Gene/Proteome Database (LMPD)

LMPD ID
LMP002637
Gene ID
8647
Species
Homo sapiens (Human)
Gene Name
ATP-binding cassette, sub-family B (MDR/TAP), member 11
Gene Symbol
ABCB11
Synonyms
ABC16; BRIC2; BSEP; PFIC-2; PFIC2; PGY4; SPGP
Alternate Names
bile salt export pump; sister p-glycoprotein; ABC member 16, MDR/TAP subfamily; ATP-binding cassette sub-family B member 11; progressive familial intrahepatic cholestasis 2
Chromosome
2
Map Location
2q24
Summary
The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance. The protein encoded by this gene is the major canalicular bile salt export pump in man. Mutations in this gene cause a form of progressive familial intrahepatic cholestases which are a group of inherited disorders with severe cholestatic liver disease from early infancy. [provided by RefSeq, Jul 2008]
Orthologs
View orthologs and multiple alignments for ABCB11

Proteins

bile salt export pump
Refseq ID NP_003733
Protein GI 21536378
UniProt ID O95342
mRNA ID NM_003742
Length 1321
RefSeq Status REVIEWED
MSDSVILRSIKKFGEENDGFESDKSYNNDKKSRLQDEKKGDGVRVGFFQLFRFSSSTDIWLMFVGSLCAFLHGIAQPGVLLIFGTMTDVFIDYDVELQELQIPGKACVNNTIVWTNSSLNQNMTNGTRCGLLNIESEMIKFASYYAGIAVAVLITGYIQICFWVIAAARQIQKMRKFYFRRIMRMEIGWFDCNSVGELNTRFSDDINKINDAIADQMALFIQRMTSTICGFLLGFFRGWKLTLVIISVSPLIGIGAATIGLSVSKFTDYELKAYAKAGVVADEVISSMRTVAAFGGEKREVERYEKNLVFAQRWGIRKGIVMGFFTGFVWCLIFLCYALAFWYGSTLVLDEGEYTPGTLVQIFLSVIVGALNLGNASPCLEAFATGRAAATSIFETIDRKPIIDCMSEDGYKLDRIKGEIEFHNVTFHYPSRPEVKILNDLNMVIKPGEMTALVGPSGAGKSTALQLIQRFYDPCEGMVTVDGHDIRSLNIQWLRDQIGIVEQEPVLFSTTIAENIRYGREDATMEDIVQAAKEANAYNFIMDLPQQFDTLVGEGGGQMSGGQKQRVAIARALIRNPKILLLDMATSALDNESEAMVQEVLSKIQHGHTIISVAHRLSTVRAADTIIGFEHGTAVERGTHEELLERKGVYFTLVTLQSQGNQALNEEDIKDATEDDMLARTFSRGSYQDSLRASIRQRSKSQLSYLVHEPPLAVVDHKSTYEEDRKDKDIPVQEEVEPAPVRRILKFSAPEWPYMLVGSVGAAVNGTVTPLYAFLFSQILGTFSIPDKEEQRSQINGVCLLFVAMGCVSLFTQFLQGYAFAKSGELLTKRLRKFGFRAMLGQDIAWFDDLRNSPGALTTRLATDASQVQGAAGSQIGMIVNSFTNVTVAMIIAFSFSWKLSLVILCFFPFLALSGATQTRMLTGFASRDKQALEMVGQITNEALSNIRTVAGIGKERRFIEALETELEKPFKTAIQKANIYGFCFAFAQCIMFIANSASYRYGGYLISNEGLHFSYVFRVISAVVLSATALGRAFSYTPSYAKAKISAARFFQLLDRQPPISVYNTAGEKWDNFQGKIDFVDCKFTYPSRPDSQVLNGLSVSISPGQTLAFVGSSGCGKSTSIQLLERFYDPDQGKVMIDGHDSKKVNVQFLRSNIGIVSQEPVLFACSIMDNIKYGDNTKEIPMERVIAAAKQAQLHDFVMSLPEKYETNVGSQGSQLSRGEKQRIAIARAIVRDPKILLLDEATSALDTESEKTVQVALDKAREGRTCIVIAHRLSTIQNADIIAVMAQGVVIEKGTHEELMAQKGAYYKLVTTGSPIS

Gene Information

Entrez Gene ID
8647
Gene Name
ATP-binding cassette, sub-family B (MDR/TAP), member 11
Gene Symbol
ABCB11
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0045177 IEA:Ensembl C apical part of cell
GO:0070062 IDA:UniProt C extracellular vesicular exosome
GO:0016021 IBA:RefGenome C integral component of membrane
GO:0005887 TAS:ProtInc C integral component of plasma membrane
GO:0046581 IEA:Ensembl C intercellular canaliculus
GO:0016020 TAS:ProtInc C membrane
GO:0005886 TAS:Reactome C plasma membrane
GO:0005524 TAS:ProtInc F ATP binding
GO:0042626 IBA:RefGenome F ATPase activity, coupled to transmembrane movement of substances
GO:0015432 TAS:ProtInc F bile acid-exporting ATPase activity
GO:0015126 IBA:RefGenome F canalicular bile acid transmembrane transporter activity
GO:0008554 TAS:ProtInc F sodium-exporting ATPase activity, phosphorylative mechanism
GO:0005215 TAS:ProtInc F transporter activity
GO:0015721 TAS:Reactome P bile acid and bile salt transport
GO:0006699 TAS:Reactome P bile acid biosynthetic process
GO:0008206 TAS:Reactome P bile acid metabolic process
GO:0015722 IBA:RefGenome P canalicular bile acid transport
GO:0044281 TAS:Reactome P small molecule metabolic process
GO:0035725 TAS:GOC P sodium ion transmembrane transport
GO:0055085 IBA:RefGenome P transmembrane transport
GO:0006810 TAS:ProtInc P transport

KEGG Pathway Links

KEGG Pathway ID Description
hsa02010 ABC transporters
hsa04976 Bile secretion

Domain Information

InterPro Annotations

Accession Description
IPR003593 AAA+ ATPase domain
IPR011527 ABC transporter type 1, transmembrane domain
IPR017871 ABC transporter, conserved site
IPR003439 ABC_transporter-like
IPR027417 P-loop containing nucleoside triphosphate hydrolase

UniProt Annotations

Entry Information

Gene Name
ATP-binding cassette, sub-family B (MDR/TAP), member 11
Protein Entry
ABCBB_HUMAN
UniProt ID
O95342
Species
Human

Comments

Comment Type Description
Biophysicochemical Properties Kinetic parameters: KM=30.4 uM for taurocholate ; Vmax=232 pmol/min/mg enzyme for taurocholate transport ;
Disease Cholestasis, benign recurrent intrahepatic, 2 (BRIC2) [MIM
Disease Cholestasis, progressive familial intrahepatic, 2 (PFIC2) [MIM
Domain Multifunctional polypeptide with two homologous halves, each containing a hydrophobic membrane-anchoring domain and an ATP binding cassette (ABC) domain.
Function Involved in the ATP-dependent secretion of bile salts into the canaliculus of hepatocytes.
Similarity Belongs to the ABC transporter superfamily. ABCB family. Multidrug resistance exporter (TC 3.A.1.201) subfamily.
Similarity Contains 2 ABC transmembrane type-1 domains.
Similarity Contains 2 ABC transporter domains.
Subcellular Location Membrane; Multi-pass membrane protein.
Subunit Interacts with HAX1.
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.
Web Resource Name=ABCMdb; Note=Database for mutations in ABC proteins; URL="http://abcmutations.hegelab.org/proteinDetails?uniprot_id=O95342";

Identical and Related Proteins

Unique RefSeq proteins for LMP002637 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
21536378 RefSeq NP_003733 1321 bile salt export pump

Identical Sequences to LMP002637 proteins

Reference Database Accession Length Protein Name
GI:21536378 EMBL CAI54162.1 1321 unnamed protein product [Homo sapiens]
GI:21536378 GenBank EAX11285.1 1321 ATP-binding cassette, sub-family B (MDR/TAP), member 11, isoform CRA_a [Homo sapiens]
GI:21536378 GenBank ADA52870.1 1321 Sequence 700 from patent US 7625699
GI:21536378 SwissProt O95342.2 1321 RecName: Full=Bile salt export pump; AltName: Full=ATP-binding cassette sub-family B member 11 [Homo sapiens]

Related Sequences to LMP002637 proteins

Reference Database Accession Length Protein Name
GI:21536378 GenBank AAC77455.1 1321 bile salt export pump [Homo sapiens]
GI:21536378 GenBank AAD28285.1 1321 bile salt export pump [Homo sapiens]
GI:21536378 GenBank EAX11287.1 1330 ATP-binding cassette, sub-family B (MDR/TAP), member 11, isoform CRA_c, partial [Homo sapiens]
GI:21536378 GenBank AEH74439.1 1321 Sequence 49 from patent US 7955805
GI:21536378 RefSeq XP_003824353.1 1321 PREDICTED: bile salt export pump [Pan paniscus]
GI:21536378 RefSeq XP_006712880.1 1335 PREDICTED: bile salt export pump isoform X1 [Homo sapiens]