The Bile Salt Export Pump: Molecular Structure, Study Models and Small-Molecule Drugs for the Treatment of Inherited BSEP Deficiencies
出版年份 2021 全文链接
标题
The Bile Salt Export Pump: Molecular Structure, Study Models and Small-Molecule Drugs for the Treatment of Inherited BSEP Deficiencies
作者
关键词
-
出版物
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 22, Issue 2, Pages 784
出版商
MDPI AG
发表日期
2021-01-15
DOI
10.3390/ijms22020784
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注意:仅列出部分参考文献,下载原文获取全部文献信息。- 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
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