title: Recent advances in the exploration of the bile salt export pump (BSEP/ABCB11) function.
abstract: BACKGROUND
  The bile salt export pump (BSEP/ABCB11), residing in the apical membrane of hepatocyte, mediates the secretion of bile salts into the bile. A range of human diseases is associated with the malfunction of BSEP, including fatal hereditary liver disorders and mild cholestatic conditions. Manifestation of these diseases primarily depends on the mutation type; however, other factors such as hormonal changes and drug interactions can also trigger or influence the related diseases.
  
  
  METHODS
  Here, we summarize the recent knowledge on BSEP by covering its transport properties, cellular localization, regulation and major mutations/polymorphisms, as well as the hereditary and acquired diseases associated with BSEP dysfunction. We discuss the different model expression systems employed to understand the function of the BSEP variants, their drug interactions and the contemporary therapeutic interventions.
  
  
  CONCLUSIONS
  The limitations of the available model expression systems for BSEP result in controversial conclusions, and obstruct our deeper insight into BSEP deficiencies and BSEP-related drug interactions. The knowledge originating from different methodologies, such as clinical studies, molecular genetics, as well as in vitro and in silico modeling, should be integrated and harmonized. Increasing availability of robust molecular biological tools and our better understanding of the mechanism of BSEP deficiencies should make the personalized, mutation-based therapeutic interventions more attainable.
authors.affiliation: a Institute of Enzymology, Research Centre for Natural Sciences , Hungarian Academy of Sciences , Magyar tudósok körútja 2, Budapest 1117 , Hungary.
authors.forename: Ágnes
authors.initials: Á
authors.lastname: Telbisz
authors.affiliation: a Institute of Enzymology, Research Centre for Natural Sciences , Hungarian Academy of Sciences , Magyar tudósok körútja 2, Budapest 1117 , Hungary.
authors.forename: László
authors.initials: L
authors.lastname: Homolya
mesh_terms: D000074020:ATP Binding Cassette Transporter, Subfamily B, Member 11; D018528:ATP-Binding Cassette Transporters / Q000235:genetics / Q000378:metabolism*; D000818:Animals; D001647:Bile Acids and Salts / Q000378:metabolism*; D002779:Cholestasis / Q000235:genetics / Q000503:physiopathology*; D003198:Computer Simulation; D022781:Hepatocytes / Q000378:metabolism; D006801:Humans; D008107:Liver Diseases / Q000235:genetics / Q000503:physiopathology*; D008967:Molecular Biology; D009154:Mutation; D011110:Polymorphism, Genetic
chemical_list: C093491:ABCB11 protein, human; D000074020:ATP Binding Cassette Transporter, Subfamily B, Member 11; D018528:ATP-Binding Cassette Transporters; D001647:Bile Acids and Salts
keywords: BSEP/ABCB11; bile salt transport; cholestasis; in vitro models; mutations; pharmacological correction