Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases

Cholangiocyte pathobiology

(2019) Jesus M. Banales et al.   Nature Reviews Gastroenterology & Hepatology

Pathophysiologic implications of innate immunity and autoinflammation in the biliary epithelium

(2018) Mario Strazzabosco et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Genetics and mechanisms of hepatic cystogenesis

(2018) L.F.M. van de Laarschot et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Proliferation-independent role of NF2 (merlin) in limiting biliary morphogenesis

(2018) Samira Benhamouche-Trouillet et al.   DEVELOPMENT

Mouse Model of Alagille Syndrome and Mechanisms of Jagged1 Missense Mutations

(2018) Emma R. Andersson et al.   GASTROENTEROLOGY

BILIARY ATRESIA: Clinical and Research Challenges for the 21st Century

(2018) Jorge A. Bezerra et al.   HEPATOLOGY

Src kinase inhibition reduces inflammatory and cytoskeletal changes in ΔF508 human cholangiocytes and improves cystic fibrosis transmembrane conductance regulator correctors efficacy

(2018) Romina Fiorotto et al.   HEPATOLOGY

Cholangiocyte autophagy contributes to hepatic cystogenesis in polycystic liver disease and represents a potential therapeutic target

(2018) Anatoliy I. Masyuk et al.   HEPATOLOGY

β-Catenin and interleukin-1β-dependent chemokine (C-X-C motif) ligand 10 production drives progression of disease in a mouse model of congenital hepatic fibrosis

(2018) Eleanna Kaffe et al.   HEPATOLOGY

OUP accepted manuscript

(2018)   HUMAN MOLECULAR GENETICS

Development of the liver: Insights into organ and tissue morphogenesis

(2018) Elke A. Ober et al.   JOURNAL OF HEPATOLOGY

Clinical management of polycystic liver disease

(2018) René M.M. van Aerts et al.   JOURNAL OF HEPATOLOGY

Macrophages contribute to the pathogenesis of sclerosing cholangitis in mice

(2018) Maria Eugenia Guicciardi et al.   JOURNAL OF HEPATOLOGY

Cenicriviroc, a cytokine receptor antagonist, potentiates all-trans retinoic acid in reducing liver injury in cholestatic rodents

(2018) Dongke Yu et al.   LIVER INTERNATIONAL

Genetics of Autosomal Recessive Polycystic Kidney Disease and Its Differential Diagnoses

(2018) Carsten Bergmann   Frontiers in Pediatrics

Alagille Syndrome

(2018) Ellen Mitchell et al.   Clinics in Liver Disease

De novo formation of the biliary system by TGFβ-mediated hepatocyte transdifferentiation

(2018) Johanna R. Schaub et al.   NATURE

Hippo Signaling in the Liver Regulates Organ Size, Cell Fate, and Carcinogenesis

(2017) Sachin H. Patel et al.   GASTROENTEROLOGY

Hepatic Progenitor Cells

(2017) Matthias Van Haele et al.   GASTROENTEROLOGY CLINICS OF NORTH AMERICA

Isolated polycystic liver disease genes define effectors of polycystin-1 function

(2017) Whitney Besse et al.   JOURNAL OF CLINICAL INVESTIGATION

Adenylyl cyclase 5 links changes in calcium homeostasis to cAMP-dependent cyst growth in polycystic liver disease

(2017) Carlo Spirli et al.   JOURNAL OF HEPATOLOGY

Targeting the gut-liver axis in liver disease

(2017) Reiner Wiest et al.   JOURNAL OF HEPATOLOGY

Cystic Fibrosis–related Liver Disease

(2017) Dominique Debray et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Directed differentiation of human induced pluripotent stem cells into functional cholangiocyte-like cells

(2017) Fotios Sampaziotis et al.   Nature Protocols

Genetics: Novel causative genes for polycystic liver disease

(2017) María J. Perugorria et al.   Nature Reviews Gastroenterology & Hepatology

Notch signaling and progenitor/ductular reaction in steatohepatitis

(2017) Carola M. Morell et al.   PLoS One

New treatments targeting the basic defects in cystic fibrosis

(2017) Isabelle Fajac et al.   PRESSE MEDICALE

Gene regulatory networks in differentiation and direct reprogramming of hepatic cells

(2017) Claude Gérard et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Mutations in GANAB , Encoding the Glucosidase IIα Subunit, Cause Autosomal-Dominant Polycystic Kidney and Liver Disease

(2016) Binu Porath et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Cystic fibrosis: a clinical view

(2016) Carlo Castellani et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

The cystic fibrosis transmembrane conductance regulator controls biliary epithelial inflammation and permeability by regulating Src tyrosine kinase activity

(2016) Romina Fiorotto et al.   HEPATOLOGY

Macrophage recruitment by fibrocystin-defective biliary epithelial cells promotes portal fibrosis in congenital hepatic fibrosis

(2016) Luigi Locatelli et al.   HEPATOLOGY

Ursodeoxycholic acid in advanced polycystic liver disease: A phase 2 multicenter randomized controlled trial

(2016) Hedwig M.A. D’Agnolo et al.   JOURNAL OF HEPATOLOGY

From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations

(2016) Gudio Veit et al.   MOLECULAR BIOLOGY OF THE CELL

The polycystin complex mediates Wnt/Ca2+ signalling

(2016) Seokho Kim et al.   NATURE CELL BIOLOGY

Cystic fibrosis from the gastroenterologist's perspective

(2016) Chee Y. Ooi et al.   Nature Reviews Gastroenterology & Hepatology

Notch3 drives development and progression of cholangiocarcinoma

(2016) Rachel V. Guest et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Medical therapy for polycystic liver disease

(2016) S Khan et al.   ANNALS OF THE ROYAL COLLEGE OF SURGEONS OF ENGLAND

Long-Term Culture of Genome-Stable Bipotent Stem Cells from Adult Human Liver

(2015) Meritxell Huch et al.   CELL

Posttranslational regulation of polycystin-2 protein expression as a novel mechanism of cholangiocyte reaction and repair from biliary damage

(2015) Carlo Spirli et al.   HEPATOLOGY

Stimulation of nuclear receptor peroxisome proliferator-activated receptor-γ limits NF-κB-dependent inflammation in mouse cystic fibrosis biliary epithelium

(2015) Roberto Scirpo et al.   HEPATOLOGY

Jagged1 heterozygosity in mice results in a congenital cholangiopathy which is reversed by concomitant deletion of one copy ofPoglut1(Rumi)

(2015) Shakeel M. Thakurdas et al.   HEPATOLOGY

Outcome in patients with cystic fibrosis liver disease

(2015) Marion Rowland et al.   Journal of Cystic Fibrosis

Surgical Management of Caroli’s Disease: Single Center Experience and Review of the Literature

(2015) Maitham A. Moslim et al.   JOURNAL OF GASTROINTESTINAL SURGERY

Ursodeoxycholic acid inhibits hepatic cystogenesis in experimental models of polycystic liver disease

(2015) Patricia Munoz-Garrido et al.   JOURNAL OF HEPATOLOGY

The Cholangiopathies

(2015) Konstantinos N. Lazaridis et al.   MAYO CLINIC PROCEEDINGS

Vasopressin and disruption of calcium signalling in polycystic kidney disease

(2015) Fouad T. Chebib et al.   Nature Reviews Nephrology

Proliferation-Independent Initiation of Biliary Cysts in Polycystic Liver Diseases

(2015) Jean-Bernard Beaudry et al.   PLoS One

Intestinal Lesions Are Associated with Altered Intestinal Microbiome and Are More Frequent in Children and Young Adults with Cystic Fibrosis and Cirrhosis

(2015) Thomas Flass et al.   PLoS One

Intrahepatic Bile Duct Regeneration in Mice Does Not Require Hnf6 or Notch Signaling through Rbpj

(2014) Teagan J. Walter et al.   AMERICAN JOURNAL OF PATHOLOGY

Hippo Pathway Activity Influences Liver Cell Fate

(2014) Dean Yimlamai et al.   CELL

Tyrosine phosphorylation in Toll-like receptor signaling

(2014) Saurabh Chattopadhyay et al.   CYTOKINE & GROWTH FACTOR REVIEWS

Interactions Between the Intestinal Microbiome and Liver Diseases

(2014) Bernd Schnabl et al.   GASTROENTEROLOGY

Inhibition of metalloprotease hyperactivity in cystic cholangiocytes halts the development of polycystic liver diseases

(2014) Aura D Urribarri et al.   GUT

Emerging roles of Notch signaling in liver disease

(2014) Fabian Geisler et al.   HEPATOLOGY

Cystic Fibrosis Related Liver Disease—Another Black Box in Hepatology

(2014) Katharina Staufer et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Therapeutic modulation of Notch signalling — are we there yet?

(2014) Emma R. Andersson et al.   NATURE REVIEWS DRUG DISCOVERY

Polycystic liver diseases: advanced insights into the molecular mechanisms

(2014) Maria J. Perugorria et al.   Nature Reviews Gastroenterology & Hepatology

Whole-exome sequencing reveals LRP5 mutations and canonical Wnt signaling associated with hepatic cystogenesis

(2014) W. R. Cnossen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ABCB4: Insights from pathobiology into therapy

(2014) Thomas Falguières et al.   Clinics and Research in Hepatology and Gastroenterology

Liver involvement in children with ciliopathies

(2014) N. Rock et al.   Clinics and Research in Hepatology and Gastroenterology

Centrosomal Abnormalities Characterize Human and Rodent Cystic Cholangiocytes and Are Associated with Cdc25A Overexpression

(2013) Tatyana V. Masyuk et al.   AMERICAN JOURNAL OF PATHOLOGY

Ciliary subcellular localization of TGR5 determines the cholangiocyte functional response to bile acid signaling

(2013) Anatoliy I. Masyuk et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Cross-talk between Notch and Hedgehog regulates hepatic stellate cell fate in mice

(2013) Guanhua Xie et al.   HEPATOLOGY

Protein kinase a-dependent pSer675 -β-catenin, a novel signaling defect in a mouse model of congenital hepatic fibrosis

(2013) Carlo Spirli et al.   HEPATOLOGY

Increased T helper type 17 response to pathogen stimulation in patients with primary sclerosing cholangitis

(2013) Janosch Katt et al.   HEPATOLOGY

Notch signaling regulates tubular morphogenesis during repair from biliary damage in mice

(2013) Romina Fiorotto et al.   JOURNAL OF HEPATOLOGY

Notch signaling and new therapeutic options in liver disease

(2013) Carola Maria Morell et al.   JOURNAL OF HEPATOLOGY

Primary sclerosing cholangitis

(2013) Gideon M Hirschfield et al.   LANCET

Diagnosis and management of polycystic liver disease

(2013) Tom J. G. Gevers et al.   Nature Reviews Gastroenterology & Hepatology

Bile duct cyst in adults: Interventional treatment, resection, or transplantation?

(2013) Herwig Cerwenka   WORLD JOURNAL OF GASTROENTEROLOGY

Notch signalling beyond liver development: Emerging concepts in liver repair and oncogenesis

(2013) Carola Maria Morell et al.   Clinics and Research in Hepatology and Gastroenterology

The Cystic Fibrosis Intestine

(2013) R. C. De Lisle et al.   Cold Spring Harbor Perspectives in Medicine

Notch Signaling Is Activated in Human Hepatocellular Carcinoma and Induces Tumor Formation in Mice

(2012) Augusto Villanueva et al.   GASTROENTEROLOGY

Notch Signaling in Hepatocellular Carcinoma: Guilty in Association!

(2012) Mario Strazzabosco et al.   GASTROENTEROLOGY

Mucin-producing cholangiocarcinoma might derive from biliary tree stem/progenitor cells located in peribiliary glands

(2012) Vincenzo Cardinale et al.   HEPATOLOGY

Cyclic AMP/PKA-dependent paradoxical activation of Raf/MEK/ERK signaling in polycystin-2 defective mice treated with sorafenib

(2012) Carlo Spirli et al.   HEPATOLOGY

Genome-wide association analysis in Primary sclerosing cholangitis and ulcerative colitis identifies risk loci atGPR35andTCF4

(2012) David Ellinghaus et al.   HEPATOLOGY

Constitutive Notch2 signaling induces hepatic tumors in mice

(2012) Michael T. Dill et al.   HEPATOLOGY

Pasireotide is more effective than octreotide in reducing hepatorenal cystogenesis in rodents with polycystic kidney and liver diseases

(2012) Tatyana V. Masyuk et al.   HEPATOLOGY

Cholangiocarcinomas can originate from hepatocytes in mice

(2012) Biao Fan et al.   JOURNAL OF CLINICAL INVESTIGATION

Intrahepatic cholangiocarcinoma can arise from Notch-mediated conversion of hepatocytes

(2012) Sayaka Sekiya et al.   JOURNAL OF CLINICAL INVESTIGATION

Extended analysis of a genome-wide association study in primary sclerosing cholangitis detects multiple novel risk loci

(2012) Trine Folseraas et al.   JOURNAL OF HEPATOLOGY

Development of the bile ducts: Essentials for the clinical hepatologist

(2012) Mario Strazzabosco et al.   JOURNAL OF HEPATOLOGY

The balance between Notch/Wnt signaling regulates progenitor cells’ commitment during liver repair: Mystery solved?

(2012) Mario Strazzabosco et al.   JOURNAL OF HEPATOLOGY

Outcomes of liver transplantation for patients with alagille syndrome: The studies of pediatric liver transplantation experience

(2012) Binita M. Kamath et al.   LIVER TRANSPLANTATION

Macrophage-derived Wnt opposes Notch signaling to specify hepatic progenitor cell fate in chronic liver disease

(2012) Luke Boulter et al.   NATURE MEDICINE

The complex world of WNT receptor signalling

(2012) Christof Niehrs   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Secondary, Somatic Mutations Might Promote Cyst Formation in Patients With Autosomal Dominant Polycystic Liver Disease

(2011) Manoe J. Janssen et al.   GASTROENTEROLOGY

Polycystic Liver Diseases: Congenital Disorders of Cholangiocyte Signaling

(2011) Mario Strazzabosco et al.   GASTROENTEROLOGY

Loss of CFTR Affects Biliary Epithelium Innate Immunity and Causes TLR4–NF-κB—Mediated Inflammatory Response in Mice

(2011) Romina Fiorotto et al.   GASTROENTEROLOGY

Inhibition of Cdc25A Suppresses Hepato-renal Cystogenesis in Rodent Models of Polycystic Kidney and Liver Disease

(2011) Tatyana V. Masyuk et al.   GASTROENTEROLOGY

A classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis

(2011) Peggy Raynaud et al.   HEPATOLOGY

Altered store operated calcium entry increases cyclic 3′,5′-adenosine monophosphate production and extracellular signal-regulated kinases 1 and 2 phosphorylation in polycystin-2-defective cholangiocytes

(2011) Carlo Spirli et al.   HEPATOLOGY

Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease

(2011) Dominique Debray et al.   Journal of Cystic Fibrosis

Liver disease in adult patients with cystic fibrosis: A frequent and independent prognostic factor associated with death or lung transplantation

(2011) Ariane Chryssostalis et al.   JOURNAL OF HEPATOLOGY

Enhanced innate immune responsiveness and intolerance to intestinal endotoxins in human biliary epithelial cells contributes to chronic cholangitis

(2011) Tobias Mueller et al.   LIVER INTERNATIONAL

A genetic interaction network of five genes for human polycystic kidney and liver diseases defines polycystin-1 as the central determinant of cyst formation

(2011) Sorin V Fedeles et al.   NATURE GENETICS

Ciliopathies

(2011) Friedhelm Hildebrandt et al.   NEW ENGLAND JOURNAL OF MEDICINE

Biliary exosomes influence cholangiocyte regulatory mechanisms and proliferation through interaction with primary cilia

(2010) Anatoliy I. Masyuk et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Jagged1 in the portal vein mesenchyme regulates intrahepatic bile duct development: insights into Alagille syndrome

(2010) J. J. Hofmann et al.   DEVELOPMENT

The Merlin/NF2 Tumor Suppressor Functions through the YAP Oncoprotein to Regulate Tissue Homeostasis in Mammals

(2010) Nailing Zhang et al.   DEVELOPMENTAL CELL

Organogenesis and Development of the Liver

(2010) Karim Si-Tayeb et al.   DEVELOPMENTAL CELL

Activation of Trpv4 Reduces the Hyperproliferative Phenotype of Cystic Cholangiocytes From an Animal Model of ARPKD

(2010) Sergio A. Gradilone et al.   GASTROENTEROLOGY

CFTR chloride channel in the apical compartments: spatiotemporal coupling to its interacting partners

(2010) Chunying Li et al.   Integrative Biology

Organogenesis relies on SoxC transcription factors for the survival of neural and mesenchymal progenitors

(2010) Pallavi Bhattaram et al.   Nature Communications

Molecular Basis of Autosomal Recessive Polycystic Kidney Disease (ARPKD)

(2009) Lulu Al-Bhalal et al.   ADVANCES IN ANATOMIC PATHOLOGY

The Notch signaling pathway: Transcriptional regulation at Notch target genes

(2009) T. Borggrefe et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Cholangiociliopathies: genetics, molecular mechanisms and potential therapies

(2009) Tatyana Masyuk et al.   CURRENT OPINION IN GASTROENTEROLOGY

Notch signaling controls liver development by regulating biliary differentiation

(2009) Y. Zong et al.   DEVELOPMENT

Polycystins play a key role in the modulation of cholangiocyte proliferation

(2009) Alessia Torrice et al.   DIGESTIVE AND LIVER DISEASE

Liver Cyst Cytokines Promote Endothelial Cell Proliferation and Development

(2009) Kelley S. Brodsky et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

Mechanisms of Liver Development: Concepts for Understanding Liver Disorders and Design of Novel Therapies

(2009) Frédéric P. Lemaigre   GASTROENTEROLOGY

Stem Cells and Liver Regeneration

(2009) Andrew W. Duncan et al.   GASTROENTEROLOGY

ERK1/2-Dependent Vascular Endothelial Growth Factor Signaling Sustains Cyst Growth in Polycystin-2 Defective Mice

(2009) Carlo Spirli et al.   GASTROENTEROLOGY

Mammalian target of rapamycin regulates vascular endothelial growth factor-dependent liver cyst growth in polycystin-2-defective mice

(2009) Carlo Spirli et al.   HEPATOLOGY

Cystic fibrosis

(2009) Brian P O'Sullivan et al.   LANCET

STIM protein coupling in the activation of Orai channels

(2009) Y. Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Pediatric liver transplantation

(2009) Marco Spada et al.   WORLD JOURNAL OF GASTROENTEROLOGY

Hepatic Cystogenesis Is Associated with Abnormal Expression and Location of Ion Transporters and Water Channels in an Animal Model of Autosomal Recessive Polycystic Kidney Disease

(2008) Jesús M. Banales et al.   AMERICAN JOURNAL OF PATHOLOGY

Cholangiocyte primary cilia are chemosensory organelles that detect biliary nucleotides via P2Y12 purinergic receptors

(2008) Anatoliy I. Masyuk et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Functional Anatomy of Normal Bile Ducts

(2008) Mario Strazzabosco et al.   Anatomical Record-Advances in Integrative Anatomy and Evolutionary Biology

Polycystic Kidney Disease

(2008) Peter C. Harris et al.   Annual Review of Medicine

The cAMP effectors Epac and protein kinase a (PKA) are involved in the hepatic cystogenesis of an animal model of autosomal recessive polycystic kidney disease (ARPKD)

(2008) Jesús M. Banales et al.   HEPATOLOGY

Bile duct proliferation in Jag1/fringe heterozygous mice identifies candidate modifiers of the alagille syndrome hepatic phenotype

(2008) Matthew J. Ryan et al.   HEPATOLOGY

MicroRNA15a modulates expression of the cell-cycle regulator Cdc25A and affects hepatic cystogenesis in a rat model of polycystic kidney disease

(2008) Seung-Ok Lee et al.   JOURNAL OF CLINICAL INVESTIGATION

Fibropolycystic liver disease in children

(2008) Myka Call Veigel et al.   PEDIATRIC RADIOLOGY