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

Improved survival of porcine acute liver failure by a bioartificial liver device implanted with induced human functional hepatocytes

(2016) Xiao-Lei Shi et al.   CELL RESEARCH

Biliary atresia and other cholestatic childhood diseases: Advances and future challenges

(2016) Henkjan J. Verkade et al.   JOURNAL OF HEPATOLOGY

Molecular Genetic Dissection and Neonatal/Infantile Intrahepatic Cholestasis Using Targeted Next-Generation Sequencing

(2016) Takao Togawa et al.   JOURNAL OF PEDIATRICS

Pluripotent stem cells in disease modelling and drug discovery

(2016) Yishai Avior et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mutations in the nuclear bile acid receptor FXR cause progressive familial intrahepatic cholestasis

(2016) Natalia Gomez-Ospina et al.   Nature Communications

Rho-kinase/myosin light chain kinase pathway plays a key role in the impairment of bile canaliculi dynamics induced by cholestatic drugs

(2016) Ahmad Sharanek et al.   Scientific Reports

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

Potential of human induced pluripotent stem cells in studies of liver disease

(2015) Fotios Sampaziotis et al.   HEPATOLOGY

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

(2015) Emmanuel Gonzales et al.   HEPATOLOGY

Bile salt export pump-reactive antibodies form a polyclonal, multi-inhibitory response in antibody-induced bile salt export pump deficiency

(2015) Jan Stindt et al.   HEPATOLOGY

Direct Reprogramming of Human Fibroblasts to Functional and Expandable Hepatocytes

(2014) Pengyu Huang et al.   Cell Stem Cell

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

Mutations in TJP2 cause progressive cholestatic liver disease

(2014) Melissa Sambrotta et al.   NATURE GENETICS

Prediction of interindividual differences in hepatic functions and drug sensitivity by using human iPS-derived hepatocytes

(2014) Kazuo Takayama et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Long-Term Self-Renewal of Human ES/iPS-Derived Hepatoblast-like Cells on Human Laminin 111-Coated Dishes

(2013) Kazuo Takayama et al.   Stem Cell Reports

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

(2012) Hisamitsu Hayashi et al.   HEPATOLOGY

Generation of metabolically functioning hepatocytes from human pluripotent stem cells by FOXA2 and HNF1α transduction

(2012) Kazuo Takayama et al.   JOURNAL OF 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

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

(2011) Ping Lam et al.   HEPATOLOGY

ATP8B1 and ABCB11 analysis in 62 children with normal gamma-glutamyl transferase progressive familial intrahepatic cholestasis (PFIC): Phenotypic differences between PFIC1 and PFIC2 and natural history

(2010) Anne Davit-Spraul et al.   HEPATOLOGY

Relapsing features of bile salt export pump deficiency after liver transplantation in two patients with progressive familial intrahepatic cholestasis type 2

(2010) Giuseppe Maggiore et al.   JOURNAL OF HEPATOLOGY

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

(2009) Renxue Wang et al.   HEPATOLOGY

Efficient induction of transgene-free human pluripotent stem cells using a vector based on Sendai virus, an RNA virus that does not integrate into the host genome

(2009) Noemi FUSAKI et al.   PROCEEDINGS OF THE JAPAN ACADEMY SERIES B-PHYSICAL AND BIOLOGICAL SCIENCES

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

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

(2008) Lin Wang et al.   HEPATOLOGY