Primary biliary cholangitis: pathogenesis and therapeutic opportunities

Ursodeoxycholic acid therapy and liver transplant-free survival in patients with primary biliary cholangitis

(2019) Maren H. Harms et al.   JOURNAL OF HEPATOLOGY

Role of the bicarbonate-responsive soluble adenylyl cyclase in cholangiocyte apoptosis in primary biliary cholangitis; a new hypothesis

(2018) Jung-Chin Chang et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

The microbiome and autoimmunity: a paradigm from the gut–liver axis

(2018) Bo Li et al.   Cellular & Molecular Immunology

Farnesoid X receptor modulators 2014-present: a patent review

(2018) Valentina Sepe et al.   EXPERT OPINION ON THERAPEUTIC PATENTS

MicroRNA-506 promotes primary biliary cholangitis-like features in cholangiocytes and immune activation

(2018) Oihane Erice et al.   HEPATOLOGY

The interplay of type I and type II interferons in murine autoimmune cholangitis as a basis for sex-biased autoimmunity

(2018) Heekyong R. Bae et al.   HEPATOLOGY

Primary biliary cholangitis, DNA, and beyond: The Relative contribution of genes

(2018) Aliya F. Gulamhusein et al.   HEPATOLOGY

MAIT cells are chronically activated in patients with autoimmune liver disease and promote profibrogenic hepatic stellate cell activation

(2018) Katrin Böttcher et al.   HEPATOLOGY

Modulation of the intestinal bile acid/farnesoid X receptor/fibroblast growth factor 15 axis improves alcoholic liver disease in mice

(2018) Phillipp Hartmann et al.   HEPATOLOGY

Linking the gut and liver: crosstalk between regulatory T cells and mucosa-associated invariant T cells

(2018) Muhammad Atif et al.   Hepatology International

Persistent reduction of mucosal-associated invariant T cells in primary biliary cholangitis

(2018) Toru Setsu et al.   JOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY

Results of a randomised controlled trial of budesonide add-on therapy in patients with primary biliary cholangitis and an incomplete response to ursodeoxycholic acid

(2018) G. Hirschfield et al.   JOURNAL OF HEPATOLOGY

Identification of a xenobiotic as a potential environmental trigger in primary biliary cholangitis

(2018) Philip M. Probert et al.   JOURNAL OF HEPATOLOGY

Bilirubin in the Liver–Gut Signaling Axis

(2018) Abdul-Rizaq Hamoud et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

Primary biliary cholangitis: a tale of epigenetically-induced secretory failure?

(2018) Pedro M. Rodrigues et al.   JOURNAL OF HEPATOLOGY

Pilot study with IBAT inhibitor A4250 for the treatment of cholestatic pruritus in primary biliary cholangitis

(2018) Samer Al-Dury et al.   Scientific Reports

Influence of gut microbiota on the development and progression of nonalcoholic steatohepatitis

(2017) Fabiana de Faria Ghetti et al.   EUROPEAN JOURNAL OF NUTRITION

A functional characteristic of cysteine-rich protein 61: Modulation of myeloid-derived suppressor cells in liver inflammation

(2017) Haiyan Zhang et al.   HEPATOLOGY

Chemokine receptor CXCR3 deficiency exacerbates murine autoimmune cholangitis by promoting pathogenic CD8+ T cell activation

(2017) Hong-Di Ma et al.   JOURNAL OF AUTOIMMUNITY

Immunoglobulin A and liver diseases

(2017) Tatsuo Inamine et al.   JOURNAL OF GASTROENTEROLOGY

miR-425 regulates inflammatory cytokine production in CD4 + T cells via N-Ras upregulation in primary biliary cholangitis

(2017) Ryo Nakagawa et al.   JOURNAL OF HEPATOLOGY

Targeting the gut-liver axis in liver disease

(2017) Reiner Wiest et al.   JOURNAL OF HEPATOLOGY

The gut microbiota contributes to a mouse model of spontaneous bile duct inflammation

(2017) Elisabeth Schrumpf et al.   JOURNAL OF HEPATOLOGY

Steroidal or non-steroidal FXR agonists – Is that the question?

(2017) Len Verbeke et al.   JOURNAL OF HEPATOLOGY

Effect of ileal bile acid transporter inhibitor GSK2330672 on pruritus in primary biliary cholangitis: a double-blind, randomised, placebo-controlled, crossover, phase 2a study

(2017) Vinod S Hegade et al.   LANCET

High-definition PBC: biology, models and therapeutic advances

(2017) Gwilym J. Webb et al.   Nature Reviews Gastroenterology & Hepatology

Dysregulation of Circulating Tfr/Tfh Ratio in Primary biliary cholangitis

(2017) J. Zheng et al.   SCANDINAVIAN JOURNAL OF IMMUNOLOGY

Principal contribution of HLA-DQ alleles, DQB1*06:04 and DQB1*03:01, to disease resistance against primary biliary cholangitis in a Japanese population

(2017) Michio Yasunami et al.   Scientific Reports

Pathogenesis of Kupffer Cells in Cholestatic Liver Injury

(2016) Keisaku Sato et al.   AMERICAN JOURNAL OF PATHOLOGY

The Immunogenetics of Autoimmune Cholestasis

(2016) Palak J. Trivedi et al.   Clinics in Liver Disease

T follicular helper cells in human autoimmunity

(2016) Hideki Ueno   CURRENT OPINION IN IMMUNOLOGY

Chronic expression of interferon-gamma leads to murine autoimmune cholangitis with a female predominance

(2016) Heekyong R. Bae et al.   HEPATOLOGY

Soluble Adenylyl Cyclase Regulates Bile Salt-Induced Apoptosis in Human Cholangiocytes

(2016) Jung-Chin Chang et al.   HEPATOLOGY

Biliary epithelium and liver B cells exposed to bacteria activate intrahepatic MAIT cells through MR1

(2016) Hannah C. Jeffery et al.   JOURNAL OF HEPATOLOGY

Discovery of 3α,7α,11β-Trihydroxy-6α-ethyl-5β-cholan-24-oic Acid (TC-100), a Novel Bile Acid as Potent and Highly Selective FXR Agonist for Enterohepatic Disorders

(2016) Roberto Pellicciari et al.   JOURNAL OF MEDICINAL CHEMISTRY

Apoptosis in response to microbial infection induces autoreactive TH17 cells

(2016) Laura Campisi et al.   NATURE IMMUNOLOGY

A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis

(2016) Frederik Nevens et al.   NEW ENGLAND JOURNAL OF MEDICINE

Activation of necroptosis in human and experimental cholestasis

(2016) Marta B Afonso et al.   Cell Death & Disease

The gut microbiome in autoimmunity: Sex matters

(2015) Andres Gomez et al.   CLINICAL IMMUNOLOGY

Follicles, germinal centers, and immune mechanisms in primary biliary cirrhosis

(2015) Gwilym J. Webb et al.   HEPATOLOGY

Natural killer cells regulate T cell immune responses in primary biliary cirrhosis

(2015) Shinji Shimoda et al.   HEPATOLOGY

Absence of the intestinal microbiota exacerbates hepatobiliary disease in a murine model of primary sclerosing cholangitis

(2015) James H. Tabibian et al.   HEPATOLOGY

CXCR5+CD4+T follicular helper cells participate in the pathogenesis of primary biliary cirrhosis

(2015) Lifeng Wang et al.   HEPATOLOGY

Fibrates and cholestasis

(2015) Nisanne S. Ghonem et al.   HEPATOLOGY

Interleukin-17 and innate immunity in infections and chronic inflammation

(2015) Natasa Isailovic et al.   JOURNAL OF AUTOIMMUNITY

A possible involvement of endoplasmic reticulum stress in biliary epithelial autophagy and senescence in primary biliary cirrhosis

(2015) Motoko Sasaki et al.   JOURNAL OF GASTROENTEROLOGY

New paradigms in the treatment of hepatic cholestasis: From UDCA to FXR, PXR and beyond

(2015) Ulrich Beuers et al.   JOURNAL OF HEPATOLOGY

Molecular mechanism of PPARα action and its impact on lipid metabolism, inflammation and fibrosis in non-alcoholic fatty liver disease

(2015) Michal Pawlak et al.   JOURNAL OF HEPATOLOGY

Testosterone Suppresses Hepatic Inflammation by the Downregulation of IL-17, CXCL-9, and CXCL-10 in a Mouse Model of Experimental Acute Cholangitis

(2015) Dorothee Schwinge et al.   JOURNAL OF IMMUNOLOGY

Genome-Wide Association Studies in Primary Biliary Cirrhosis

(2015) Aliya Gulamhusein et al.   SEMINARS IN LIVER DISEASE

International genome-wide meta-analysis identifies new primary biliary cirrhosis risk loci and targetable pathogenic pathways

(2015) Heather J. Cordell et al.   Nature Communications

Fenofibrate is effective adjunctive therapy in the treatment of primary biliary cirrhosis: A meta-analysis

(2015) Alla Y. Grigorian et al.   Clinics and Research in Hepatology and Gastroenterology

Intestinal permeability – a new target for disease prevention and therapy

(2014) Stephan C Bischoff et al.   BMC GASTROENTEROLOGY

TGR5: Pathogenetic Role and/or Therapeutic Target in Fibrosing Cholangitis?

(2014) Verena Keitel et al.   CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY

The bile acid TGR5 membrane receptor: From basic research to clinical application

(2014) Henri Duboc et al.   DIGESTIVE AND LIVER DISEASE

Levels of Alkaline Phosphatase and Bilirubin Are Surrogate End Points of Outcomes of Patients With Primary Biliary Cirrhosis: An International Follow-up Study

(2014) Willem J. Lammers et al.   GASTROENTEROLOGY

The Bile Acid Receptor TGR5 Activates the TRPA1 Channel to Induce Itch in Mice

(2014) TinaMarie Lieu et al.   GASTROENTEROLOGY

The transrepressive activity of peroxisome proliferator-activated receptor alpha is necessary and sufficient to prevent liver fibrosis in mice

(2014) Michal Pawlak et al.   HEPATOLOGY

Vitamin D modulates biliary fibrosis in ABCB4-deficient mice

(2014) Katrin Hochrath et al.   Hepatology International

Combination therapy of bezafibrate and ursodeoxycholic acid for primary biliary cirrhosis: A meta-analysis

(2014) Yan Zhang et al.   HEPATOLOGY RESEARCH

Post-translational Regulation of the Type III Inositol 1,4,5-Trisphosphate Receptor by miRNA-506

(2014) Meenakshisundaram Ananthanarayanan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Modification on Ursodeoxycholic Acid (UDCA) Scaffold. Discovery of Bile Acid Derivatives As Selective Agonists of Cell-Surface G-Protein Coupled Bile Acid Receptor 1 (GP-BAR1)

(2014) Valentina Sepe et al.   JOURNAL OF MEDICINAL CHEMISTRY

Crystal structures of human soluble adenylyl cyclase reveal mechanisms of catalysis and of its activation through bicarbonate

(2014) S. Kleinboelting et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Breach of Tolerance: Primary Biliary Cirrhosis

(2014) Lifeng Wang et al.   SEMINARS IN LIVER DISEASE

Environmental Factors in Primary Biliary Cirrhosis

(2014) Brian Juran et al.   SEMINARS IN LIVER DISEASE

Animal Models of Primary Biliary Cirrhosis

(2014) Jinjun Wang et al.   SEMINARS IN LIVER DISEASE

Glucocorticoid receptor-dependent immunomodulatory effect of ursodeoxycholic acid on liver lymphocytes in mice

(2013) Toshimichi Takigawa et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

The Immunobiology and Pathophysiology of Primary Biliary Cirrhosis

(2013) Gideon M. Hirschfield et al.   Annual Review of Pathology-Mechanisms of Disease

A Vitamin D Receptor/SMAD Genomic Circuit Gates Hepatic Fibrotic Response

(2013) Ning Ding et al.   CELL

Escherichia coliinfection induces autoimmune cholangitis and anti-mitochondrial antibodies in non-obese diabetic (NOD).B6 (Idd10/Idd18) mice

(2013) J. J. Wang et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

Apotopes and innate immune system: Novel players in the primary biliary cirrhosis scenario

(2013) Ana Lleo et al.   DIGESTIVE AND LIVER DISEASE

Pathway-based analysis of primary biliary cirrhosis genome-wide association studies

(2013) S P Kar et al.   GENES AND IMMUNITY

The receptor TGR5 protects the liver from bile acid overload during liver regeneration in mice

(2013) Noémie Péan et al.   HEPATOLOGY

Peroxisome proliferator-activated receptor α activates human multidrug resistance transporter 3/ATP-binding cassette protein subfamily B4 transcription and increases rat biliary phosphatidylcholine secretion

(2013) Nisanne S. Ghonem et al.   HEPATOLOGY

IL-12/Th1 and IL-23/Th17 biliary microenvironment in primary biliary cirrhosis: Implications for therapy

(2013) Chen-Yen Yang et al.   HEPATOLOGY

Gender Bias in Autoimmunity Is Influenced by Microbiota

(2013) Leonid Yurkovetskiy et al.   IMMUNITY

Overexpression of microRNA-21 is associated with elevated pro-inflammatory cytokines in dominant-negative TGF-β receptor type II mouse

(2013) Yugo Ando et al.   JOURNAL OF AUTOIMMUNITY

Intestinal CYP3A4 protects against lithocholic acid-induced hepatotoxicity in intestine-specific VDR-deficient mice

(2013) Jie Cheng et al.   JOURNAL OF LIPID RESEARCH

Peroxisome Proliferator-Activated Receptor Targets for the Treatment of Metabolic Diseases

(2013) Francisco A. Monsalve et al.   MEDIATORS OF INFLAMMATION

Sex Differences in the Gut Microbiome Drive Hormone-Dependent Regulation of Autoimmunity

(2013) J. G. M. Markle et al.   SCIENCE

Expression of bile acid receptor TGR5 in gastric adenocarcinoma

(2012) Weibiao Cao et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Peroxisome proliferator-activated receptor δ agonist attenuates hepatic steatosis by anti-inflammatory mechanism

(2012) Mi Young Lee et al.   EXPERIMENTAL AND MOLECULAR MEDICINE

Glucocorticoids Promote Hepatic Cholestasis in Mice by Inhibiting the Transcriptional Activity of the Farnesoid X Receptor

(2012) Yan Lu et al.   GASTROENTEROLOGY

Up-regulation of microRNA 506 leads to decreased Cl−/HCO3− anion exchanger 2 expression in biliary epithelium of patients with primary biliary cirrhosis

(2012) Jesús M. Banales et al.   HEPATOLOGY

Genetic variations in interleukin-12 related genes in immune-mediated diseases

(2012) R.L.J. van Wanrooij et al.   JOURNAL OF AUTOIMMUNITY

Activation of the Farnesoid X Receptor Induces Hepatic Expression and Secretion of Fibroblast Growth Factor 21

(2012) Holly A. Cyphert et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

CXCR3-dependent recruitment and CCR6-mediated positioning of Th-17 cells in the inflamed liver

(2012) Ye Htun Oo et al.   JOURNAL OF HEPATOLOGY

Bile acid transporters and regulatory nuclear receptors in the liver and beyond

(2012) Emina Halilbasic et al.   JOURNAL OF HEPATOLOGY

Classification of Inhibitors of Hepatic Organic Anion Transporting Polypeptides (OATPs): Influence of Protein Expression on Drug–Drug Interactions

(2012) Maria Karlgren et al.   JOURNAL OF MEDICINAL CHEMISTRY

Nuclear receptor PXR, transcriptional circuits and metabolic relevance

(2011) Chibueze A. Ihunnah et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Vitamin D inhibits proliferation and profibrotic marker expression in hepatic stellate cells and decreases thioacetamide-induced liver fibrosis in rats

(2011) S. Abramovitch et al.   GUT

Human leukocyte antigen in primary biliary cirrhosis: An old story now reviving

(2011) Pietro Invernizzi   HEPATOLOGY

Fine phenotypic and functional characterization of effector cluster of differentiation 8 positive T cells in human patients with primary biliary cirrhosis

(2011) Masanobu Tsuda et al.   HEPATOLOGY

The G-Protein-coupled bile acid receptor, Gpbar1 (TGR5), negatively regulates hepatic inflammatory response through antagonizing nuclear factor kappa light-chain enhancer of activated B cells (NF-κB) in mice

(2011) Yan-Dong Wang et al.   HEPATOLOGY

Dual farnesoid X receptor/TGR5 agonist INT-767 reduces liver injury in the Mdr2 −/− (Abcb4 −/− ) mouse cholangiopathy model by promoting biliary HCO 3− output

(2011) Anna Baghdasaryan et al.   HEPATOLOGY

Towards common denominators in primary biliary cirrhosis: The role of IL-12

(2011) Ana Lleo et al.   JOURNAL OF HEPATOLOGY

Genome-wide association study identifies 12 new susceptibility loci for primary biliary cirrhosis

(2011) George F Mells et al.   NATURE GENETICS

Th1 and Th17 cells

(2010) Jesse M. Damsker et al.   Annals of the New York Academy of Sciences

Murine models of autoimmune cholangitis

(2010) Yoshiyuki Ueno et al.   CURRENT OPINION IN GASTROENTEROLOGY

Pathogenesis of Cholestatic Liver Disease and Therapeutic Approaches

(2010) Gideon M. Hirschfield et al.   GASTROENTEROLOGY

Glycosylation of fibroblast growth factor receptor 4 is a key regulator of fibroblast growth factor 19-mediated down-regulation of cytochrome P450 7A1

(2010) Vassilis Triantis et al.   HEPATOLOGY

Biliary apotopes and anti-mitochondrial antibodies activate innate immune responses in primary biliary cirrhosis

(2010) Ana Lleo et al.   HEPATOLOGY

Gut-Residing Segmented Filamentous Bacteria Drive Autoimmune Arthritis via T Helper 17 Cells

(2010) Hsin-Jung Wu et al.   IMMUNITY

Regulation of Bile Acid Synthesis by Fat-soluble Vitamins A and D

(2010) Daniel R. Schmidt et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Primary biliary cirrhosis and autoimmune hepatitis: apotopes and epitopes

(2010) Ana Lleo et al.   JOURNAL OF GASTROENTEROLOGY

Modulation of the microenvironment by senescent biliary epithelial cells may be involved in the pathogenesis of primary biliary cirrhosis

(2010) Motoko Sasaki et al.   JOURNAL OF HEPATOLOGY

Demographic, lifestyle, medical and familial factors associated with primary biliary cirrhosis

(2010) Christophe Corpechot et al.   JOURNAL OF HEPATOLOGY

CD8 T Cells Mediate Direct Biliary Ductule Damage in Nonobese Diabetic Autoimmune Biliary Disease

(2010) G.-X. Yang et al.   JOURNAL OF IMMUNOLOGY

Long-term bacterial exposure can trigger nonsuppurative destructive cholangitis associated with multifocal epithelial inflammation

(2010) Ikuko Haruta et al.   LABORATORY INVESTIGATION

Genome-wide meta-analyses identify three loci associated with primary biliary cirrhosis

(2010) Xiangdong Liu et al.   NATURE GENETICS

Imbalance between T helper type 17 and T regulatory cells in patients with primary biliary cirrhosis: the serum cytokine profile and peripheral cell population

(2009) G. Rong et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

A Quantitative Enterohepatic Circulation Model

(2009) Thorsten Lehr et al.   CLINICAL PHARMACOKINETICS

Innate Immunity and Primary Biliary Cirrhosis

(2009) Carlo Selmi et al.   CURRENT MOLECULAR MEDICINE

CX3CL1 (fractalkine): A signpost for biliary inflammation in primary biliary cirrhosis

(2009) Shinji Shimoda et al.   HEPATOLOGY

Apotopes and the biliary specificity of primary biliary cirrhosis

(2009) Ana Lleo et al.   HEPATOLOGY

The membrane-bound bile acid receptor TGR5 is localized in the epithelium of human gallbladders

(2009) Verena Keitel et al.   HEPATOLOGY

Primary biliary cirrhosis is associated with altered hepatic microRNA expression

(2009) Kerstien A. Padgett et al.   JOURNAL OF AUTOIMMUNITY

Molecular physiology and genetics of Na+-independent SLC4 anion exchangers

(2009) S. L. Alper   JOURNAL OF EXPERIMENTAL BIOLOGY

EASL Clinical Practice Guidelines: Management of cholestatic liver diseases

(2009)   JOURNAL OF HEPATOLOGY

Bile acid transporters

(2009) Paul A. Dawson et al.   JOURNAL OF LIPID RESEARCH

Peroxisome proliferator-activated receptor delta activation leads to increased transintestinal cholesterol efflux

(2009) Carlos L. J. Vrins et al.   JOURNAL OF LIPID RESEARCH

Primary Biliary Cirrhosis Associated withHLA, IL12A,andIL12RB2Variants

(2009) Gideon M. Hirschfield et al.   NEW ENGLAND JOURNAL OF MEDICINE

Expression and function of the bile acid receptor TGR5 in Kupffer cells

(2008) Verena Keitel et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Liver Autoimmunity Triggered by Microbial Activation of Natural Killer T Cells

(2008) Jochen Mattner et al.   Cell Host & Microbe

Ae2a,b-Deficient Mice Develop Antimitochondrial Antibodies and Other Features Resembling Primary Biliary Cirrhosis

(2008) January T. Salas et al.   GASTROENTEROLOGY

Bile Salts Control the Antimicrobial Peptide Cathelicidin Through Nuclear Receptors in the Human Biliary Epithelium

(2008) Emilie D'Aldebert et al.   GASTROENTEROLOGY

Telomere shortening in the damaged small bile ducts in primary biliary cirrhosis reflects ongoing cellular senescence

(2008) Motoko Sasaki et al.   HEPATOLOGY

Human leukocyte antigen polymorphisms in italian primary biliary cirrhosis: A multicenter study of 664 patients and 1992 healthy controls

(2008) Pietro Invernizzi et al.   HEPATOLOGY

Loss of tolerance in C57BL/6 mice to the autoantigen E2 subunit of pyruvate dehydrogenase by a xenobiotic with ensuing biliary ductular disease

(2008) Kanji Wakabayashi et al.   HEPATOLOGY

CD4 T-cell autoreactivity to the mitochondrial autoantigen PDC-E2 in AMA-negative primary biliary cirrhosis

(2008) Shinji Shimoda et al.   JOURNAL OF AUTOIMMUNITY

Novel Potent and Selective Bile Acid Derivatives as TGR5 Agonists: Biological Screening, Structure−Activity Relationships, and Molecular Modeling Studies

(2008) Hiroyuki Sato et al.   JOURNAL OF MEDICINAL CHEMISTRY

Death receptor 5 mediated-apoptosis contributes to cholestatic liver disease

(2008) K. Takeda et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Hepatoprotective role of PXR activation and MRP3 in cholic acid-induced cholestasis

(2007) S Teng et al.   BRITISH JOURNAL OF PHARMACOLOGY