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

Liver international, 2020-08, Vol.40 (8), p.1917-1925

2020

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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)

Hepatology (Baltimore, Md.), 2017-02, Vol.65 (2), p.560-570

2017

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Inhibition of receptor-interacting protein kinase 1 improves experimental non-alcoholic fatty liver disease

Journal of hepatology, 2020-04, Vol.72 (4), p.627-635

2020

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A functional classification of ABCB4 variations causing progressive familial intrahepatic cholestasis type 3

Hepatology (Baltimore, Md.), 2016-05, Vol.63 (5), p.1620-1631

2016

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Effect of CFTR correctors on the traffic and the function of intracellularly retained ABCB4 variants

Liver international, 2021-06, Vol.41 (6), p.1344-1357

2021

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Molecular Regulation of Canalicular ABC Transporters

International journal of molecular sciences, 2021-02, Vol.22 (4), p.2113

2021

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Ivacaftor-Mediated Potentiation of ABCB4 Missense Mutations Affecting Critical Motifs of the NBDs: Repositioning Perspectives for Hepatobiliary Diseases

International journal of molecular sciences, 2023-01, Vol.24 (2), p.1236

2023

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RAB10 Interacts with ABCB4 and Regulates Its Intracellular Traffic

International journal of molecular sciences, 2021-06, Vol.22 (13), p.7087

2021

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Basolateral internalization of GPI-anchored proteins occurs via a clathrin-independent flotillin-dependent pathway in polarized hepatic cells

Molecular biology of the cell, 2009-09, Vol.20 (17), p.3792-3800

2009

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Structural analogues of roscovitine rescue the intracellular traffic and the function of ER-retained ABCB4 variants in cell models

Scientific reports, 2019-04, Vol.9 (1), p.6653, Article 6653

2019

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Phosphorylation of ABCB4 impacts its function: Insights from disease‐causing mutations

Hepatology (Baltimore, Md.), 2014-08, Vol.60 (2), p.610-621

2014

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A PDZ-Like Motif in the Biliary Transporter ABCB4 Interacts with the Scaffold Protein EBP50 and Regulates ABCB4 Cell Surface Expression

PloS one, 2016-01, Vol.11 (1), p.e0146962-e0146962

2016

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Oligomerization is required for normal endocytosis/transcytosis of a GPI-anchored protein in polarized hepatic cells

Journal of cell science, 2013-08, Vol.126 (Pt 15), p.3409-3416

2013

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MRCK-Alpha and Its Effector Myosin II Regulatory Light Chain Bind ABCB4 and Regulate Its Membrane Expression

Cells (Basel, Switzerland), 2022-02, Vol.11 (4), p.617

2022

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A missense mutation in ABCB4 gene involved in progressive familial intrahepatic cholestasis type 3 leads to a folding defect that can be rescued by low temperature

Hepatology (Baltimore, Md.), 2009-04, Vol.49 (4), p.1218-1227

2009

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The necroptosis-inducing pseudokinase mixed lineage kinase domain-like regulates the adipogenic differentiation of pre-adipocytes

iScience, 2022-10, Vol.25 (10), p.105166-105166, Article 105166

2022

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Comparison of in silico prediction and experimental assessment of ABCB4 variants identified in patients with biliary diseases

The international journal of biochemistry & cell biology, 2017-08, Vol.89, p.101-109

2017

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Effects of Cellular, Chemical, and Pharmacological Chaperones on the Rescue of a Trafficking-defective Mutant of the ATP-binding Cassette Transporter Proteins ABCB1/ABCB4

The Journal of biological chemistry, 2012-02, Vol.287 (7), p.5070-5078

2012

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ABCB4: Insights from pathobiology into therapy

Clinics and research in hepatology and gastroenterology, 2014-10, Vol.38 (5), p.557-563

2014

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Sphingolipid trafficking and protein sorting in epithelial cells

FEBS Letters, 2002-10, Vol.529 (1), p.54-59

2002

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Raft-mediated trafficking of apical resident proteins occurs in both direct and transcytotic pathways in polarized hepatic cells: role of distinct lipid microdomains

Molecular biology of the cell, 2003-02, Vol.14 (2), p.611-624

2003

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Membrane dynamics and cell polarity: the role of sphingolipids

Journal of lipid research, 2003-05, Vol.44 (5), p.869-877

2003

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Apical Secretion and Sialylation of Soluble Dipeptidyl Peptidase IV Are Two Related Events

Experimental cell research, 2000-07, Vol.258 (1), p.184-194

2000

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Ivacaftor-Mediated Potentiation of ABCB4 Missense Mutations Affecting Critical Motifs of the NBDs: Repositioning Perspectives for Hepatobiliary Diseases

International journal of molecular sciences, 2023-01, Vol.24

2023

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Membrane domains and polarized trafficking of sphingolipids

Seminars in cell & developmental biology, 2001-04, Vol.12 (2), p.149-161

2001

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

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)

Inhibition of receptor-interacting protein kinase 1 improves experimental non-alcoholic fatty liver disease

A functional classification of ABCB4 variations causing progressive familial intrahepatic cholestasis type 3

Effect of CFTR correctors on the traffic and the function of intracellularly retained ABCB4 variants

Molecular Regulation of Canalicular ABC Transporters

Ivacaftor-Mediated Potentiation of ABCB4 Missense Mutations Affecting Critical Motifs of the NBDs: Repositioning Perspectives for Hepatobiliary Diseases

RAB10 Interacts with ABCB4 and Regulates Its Intracellular Traffic

Basolateral internalization of GPI-anchored proteins occurs via a clathrin-independent flotillin-dependent pathway in polarized hepatic cells

Structural analogues of roscovitine rescue the intracellular traffic and the function of ER-retained ABCB4 variants in cell models

Phosphorylation of ABCB4 impacts its function: Insights from disease‐causing mutations

A PDZ-Like Motif in the Biliary Transporter ABCB4 Interacts with the Scaffold Protein EBP50 and Regulates ABCB4 Cell Surface Expression

Oligomerization is required for normal endocytosis/transcytosis of a GPI-anchored protein in polarized hepatic cells

MRCK-Alpha and Its Effector Myosin II Regulatory Light Chain Bind ABCB4 and Regulate Its Membrane Expression

A missense mutation in ABCB4 gene involved in progressive familial intrahepatic cholestasis type 3 leads to a folding defect that can be rescued by low temperature

The necroptosis-inducing pseudokinase mixed lineage kinase domain-like regulates the adipogenic differentiation of pre-adipocytes

Comparison of in silico prediction and experimental assessment of ABCB4 variants identified in patients with biliary diseases

Effects of Cellular, Chemical, and Pharmacological Chaperones on the Rescue of a Trafficking-defective Mutant of the ATP-binding Cassette Transporter Proteins ABCB1/ABCB4

ABCB4: Insights from pathobiology into therapy

Sphingolipid trafficking and protein sorting in epithelial cells

Raft-mediated trafficking of apical resident proteins occurs in both direct and transcytotic pathways in polarized hepatic cells: role of distinct lipid microdomains

Membrane dynamics and cell polarity: the role of sphingolipids

Apical Secretion and Sialylation of Soluble Dipeptidyl Peptidase IV Are Two Related Events

Ivacaftor-Mediated Potentiation of ABCB4 Missense Mutations Affecting Critical Motifs of the NBDs: Repositioning Perspectives for Hepatobiliary Diseases

Membrane domains and polarized trafficking of sphingolipids

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