2013
Biosynthesis and trafficking of the bile salt export pump, BSEP: Therapeutic implications of BSEP mutations
Soroka CJ, Boyer JL. Biosynthesis and trafficking of the bile salt export pump, BSEP: Therapeutic implications of BSEP mutations. Molecular Aspects Of Medicine 2013, 37: 3-14. PMID: 23685087, PMCID: PMC3784619, DOI: 10.1016/j.mam.2013.05.001.Peer-Reviewed Original ResearchMeSH KeywordsATP Binding Cassette Transporter, Subfamily B, Member 11ATP-Binding Cassette TransportersBile Acids and SaltsBiliary TractCholestasis, IntrahepaticFemaleHepatocytesHumansMutationPregnancyConceptsBenign recurrent intrahepatic cholestasis type 2Progressive familial intrahepatic cholestasis type 2Cell biological aspectsBile salt export pumpTranslational regulationCell biologyMembrane transportersPrimary transporterBile salt-dependent bile flowType 2Bile acidsRate-limiting stepExport pumpDrug-induced cholestasisBiological aspectsBiosynthesisTraffickingTransportersBSEP mutationsMutationsCholestatic diseaseIntrahepatic cholestasisBile flowBiliary systemEnterohepatic circulation
2012
A C‐terminal tyrosine‐based motif in the bile salt export pump directs clathrin‐dependent endocytosis
Lam P, Xu S, Soroka CJ, Boyer JL. A C‐terminal tyrosine‐based motif in the bile salt export pump directs clathrin‐dependent endocytosis. Hepatology 2012, 55: 1901-1911. PMID: 22161577, PMCID: PMC3319652, DOI: 10.1002/hep.25523.Peer-Reviewed Original ResearchMeSH KeywordsAmino Acid MotifsAnimalsATP Binding Cassette Transporter, Subfamily B, Member 11ATP-Binding Cassette TransportersChlorocebus aethiopsClathrinCOS CellsDynaminsEndocytosisHEK293 CellsHeLa CellsHumansRab5 GTP-Binding ProteinsTyrosineConceptsTyrosine-based motifNucleotide-binding domainEndocytic motifTransmembrane segmentsCanonical tyrosine-based motifTyrosine-based endocytic motifCytoplasmic nucleotide-binding domainsClathrin-dependent pathwayClathrin-dependent endocytosisRegulation of traffickingBroad substrate specificitySite-directed mutagenesisATP-dependent transportHEK293T cellsCytoplasmic terminiEndocytosis motifCassette proteinSubsequent sequence analysisTerminal tailSubstrate specificityConstitutive internalizationReporter systemSequence analysisBile salt export pumpTerminal end
2010
The Bile Salt Export Pump: Clinical and Experimental Aspects of Genetic and Acquired Cholestatic Liver Disease
Lam P, Soroka C, Boyer J. The Bile Salt Export Pump: Clinical and Experimental Aspects of Genetic and Acquired Cholestatic Liver Disease. Seminars In Liver Disease 2010, 30: 125-133. PMID: 20422495, PMCID: PMC3008346, DOI: 10.1055/s-0030-1253222.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsATP Binding Cassette Transporter, Subfamily B, Member 11ATP-Binding Cassette TransportersBile Acids and SaltsBile CanaliculiCholestasis, IntrahepaticEndoplasmic ReticulumGlycosylationHumansMiceMice, KnockoutModels, AnimalMutationPhosphorylationUbiquitinationConceptsBenign recurrent intrahepatic cholestasis type 2Progressive familial intrahepatic cholestasis type 2Bile salt export pumpDrug-induced cholestasisType 2Export pumpCholestatic liver diseaseBile salt secretionForms of cholestasisDifferent genetic formsLiver diseaseCholestatic disordersIntrahepatic cholestasisBSEP expressionPathophysiologic processesBSEP deficiencyAnimal modelsCholestasisGenetic formsBile saltsSalt secretionMolecular characteristicsPivotal roleCell culturesPrimary transporter
2008
ATP8B1 Deficiency Disrupts the Bile Canalicular Membrane Bilayer Structure in Hepatocytes, But FXR Expression and Activity Are Maintained
Cai S, Gautam S, Nguyen T, Soroka CJ, Rahner C, Boyer JL. ATP8B1 Deficiency Disrupts the Bile Canalicular Membrane Bilayer Structure in Hepatocytes, But FXR Expression and Activity Are Maintained. Gastroenterology 2008, 136: 1060-1069.e4. PMID: 19027009, PMCID: PMC3439851, DOI: 10.1053/j.gastro.2008.10.025.Peer-Reviewed Original ResearchMeSH Keywords4-Chloro-7-nitrobenzofurazanAdenosine TriphosphatasesAnimalsATP Binding Cassette Transporter, Subfamily B, Member 11ATP-Binding Cassette TransportersBile CanaliculiCaco-2 CellsChenodeoxycholic AcidDNA-Binding ProteinsGastrointestinal AgentsGene ExpressionHepatocytesHumansMultidrug Resistance-Associated Protein 2PhosphatidylserinesPhospholipid Transfer ProteinsRatsReceptors, Cytoplasmic and NuclearRNA, Small InterferingTranscription FactorsTransfectionConceptsAminophospholipid flippaseMessenger RNAMembrane bilayer structureCanalicular membraneFarnesoid X receptorRat hepatocytesSmall heterodimer partnerMembrane transportersNBD-phosphatidylserineHeterodimer partnerDeficiency disruptsLuminal accumulationMembrane disruptionRNAConflicting hypothesesRat cellsFlippaseProtein levelsProtein expressionX receptorExpressionBSEP functionATP8B1CellsMembrane
2001
Bile salt export pump is highly conserved during vertebrate evolution and its expression is inhibited by PFIC type II mutations
Cai S, Wang L, Ballatori N, Boyer J. Bile salt export pump is highly conserved during vertebrate evolution and its expression is inhibited by PFIC type II mutations. AJP Gastrointestinal And Liver Physiology 2001, 281: g316-g322. PMID: 11447010, DOI: 10.1152/ajpgi.2001.281.2.g316.Peer-Reviewed Original ResearchMeSH KeywordsAmino Acid SequenceAnimalsATP Binding Cassette Transporter, Subfamily B, Member 11ATP-Binding Cassette TransportersBiological TransportCholestasis, IntrahepaticCloning, MolecularConserved SequenceEvolution, MolecularHumansLiverMolecular Sequence DataMutationPhylogenyRNA, MessengerSequence Homology, Amino AcidSkates, FishSpodopteraTaurocholic AcidTransfectionConceptsSf9 cellsATP-dependent transport proteinsFull-length cloneATP-dependent export pumpLiver cDNA libraryNorthern blot analysisStructure-function relationshipsVertebrate evolutionEvolutionary originHuman BSEPBile salt export pump BSEPMutant proteinsSkate Raja erinaceaHigher vertebratesCDNA libraryTransport proteinsSixfold stimulationVertebratesType II mutationAmino acidsRaja erinaceaDefective expressionBlot analysisExport pumpMutations
2000
Expression of the bile salt export pump is maintained after chronic cholestasis in the rat
Lee J, Trauner M, Soroka C, Stieger B, Meier P, Boyer J. Expression of the bile salt export pump is maintained after chronic cholestasis in the rat. Gastroenterology 2000, 118: 163-172. PMID: 10611165, DOI: 10.1016/s0016-5085(00)70425-2.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsATP Binding Cassette Transporter, Subfamily B, Member 1ATP Binding Cassette Transporter, Subfamily B, Member 11ATP-Binding Cassette TransportersBile Acids and SaltsBile DuctsCarrier ProteinsCholestasisChronic DiseaseDrug Resistance, MultipleEthinyl EstradiolLigationLipopolysaccharidesMembrane ProteinsMultidrug Resistance-Associated ProteinsOrganic Anion Transporters, Sodium-DependentRatsRats, Sprague-DawleyRNA, MessengerSymportersBile salt excretion in skate liver is mediated by a functional analog of Bsep/Spgp, the bile salt export pump
Ballatori N, Rebbeor J, Connolly G, Seward D, Lenth B, Henson J, Sundaram P, Boyer J. Bile salt excretion in skate liver is mediated by a functional analog of Bsep/Spgp, the bile salt export pump. AJP Gastrointestinal And Liver Physiology 2000, 278: g57-g63. PMID: 10644562, DOI: 10.1152/ajpgi.2000.278.1.g57.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAnimalsATP Binding Cassette Transporter, Subfamily B, Member 11ATP-Binding Cassette TransportersBile Acids and SaltsBile CanaliculiBiological TransportCholestanolsLiverMaleMembranesMolecular WeightSkates, FishTaurocholic AcidConceptsTransport activityPlasma membrane vesiclesApparent molecular massVertebrate evolutionATP-dependent componentMultidrug resistance protein 1Similar transport activityFunctional characterizationPlasma membraneApical localizationCanalicular membraneResistance protein 1Liver plasma membrane vesiclesATP-dependent uptakeSkate liverMembrane vesiclesWestern blot analysisDominant protein bandsMammalian liverMolecular massLittle skateFunctional analogueProtein 1Raja erinaceaCanalicular multidrug resistance