:		Reaction=ATP + cholate(in) + H2O = ADP + cholate(out) + H(+) + phosphate; Xref=Rhea:RHEA:50048, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:29747, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269\|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50049; Evidence={ECO:0000269\|PubMed:16332456};
Catalytic activity:		Reaction=ATP + H2O + taurocholate(in) = ADP + H(+) + phosphate + taurocholate(out); Xref=Rhea:RHEA:50052, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:36257, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269\|PubMed:15791618, ECO:0000269\|PubMed:15901796, ECO:0000269\|PubMed:16332456, ECO:0000269\|PubMed:18985798, ECO:0000269\|PubMed:19228692, ECO:0000269\|PubMed:20010382, ECO:0000269\|PubMed:20398791, ECO:0000269\|PubMed:22262466, ECO:0000269\|PubMed:24711118, ECO:0000269\|PubMed:29507376, ECO:0000269\|PubMed:32203132}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50053; Evidence={ECO:0000269\|PubMed:15791618, ECO:0000269\|PubMed:15901796, ECO:0000269\|PubMed:16332456, ECO:0000269\|PubMed:18985798, ECO:0000269\|PubMed:19228692, ECO:0000269\|PubMed:20010382, ECO:0000269\|PubMed:20398791, ECO:0000269\|PubMed:22262466, ECO:0000269\|PubMed:24711118, ECO:0000269\|PubMed:29507376, ECO:0000305\|PubMed:32203132};
Catalytic activity:		Reaction=ATP + glycocholate(in) + H2O = ADP + glycocholate(out) + H(+) + phosphate; Xref=Rhea:RHEA:50056, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:29746, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269\|PubMed:15791618, ECO:0000269\|PubMed:16332456, ECO:0000269\|PubMed:32203132}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50057; Evidence={ECO:0000269\|PubMed:15791618, ECO:0000269\|PubMed:16332456, ECO:0000305\|PubMed:32203132};
Catalytic activity:		Reaction=ATP + glycochenodeoxycholate(in) + H2O = ADP + glycochenodeoxycholate(out) + H(+) + phosphate; Xref=Rhea:RHEA:50060, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:36252, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269\|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50061; Evidence={ECO:0000269\|PubMed:16332456};
Catalytic activity:		Reaction=ATP + H2O + taurochenodeoxycholate(in) = ADP + H(+) + phosphate + taurochenodeoxycholate(out); Xref=Rhea:RHEA:50064, ChEBI:CHEBI:9407, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269\|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50065; Evidence={ECO:0000269\|PubMed:16332456};
Catalytic activity:		Reaction=ATP + glycoursodeoxycholate(in) + H2O = ADP + glycoursodeoxycholate(out) + H(+) + phosphate; Xref=Rhea:RHEA:50068, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:132030, ChEBI:CHEBI:456216; Evidence={ECO:0000269\|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50069; Evidence={ECO:0000269\|PubMed:16332456};
Catalytic activity:		Reaction=ATP + H2O + tauroursodeoxycholate(in) = ADP + H(+) + phosphate + tauroursodeoxycholate(out); Xref=Rhea:RHEA:50072, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:132028, ChEBI:CHEBI:456216; Evidence={ECO:0000269\|PubMed:16332456, ECO:0000269\|PubMed:32203132}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50073; Evidence={ECO:0000269\|PubMed:16332456, ECO:0000305\|PubMed:32203132};
Catalytic activity:		Reaction=ATP + H2O + taurodeoxycholate(in) = ADP + H(+) + phosphate + taurodeoxycholate(out); Xref=Rhea:RHEA:50080, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:36261, ChEBI:CHEBI:43474, ChEBI:CHEBI:456216; Evidence={ECO:0000269\|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50081; Evidence={ECO:0000269\|PubMed:16332456};
Catalytic activity:		Reaction=ATP + H2O + taurolithocholate 3-sulfate(in) = ADP + H(+) + phosphate + taurolithocholate 3-sulfate(out); Xref=Rhea:RHEA:50084, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:58301, ChEBI:CHEBI:456216; Evidence={ECO:0000269\|PubMed:16332456}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:50085; Evidence={ECO:0000269\|PubMed:16332456};
Catalytic activity:		Reaction=ATP + H2O + pravastatin(in) = ADP + H(+) + phosphate + pravastatin(out); Xref=Rhea:RHEA:63908, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:63660, ChEBI:CHEBI:456216; Evidence={ECO:0000269\|PubMed:15901796}; PhysiologicalDirection=left-to-right; Xref=Rhea:RHEA:63909; Evidence={ECO:0000305\|PubMed:15901796};
Activity regulation:		The uptake of taurocholate is inhibited by taurolithocholate sulfate with an IC(50) of 9 uM (PubMed:16332456). Pravastatin competitively inhibits the transport of taurocholic acid (PubMed:18985798, PubMed:15901796). Cyclosporin A, glibenclamide, rifampicin and troglitazonestrongly competitively inhibit the transport activity of taurocholate (PubMed:18985798, PubMed:32203132). The canalicular transport activity of taurocholate is strongly dependent on canalicular membrane cholesterol content (PubMed:19228692). The uptake of taurocholate is increased by short- and medium-chain fatty acids (PubMed:20398791). Cholesterol increases transport capacity of taurocholate without affecting the affinity for the substrate (PubMed:24711118). {ECO:0000269\|PubMed:15901796, ECO:0000269\|PubMed:16332456, ECO:0000269\|PubMed:18985798, ECO:0000269\|PubMed:19228692, ECO:0000269\|PubMed:20398791, ECO:0000269\|PubMed:24711118, ECO:0000269\|PubMed:32203132}.
Biophysicochemical properties:		Kinetic parameters: KM=30.4 uM for taurocholate {ECO:0000269\|PubMed:17264802}; KM=6.2 uM for taurocholate {ECO:0000269\|PubMed:16332456}; KM=21.7 uM for glycocholate {ECO:0000269\|PubMed:16332456}; KM=6.6 uM for taurochenodeoxycholate {ECO:0000269\|PubMed:16332456}; KM=7.5 uM for glycochenodeoxycholate {ECO:0000269\|PubMed:16332456}; KM=9.5 uM for taurolithocholate sulfate {ECO:0000269\|PubMed:16332456}; KM=4.61 uM for taurocholate {ECO:0000269\|PubMed:15791618}; KM=4.64 uM for taurocholate {ECO:0000269\|PubMed:15901796}; KM=124 uM for pravastatin {ECO:0000269\|PubMed:15901796}; KM=19.9 uM for taurocholate {ECO:0000269\|PubMed:18985798}; Vmax=232 pmol/min/mg enzyme for taurocholate transport {ECO:0000269\|PubMed:17264802}; Vmax=2510 pmol/min/mg enzyme for taurocholate transport {ECO:0000269\|PubMed:16332456}; Vmax=2310 pmol/min/mg enzyme for taurocholate transport {ECO:0000269\|PubMed:15791618}; Vmax=4290 pmol/min/mg enzyme for taurocholate transport {ECO:0000269\|PubMed:15901796}; Vmax=1220 pmol/min/mg enzyme for pravastatin transport {ECO:0000269\|PubMed:15901796}; Vmax=98.5 pmol/min/mg enzyme for taurocholate transport {ECO:0000269\|PubMed:18985798};
Subunit:		Interacts with HAX1 (By similarity). Interacts with the adapter protein complex 2 (AP-2) throught AP2A2 or AP2A1; this interaction regulates cell membrane expresion of ABCB11 through its internalization in a clathrin-dependent manner and its subsequent degradation (PubMed:22262466). {ECO:0000250\|UniProtKB:O70127, ECO:0000269\|PubMed:22262466}.
Subcellular location:		Apical cell membrane {ECO:0000269\|PubMed:15791618, ECO:0000269\|PubMed:22262466}; Multi-pass membrane protein {ECO:0000255}. Recycling endosome membrane {ECO:0000250\|UniProtKB:O70127}; Multi-pass membrane protein {ECO:0000250\|UniProtKB:O70127}. Endosome {ECO:0000250\|UniProtKB:O70127}. Cell membrane {ECO:0000269\|PubMed:20010382, ECO:0000269\|PubMed:22262466, ECO:0000269\|PubMed:29507376, ECO:0000269\|PubMed:30431138}; Multi-pass membrane protein {ECO:0000255}. Note=Internalized at the canalicular membrane through interaction with the adapter protein complex 2 (AP-2) (PubMed:22262466). At steady state, localizes in the canalicular membrane but is also present in recycling endosomes. ABCB11 constantly and rapidly exchanges between the two sites through tubulo-vesicles carriers that move along microtubules. Microtubule-dependent trafficking of ABCB11 is enhanced by taurocholate and cAMP and regulated by STK11 through a PKA-mediated pathway. Trafficking of newly synthesized ABCB11 through endosomal compartment to the bile canalicular membrane is accelerated by cAMP but not by taurocholate (By similarity). Cell membrane expression is up-regulated by short- and medium-chain fatty acids (PubMed:20398791). {ECO:0000250\|UniProtKB:O70127, ECO:0000269\|PubMed:20398791, ECO:0000269\|PubMed:22262466}.
Tissue specificity:		Expressed predominantly, if not exclusively in the liver, where it was further localized to the canalicular microvilli and to subcanalicular vesicles of the hepatocytes by in situ.
Domain:		Multifunctional polypeptide with two homologous halves, each containing a hydrophobic membrane-anchoring domain and an ATP binding cassette (ABC) domain.
Ptm:		N-glycosylated. {ECO:0000269\|PubMed:15791618, ECO:0000269\|PubMed:18829893}.
Ptm:		Ubiquitinated; short-chain ubiquitination regulates cell-Surface expression of ABCB11. {ECO:0000269\|PubMed:18829893}.
Disease:		Cholestasis, progressive familial intrahepatic, 2 (PFIC2) [MIM:601847]: A disorder characterized by early onset of cholestasis that progresses to hepatic fibrosis, cirrhosis, and end-stage liver disease before adulthood. {ECO:0000269\|PubMed:10579978, ECO:0000269\|PubMed:11815775, ECO:0000269\|PubMed:15791618, ECO:0000269\|PubMed:18829893, ECO:0000269\|PubMed:20010382, ECO:0000269\|PubMed:24969679, ECO:0000269\|PubMed:29507376, ECO:0000269\|PubMed:9806540}. Note=The disease is caused by variants affecting the gene represented in this entry.
Disease:		Cholestasis, benign recurrent intrahepatic, 2 (BRIC2) [MIM:605479]: A disorder characterized by intermittent episodes of cholestasis without progression to liver failure. There is initial elevation of serum bile acids, followed by cholestatic jaundice which generally spontaneously resolves after periods of weeks to months. The cholestatic attacks vary in severity and duration. Patients are asymptomatic between episodes, both clinically and biochemically. {ECO:0000269\|PubMed:15300568, ECO:0000269\|PubMed:16039748, ECO:0000269\|PubMed:24711118}. Note=The disease is caused by variants affecting the gene represented in this entry.
Similarity:		Belongs to the ABC transporter superfamily. ABCB family. Multidrug resistance exporter (TC 3.A.1.201) subfamily. {ECO:0000305}.