The Bile Salt Export Pump: Molecular Structure, Study Models and Small-Molecule Drugs for the Treatment of Inherited BSEP Deficiencies

Genotype correlates with the natural history of severe bile salt export pump deficiency

(2020) Daan B.E. van Wessel et al.   JOURNAL OF HEPATOLOGY

Cryo-EM structure of human bile salts exporter ABCB11

(2020) Liang Wang et al.   CELL RESEARCH

Functional rescue of an ABCB11 mutant by ivacaftor: a new targeted pharmacotherapy approach in bile salt export pump deficiency

(2020) Elodie Mareux et al.   LIVER INTERNATIONAL

Bile acid modulators for the treatment of nonalcoholic steatohepatitis (NASH)

(2020) Stefano Fiorucci et al.   EXPERT OPINION ON INVESTIGATIONAL DRUGS

Pharmacological premature termination codon readthrough of ABCB11 in bile salt export pump deficiency: an in vitro study

(2020) Rachida Amzal et al.   HEPATOLOGY

Human ABCB1 with an ABCB11-like degenerate nucleotide binding site maintains transport activity by avoiding nucleotide occlusion

(2020) Katalin Goda et al.   PLoS Genetics

Chaperoning Endoplasmic Reticulum–Associated Degradation (ERAD) and Protein Conformational Diseases

(2019) Patrick G. Needham et al.   Cold Spring Harbor Perspectives in Biology

Long-term efficacy and safety of obeticholic acid for patients with primary biliary cholangitis: 3-year results of an international open-label extension study

(2019) Michael Trauner et al.   Lancet Gastroenterology & Hepatology

Treating the Underlying Cystic Fibrosis Transmembrane Conductance Regulator Defect in Patients with Cystic Fibrosis

(2019) Senne Cuyx et al.   SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE

Obeticholic acid for the treatment of non-alcoholic steatohepatitis: interim analysis from a multicentre, randomised, placebo-controlled phase 3 trial

(2019) Zobair M Younossi et al.   LANCET

Mechanisms of canalicular transporter endocytosis in the cholestatic rat liver

(2018) Gisel S. Miszczuk et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Natural products as modulators of the nuclear receptors and metabolic sensors LXR, FXR and RXR

(2018) Verena Hiebl et al.   BIOTECHNOLOGY ADVANCES

Transmembrane Domain Recognition during Membrane Protein Biogenesis and Quality Control

(2018) Alina Guna et al.   CURRENT BIOLOGY

Zebrafish abcb11b mutant reveals strategies to restore bile excretion impaired by bile salt export pump deficiency

(2018) Jillian L. Ellis et al.   HEPATOLOGY

Clinical phenotype and molecular analysis of a homozygous ABCB11 mutation responsible for progressive infantile cholestasis

(2018) Kazuo Imagawa et al.   JOURNAL OF HUMAN GENETICS

Advances in therapeutic use of a drug-stimulated translational readthrough of premature termination codons

(2018) Maciej Dabrowski et al.   MOLECULAR MEDICINE

Post-translational modifications of transporters

(2018) Lindsay C. Czuba et al.   PHARMACOLOGY & THERAPEUTICS

Can BSEP Inhibition Testing In Drug Discovery And Development Reduce Liver Injury Risk? - An International Transporter Consortium Perspective

(2018) J Gerry Kenna et al.   CLINICAL PHARMACOLOGY & THERAPEUTICS

Ursodeoxycholic acid improves liver function via phenylalanine/tyrosine pathway and microbiome remodelling in patients with liver dysfunction

(2018) Da Jung Kim et al.   Scientific Reports

Rapid Disruption of Genes Specifically in Livers of Mice Using Multiplex CRISPR/Cas9 Editing

(2018) Francis P. Pankowicz et al.   GASTROENTEROLOGY

Novel features in the structure of P-glycoprotein (ABCB1) in the post-hydrolytic state as determined at 7.9 Å resolution

(2018) Nopnithi Thonghin et al.   BMC STRUCTURAL BIOLOGY

New therapeutic concepts in bile acid transport and signaling for management of cholestasis

(2017) Michael Trauner et al.   HEPATOLOGY

Structure based classification for bile salt export pump (BSEP) inhibitors using comparative structural modeling of human BSEP

(2017) Sankalp Jain et al.   JOURNAL OF COMPUTER-AIDED MOLECULAR DESIGN

Metabolic preconditioning protects BSEP / ABCB11 −/− mice against cholestatic liver injury

(2017) Claudia D. Fuchs et al.   JOURNAL OF HEPATOLOGY

Sequencing of FIC1 , BSEP and MDR3 in a large cohort of patients with cholestasis revealed a high number of different genetic variants

(2017) Carola Dröge et al.   JOURNAL OF HEPATOLOGY

Model Systems for Studying the Role of Canalicular Efflux Transporters in Drug-Induced Cholestatic Liver Disease

(2017) Bruno Stieger et al.   JOURNAL OF PHARMACEUTICAL SCIENCES

Impaired N-linked glycosylation of uptake and efflux transporters in human non-alcoholic fatty liver disease

(2017) John D. Clarke et al.   LIVER INTERNATIONAL

Molecular Mechanism of Taurocholate Transport by the Bile Salt Export Pump, an ABC Transporter Associated with Intrahepatic Cholestasis

(2017) Muhammad Imran Sohail et al.   MOLECULAR PHARMACOLOGY

A Drug Regimen for Progressive Familial Cholestasis Type 2

(2017) J Jeffrey Malatack et al.   PEDIATRICS

In Vitro Drug-Induced Liver Injury Prediction: Criteria Optimization of Efflux Transporter IC50 and Physicochemical Properties

(2017) Robert W. Yucha et al.   TOXICOLOGICAL SCIENCES

Partial external biliary diversion in bile salt export pump deficiency: Association between outcome and mutation

(2017) Philipp Ellinger et al.   WORLD JOURNAL OF GASTROENTEROLOGY

Generation of a bile salt export pump deficiency model using patient-specific induced pluripotent stem cell-derived hepatocyte-like cells

(2017) Kazuo Imagawa et al.   Scientific Reports

Biliary excretion of pravastatin and taurocholate in rats with bile salt export pump (Bsep) impairment

(2016) Yaofeng Cheng et al.   BIOPHARMACEUTICS & DRUG DISPOSITION

In vitro model systems to investigate bile salt export pump (BSEP) activity and drug interactions: A review

(2016) Yaofeng Cheng et al.   CHEMICO-BIOLOGICAL INTERACTIONS

Functional defect of variants in the adenosine triphosphate-binding sites of ABCB4 and their rescue by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770)

(2016) Jean-Louis Delaunay et al.   HEPATOLOGY

Membrane Protein Quantity Control at the Endoplasmic Reticulum

(2016) Ignat Printsev et al.   JOURNAL OF MEMBRANE BIOLOGY

Sandwich-Cultured Hepatocytes as a Tool to Study Drug Disposition and Drug-Induced Liver Injury

(2016) Kyunghee Yang et al.   JOURNAL OF PHARMACEUTICAL SCIENCES

Effects of 4-phenylbutyrate therapy in a preterm infant with cholestasis and liver fibrosis

(2016) Shogo Ito et al.   PEDIATRICS INTERNATIONAL

Bipolar and Related Disorders Induced by Sodium 4-Phenylbutyrate in a Male Adolescent with Bile Salt Export Pump Deficiency Disease

(2016) Giovanni Vitale et al.   Psychiatry Investigation

Exon-skipping and mRNA decay in human liver tissue: molecular consequences of pathogenic bile salt export pump mutations

(2016) Carola Dröge et al.   Scientific Reports

Recent advances in the exploration of the bile salt export pump (BSEP/ABCB11) function

(2015) Ágnes Telbisz et al.   EXPERT OPINION ON THERAPEUTIC TARGETS

Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrate

(2015) Emmanuel Gonzales et al.   HEPATOLOGY

Successful treatment with 4-phenylbutyrate in a patient with benign recurrent intrahepatic cholestasis type 2 refractory to biliary drainage and bilirubin absorption

(2015) Hisamitsu Hayashi et al.   HEPATOLOGY RESEARCH

Bile salt export pump: a sensitive and specific immunohistochemical marker of hepatocellular carcinoma

(2015) Stephen M Lagana et al.   HISTOPATHOLOGY

Flagging Drugs That Inhibit the Bile Salt Export Pump

(2015) Floriane Montanari et al.   MOLECULAR PHARMACEUTICS

Two Case Reports of Successful Treatment of Cholestasis With Steroids in Patients With PFIC-2

(2015) G. Engelmann et al.   PEDIATRICS

Genetics and Molecular Modeling of New Mutations of Familial Intrahepatic Cholestasis in a Single Italian Center

(2015) Isabella Giovannoni et al.   PLoS One

Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation

(2014) Kris De Boeck et al.   Journal of Cystic Fibrosis

Improved Liver Function and Relieved Pruritus after 4-Phenylbutyrate Therapy in a Patient with Progressive Familial Intrahepatic Cholestasis Type 2

(2014) Sotaro Naoi et al.   JOURNAL OF PEDIATRICS

Liver transcript analysis reveals aberrant splicing due to silent and intronic variations in the ABCB11 gene

(2014) Anne Davit-Spraul et al.   MOLECULAR GENETICS AND METABOLISM

LKB1/AMPK and PKA Control ABCB11 Trafficking and Polarization in Hepatocytes

(2014) László Homolya et al.   PLoS One

In Vitro Methods to Support Transporter Evaluation in Drug Discovery and Development

(2013) K L R Brouwer et al.   CLINICAL PHARMACOLOGY & THERAPEUTICS

Sandwich-cultured hepatocytes: utility forin vitroexploration of hepatobiliary drug disposition and drug-induced hepatotoxicity

(2013) Tom De Bruyn et al.   Expert Opinion on Drug Metabolism & Toxicology

Ursodeoxycholic acid stabilizes the bile salt export pump in the apical membrane in MDCK II cells

(2013) Tatehiro Kagawa et al.   JOURNAL OF GASTROENTEROLOGY

Clinical application of transcriptional activators of bile salt transporters

(2013) Anna Baghdasaryan et al.   MOLECULAR ASPECTS OF MEDICINE

Defining the blanks – Pharmacochaperoning of SLC6 transporters and ABC transporters

(2013) Peter Chiba et al.   PHARMACOLOGICAL RESEARCH

Structures of ABCB10, a human ATP-binding cassette transporter in apo- and nucleotide-bound states

(2013) C. A. Shintre et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Refined structures of mouse P-glycoprotein

(2013) Jingzhi Li et al.   PROTEIN SCIENCE

Discovery and structural development of small molecules that enhance transport activity of bile salt export pump mutant associated with progressive familial intrahepatic cholestasis type 2

(2012) Takashi Misawa et al.   BIOORGANIC & MEDICINAL CHEMISTRY

E297G mutated bile salt export pump (BSEP) function enhancers derived from GW4064: Structural development study and separation from farnesoid X receptor-agonistic activity

(2012) Takashi Misawa et al.   BIOORGANIC & MEDICINAL CHEMISTRY LETTERS

AP2 adaptor complex mediates bile salt export pump internalization and modulates its hepatocanalicular expression and transport function

(2012) Hisamitsu Hayashi et al.   HEPATOLOGY

Successful mutation-specific chaperone therapy with 4-phenylbutyrate in a child with progressive familial intrahepatic cholestasis type 2

(2012) Emmanuel Gonzales et al.   JOURNAL OF HEPATOLOGY

ERK1/2 and p38 MAPKs Are Complementarily Involved in Estradiol 17ß-d-Glucuronide-Induced Cholestasis: Crosstalk with cPKC and PI3K

(2012) Andrea C. Boaglio et al.   PLoS One

The bile salt export pump (BSEP) in health and disease

(2012) Ralf Kubitz et al.   Clinics and Research in Hepatology and Gastroenterology

Histone H3K4 trimethylation by MLL3 as part of ASCOM complex is critical for NR activation of bile acid transporter genes and is downregulated in cholestasis

(2011) M. Ananthanarayanan et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Cellular Energy Depletion Resets Whole-Body Energy by Promoting Coactivator-Mediated Dietary Fuel Absorption

(2011) Atul R. Chopra et al.   Cell Metabolism

The Canalicular Bile Salt Export Pump BSEP (ABCB11) as a Potential Therapeutic Target

(2011) Bruno Stieger et al.   CURRENT DRUG TARGETS

Localization status of hepatocellular transporters in cholestasis

(2011) Fernando et al.   Frontiers in Bioscience-Landmark

Alterations in Lipid Metabolism Mediate Inflammation, Fibrosis, and Proliferation in a Mouse Model of Chronic Cholestatic Liver Injury

(2011) Tarek Moustafa et al.   GASTROENTEROLOGY

A C-terminal tyrosine-based motif in the bile salt export pump directs clathrin-dependent endocytosis

(2011) Ping Lam et al.   HEPATOLOGY

Interaction of bile salts with rat canalicular membrane vesicles: Evidence for bile salt resistant microdomains

(2011) Christelle Guyot et al.   JOURNAL OF HEPATOLOGY

Bile acid stimulates hepatocyte polarization through a cAMP-Epac-MEK-LKB1-AMPK pathway

(2011) D. Fu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Short- and medium-chain fatty acids enhance the cell surface expression and transport capacity of the bile salt export pump (BSEP/ABCB11)

(2010) Takuya Kato et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Sandwich-cultured hepatocytes: anin vitromodel to evaluate hepatobiliary transporter-based drug interactions and hepatotoxicity

(2010) Brandon Swift* et al.   DRUG METABOLISM REVIEWS

Phosphoinositide 3-kinase/protein kinase B signaling pathway is involved in estradiol 17β-d-glucuronide-induced cholestasis: Complementarity with classical protein kinase c

(2010) Andrea C. Boaglio et al.   HEPATOLOGY

The Bile Salt Export Pump: Clinical and Experimental Aspects of Genetic and Acquired Cholestatic Liver Disease

(2010) Ping Lam et al.   SEMINARS IN LIVER DISEASE

The mechanism of ABC transporters: general lessons from structural and functional studies of an antigenic peptide transporter

(2009) Erik Procko et al.   FASEB JOURNAL

Compensatory role of P-glycoproteins in knockout mice lacking the bile salt export pump

(2009) Renxue Wang et al.   HEPATOLOGY

De novo bile salt transporter antibodies as a possible cause of recurrent graft failure after liver transplantation: A novel mechanism of cholestasis

(2009) Verena Keitel et al.   HEPATOLOGY

Side chain structure determines unique physiologic and therapeutic properties of norursodeoxycholic acid in Mdr2−/− mice

(2009) Emina Halilbasic et al.   HEPATOLOGY

Nuclear factor erythroid 2-related factor 2 is a positive regulator of human bile salt export pump expression

(2009) Jittima Weerachayaphorn et al.   HEPATOLOGY

Polymorphic variants in the human bile salt export pump (BSEP; ABCB11): functional characterization and interindividual variability

(2009) Richard H. Ho et al.   Pharmacogenetics and Genomics

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

(2009) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Severe Bile Salt Export Pump Deficiency: 82 Different ABCB11 Mutations in 109 Families

(2008) Sandra S. Strautnieks et al.   GASTROENTEROLOGY

Missense mutations and single nucleotide polymorphisms in ABCB11 impair bile salt export pump processing and function or disrupt pre-messenger RNA splicing

(2008) Jane A. Byrne et al.   HEPATOLOGY

Low retinol levels differentially modulate bile salt-induced expression of human and mouse hepatic bile salt transporters

(2008) Martijn O. Hoeke et al.   HEPATOLOGY

Degradation of the bile salt export pump at endoplasmic reticulum in progressive familial intrahepatic cholestasis type II

(2008) Lin Wang et al.   HEPATOLOGY

Ca2+-dependent protein kinase C isoforms are critical to estradiol 17β-D-glucuronide-induced cholestasis in the rat

(2008) Fernando A. Crocenzi et al.   HEPATOLOGY

Liver receptor homolog 1 transcriptionally regulates human bile salt export pump expression

(2008) Xiulong Song et al.   JOURNAL OF LIPID RESEARCH

Involvement of Multiple Efflux Transporters in Hepatic Disposition of Fexofenadine

(2008) S. Matsushima et al.   MOLECULAR PHARMACOLOGY

Phenotypic differences in PFIC2 and BRIC2 correlate with protein stability of mutant Bsep and impaired taurocholate secretion in MDCK II cells

(2007) Tatehiro Kagawa et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY