Polycystic liver diseases: advanced insights into the molecular mechanisms

HDAC6 Is Overexpressed in Cystic Cholangiocytes and Its Inhibition Reduces Cystogenesis

(2014) Sergio A. Gradilone et al.   AMERICAN JOURNAL OF PATHOLOGY

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

Polycystin-1: a master regulator of intersecting cystic pathways

(2014) Sorin V. Fedeles et al.   TRENDS IN MOLECULAR MEDICINE

Polycystic liver disease: ductal plate malformation and the primary cilium

(2014) Edgar S. Wills et al.   TRENDS IN MOLECULAR MEDICINE

Safety and efficacy of different lanreotide doses in the treatment of polycystic liver disease: pooled analysis of individual patient data

(2013) F. Temmerman et al.   ALIMENTARY PHARMACOLOGY & THERAPEUTICS

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

Young Women With Polycystic Liver Disease Respond Best to Somatostatin Analogues: A Pooled Analysis of Individual Patient Data

(2013) Tom J.G. Gevers et al.   GASTROENTEROLOGY

Everolimus does not further reduce polycystic liver volume when added to long acting octreotide: Results from a randomized controlled trial

(2013) Melissa Chrispijn et al.   JOURNAL OF HEPATOLOGY

Strategies Targeting cAMP Signaling in the Treatment of Polycystic Kidney Disease

(2013) V. E. Torres et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Loss of cilia suppresses cyst growth in genetic models of autosomal dominant polycystic kidney disease

(2013) Ming Ma et al.   NATURE GENETICS

Diagnosis and management of polycystic liver disease

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

Telmisartan Ameliorates Fibrocystic Liver Disease in an Orthologous Rat Model of Human Autosomal Recessive Polycystic Kidney Disease

(2013) Daisuke Yoshihara et al.   PLoS One

Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases

(2013) Bassam Abu-Wasel   WORLD JOURNAL OF GASTROENTEROLOGY

Somatic second-hit mutations leads to polycystic liver diseases

(2013) Jesús M Banales   WORLD JOURNAL OF GASTROENTEROLOGY

A Notch-dependent molecular circuitry initiates pancreatic endocrine and ductal cell differentiation

(2012) H. P. Shih et al.   DEVELOPMENT

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

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

Somatostatin analog therapy for severe polycystic liver disease: results after 2 years

(2012) M. C. Hogan et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

Loss of Heterozygosity Is Present in SEC63 Germline Carriers with Polycystic Liver Disease

(2012) Manoe J. Janssen et al.   PLoS One

Global Gene Expression Profiling in PPAR-γAgonist-Treated Kidneys in an Orthologous Rat Model of Human Autosomal Recessive Polycystic Kidney Disease

(2012) Daisuke Yoshihara et al.   PPAR Research

The long-term outcome of patients with polycystic liver disease treated with lanreotide

(2011) M. Chrispijn et al.   ALIMENTARY PHARMACOLOGY & THERAPEUTICS

Systematic review: the pathophysiology and management of polycystic liver disease

(2011) F. Temmerman et al.   ALIMENTARY PHARMACOLOGY & THERAPEUTICS

A Role for the Primary Cilium in Notch Signaling and Epidermal Differentiation during Skin Development

(2011) Ellen J. Ezratty et al.   CELL

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

Embryonic Ductal Plate Cells Give Rise to Cholangiocytes, Periportal Hepatocytes, and Adult Liver Progenitor Cells

(2011) Rodolphe Carpentier 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

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

Hepato-Renal Pathology in Pkd2 ws25/− Mice, an Animal Model of Autosomal Dominant Polycystic Kidney Disease

(2010) Angela Stroope et al.   AMERICAN JOURNAL OF PATHOLOGY

Polycystic kidney disease: Pathogenesis and potential therapies

(2010) Vinita Takiar et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Secondary and tertiary structure modeling reveals effects of novel mutations in polycystic liver disease genes PRKCSH and SEC63

(2010) E Waanders et al.   CLINICAL GENETICS

Reducing Polycystic Liver Volume in ADPKD: Effects of Somatostatin Analogue Octreotide

(2010) A. Caroli et al.   Clinical Journal of the American Society of Nephrology

Somatostatin analogues for treatment of polycystic liver disease

(2010) Tom JG Gevers et al.   CURRENT OPINION IN GASTROENTEROLOGY

Notch signalling regulates left-right asymmetry through ciliary length control

(2010) S. S. Lopes et al.   DEVELOPMENT

Polycystic liver diseases

(2010) P. Onori et al.   DIGESTIVE AND LIVER DISEASE

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

(2010) Sergio A. Gradilone et al.   GASTROENTEROLOGY

Randomized Clinical Trial of Long-Acting Somatostatin for Autosomal Dominant Polycystic Kidney and Liver Disease

(2010) M. C. Hogan et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Patients with autosomal dominant polycystic kidney disease have elevated fibroblast growth factor 23 levels and a renal leak of phosphate

(2010) Ivana Pavik et al.   KIDNEY INTERNATIONAL

Inhibition of mTOR with sirolimus does not attenuate progression of liver and kidney disease in PCK rats

(2010) C. Renken et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

Sirolimus and Kidney Growth in Autosomal Dominant Polycystic Kidney Disease

(2010) Andreas L. Serra et al.   NEW ENGLAND JOURNAL OF MEDICINE

Everolimus in Patients with Autosomal Dominant Polycystic Kidney Disease

(2010) Gerd Walz et al.   NEW ENGLAND JOURNAL OF MEDICINE

Molecular Basis of Autosomal Recessive Polycystic Kidney Disease (ARPKD)

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

Liver and kidney disease in ciliopathies

(2009) Meral Gunay-Aygun   AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS

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

Lanreotide Reduces the Volume of Polycystic Liver: A Randomized, Double-Blind, Placebo-Controlled Trial

(2009) Loes van Keimpema 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

Biliary differentiation and bile duct morphogenesis in development and disease

(2009) Peggy Raynaud et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

FGF signalling during embryo development regulates cilia length in diverse epithelia

(2009) Judith M. Neugebauer et al.   NATURE

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

Morphological and Functional Features of Hepatic Cyst Epithelium in Autosomal Dominant Polycystic Kidney Disease

(2008) Domenico Alvaro et al.   AMERICAN JOURNAL OF PATHOLOGY

CXCR2 agonists in ADPKD liver cyst fluids promote cell proliferation

(2008) Claudia R. Amura et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Cholangiocyte primary cilia in liver health and disease

(2008) Anatoliy I. Masyuk et al.   DEVELOPMENTAL DYNAMICS

FGF signaling segregates biliary cell-lineage from chick hepatoblasts cooperatively with BMP4 and ECM components in vitro

(2008) Masaaki Yanai et al.   DEVELOPMENTAL DYNAMICS

Structural and Functional Analyses of Liver Cysts from the BALB/c-cpk Mouse Model of Polycystic Kidney Disease

(2008) Monalisa N. Muchatuta et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

The microRNA-30 Family Is Required for Vertebrate Hepatobiliary Development

(2008) Nicholas J. Hand et al.   GASTROENTEROLOGY

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

Loss of polycystin-1 causes centrosome amplification and genomic instability

(2008) Lorenzo Battini et al.   HUMAN MOLECULAR GENETICS

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

Matrix proteins of basement membrane of intrahepatic bile ducts are degraded in congenital hepatic fibrosis and Caroli's disease

(2008) Mitsue Yasoshima et al.   JOURNAL OF PATHOLOGY

Sirolimus Reduces Polycystic Liver Volume in ADPKD Patients

(2008) Q. Qian et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Disrupted cell adhesion but not proliferation mediates cyst formation in polycystic liver disease

(2008) Esmé Waanders et al.   MODERN PATHOLOGY

Syntaxin 5 regulates the endoplasmic reticulum channel-release properties of polycystin-2

(2008) L. Geng et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

New insights on the molecular and cell biology of human cholangiopathies

(2007) Domenico Alvaro et al.   MOLECULAR ASPECTS OF MEDICINE