Pathophysiology of Cholangiopathies

PKD2 , a Gene for Polycystic Kidney Disease That Encodes an Integral Membrane Protein

1996 • 1,360 citations

Alagille syndrome is caused by mutations in human Jagged1, which encodes a ligand for Notch1

1997 • 1,239 citations

The polycystic kidney disease 1 gene encodes a 14 kb transcript and lies within a duplicated region on chromosome 16

1994 • 805 citations

Liver regeneration and repair: Hepatocytes, progenitor cells, and stem cells

2004 • 721 citations

The canals of hering and hepatic stem cells in humans

1999 • 701 citations

Nomenclature of the finer branches of the biliary tree: Canals, ductules, and ductular reactions in human livers

2004 • 684 citations

Molecular Pathogenesis of Cholestasis

1998 • 669 citations

Molecular Pathogenesis of Cholestasis

2004 • 622 citations

The wide spectrum of multidrug resistance 3 deficiency: From neonatal cholestasis to cirrhosis of adulthood

2001 • 496 citations

Cellular commitment to oncogene-induced transformation or apoptosis is dependent on the transcription factor IRF-1

1994 • 490 citations

Regurgitation of bile acids from leaky bile ducts causes sclerosing cholangitis in Mdr2 (Abcb4) knockout mice

2004 • 445 citations

Aberrant CFTR-dependent HCO-3 transport in mutations associated with cystic fibrosis

2001 • 406 citations

The cholangiopathies: Disorders of biliary epithelia

2004 • 350 citations

Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells

1993 • 347 citations

PRIMARY BILIARY CIRRHOSIS: IDENTIFICATION OF TWO MAJOR M2 MITOCHONDRIAL AUTOANTIGENS

1988 • 298 citations

Nitric oxide in gastrointestinal epithelial cell carcinogenesis: linking inflammation to oncogenesis

2001 • 280 citations

HLA association of amoxicillin-clavulanate–induced hepatitis

1999 • 275 citations

Cholangiocarcinoma: Current concepts and insights

2003 • 270 citations

The Pathophysiology of Biliary Epithelia

2020 • 265 citations

Mitosis and apoptosis in the liver of interleukin-6-deficient mice after partial hepatectomy

1999 • 264 citations

Rat cholangiocytes absorb bile acids at their apical domain via the ileal sodium-dependent bile acid transporter.

1997 • 250 citations

Pathogenesis of Ductal Plate Abnormalities

1998 • 242 citations

Nitric oxide–mediated inhibition of DNA repair potentiates oxidative DNA damage in cholangiocytes

2001 • 218 citations

Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities.

1998 • 209 citations

High expression of MDR1, MRP1, and MRP3 in the hepatic progenitor cell compartment and hepatocytes in severe human liver disease

2003 • 192 citations

Hepatic progenitor cells in human liver diseases

2002 • 191 citations

Human Ogg1, a protein involved in the repair of 8-oxoguanine, is inhibited by nitric oxide.

2001 • 176 citations

Estrogens stimulate proliferation of intrahepatic biliary epithelium in rats

2000 • 176 citations

Heterogeneity of the proliferative capacity of rat cholangiocytes after bile duct ligation

1998 • 174 citations

Functional expression of the apical Na+-dependent bile acid transporter in large but not small rat cholangiocytes

1997 • 165 citations

The immunohistochemical phenotype of dysplastic foci in human liver: correlation with putative progenitor cells

2000 • 161 citations

Differential Modulation of the Human Liver Conjugate Transporters MRP2 and MRP3 by Bile Acids and Organic Anions

2003 • 152 citations

Beauty is in the eye of the beholder: emerging concepts and pitfalls in hepatic stellate cell research

2002 • 151 citations

The pathobiology of biliary epithelia

2002 • 148 citations

Functional heterogeneity of the intrahepatic biliary epithelium

2000 • 145 citations

Bile ductular cholestasis: An ominous histopathologic sign related to sepsis and “cholangitis lenta”

1982 • 142 citations

Human intrahepatic biliary epithelial cells proliferate in vitro in response to human hepatocyte growth factor.

1992 • 141 citations

Secretin induces the apical insertion of aquaporin-1 water channels in rat cholangiocytes

1999 • 139 citations

Alternative splicing of the rat sodium/bile acid transporter changes its cellular localization and transport properties

2000 • 137 citations

Glutathione depletion is associated with decreased Bcl-2 expression and increased apoptosis in cholangiocytes

1998 • 133 citations

Extrahepatic biliary atresia: A disease or a phenotype?

2002 • 133 citations

Anomalous development of the hepatobiliary system in theinv mouse

1999 • 132 citations

Cytokine-stimulated nitric oxide production inhibits adenylyl cyclase and cAMP-dependent secretion in cholangiocytes

2003 • 130 citations

Role and mechanisms of action of acetylcholine in the regulation of rat cholangiocyte secretory functions.

1997 • 127 citations

Growth control of human biliary epithelial cells by interleukin 6, hepatocyte growth factor, transforming growth factor β1, and activin a: Comparison of a cholangiocarcinoma cell line with primary cultures of non-neoplastic biliary epithelial cells

2000 • 127 citations

Bile acids stimulate proliferative and secretory events in large but not small cholangiocytes

1997 • 125 citations

Proinflammatory Cytokines Inhibit Secretion in Rat Bile Duct Epithelium

2001 • 123 citations

Cholangiocyte Biology and Cystic Fibrosis Liver Disease

2001 • 123 citations

Intercellular adhesion molecule-1 and MHC antigens on human intrahepatic bile duct cells: effect of pro-inflammatory cytokines.

1993 • 116 citations

Intracellular pathways mediating estrogen-induced cholangiocyte proliferation in the rat

2002 • 112 citations

Regulation and deregulation of cholangiocyte proliferation

2000 • 107 citations

Membrane dihydrolipoamide acetyltransferase (E2) on human biliary epithelial cells in primary biliary cirrhosis

1992 • 103 citations

Defective regulation of cholangiocyte Cl⁻ /HCO⁻ ₃ and Na⁺ /H⁺ exchanger activities in primary biliary cirrhosis

2002 • 102 citations

Interleukin-6, hepatocyte growth factor, and their receptors in biliary epithelial cells during a type i ductular reaction in mice: Interactions between the periductal inflammatory and stromal cells and the biliary epithelium

1998 • 102 citations

Ezrin-radixin-moesin–binding phosphoprotein 50 is expressed at the apical membrane of rat liver epithelia

2001 • 101 citations

Drug-induced cholestasis

1997 • 100 citations

Genetic predisposition to drug-induced hepatotoxicity

1997 • 97 citations

Hepatic microcirculation and peribiliary plexus in experimental biliary cirrhosis: A morphological study

1996 • 97 citations

Stromal Cell–Derived Factor-1 from Biliary Epithelial Cells Recruits CXCR4-Positive Cells: Implications for Inflammatory Liver Diseases

2003 • 94 citations

Caspase induction by IgA antimitochondrial antibody: IgA-mediated biliary injury in primary biliary cirrhosis

2004 • 93 citations

Identification, genomic organization, chromosomal mapping and mutation analysis of the human INV gene, the ortholog of a murine gene implicated in left-right axis development and biliary atresia

2001 • 92 citations

Progenitor cell involvement in cirrhotic human liver diseases: from controversy to consensus

2003 • 92 citations

Analysis of microsatellite instability, K-ras gene mutation and p53 protein overexpression in intrahepatic cholangiocarcinoma.

2002 • 90 citations

Pathophysiology of the intrahepatic biliary epithelium

2000 • 88 citations

Bile acid depletion and repletion regulate cholangiocyte growth and secretion by a phosphatidylinositol 3-kinase–dependent pathway in rats

2002 • 83 citations

Coordinate expression of regulatory genes differentiates embryonic and perinatal forms of biliary atresia

2004 • 82 citations

Regulation of Ca²⁺signaling in rat bile duct epithelia by inositol 1,4,5-trisphosphate receptor isoforms

2002 • 80 citations

Somatostatin stimulates ductal bile absorption and inhibits ductal bile secretion in mice via SSTR2 on cholangiocytes

2003 • 74 citations

Expression of the α7 Isoform of Hepatocyte Nuclear Factor (HNF) 4 Is Activated by HNF6/OC-2 and HNF1 and Repressed by HNF4α1 in the Liver

2004 • 73 citations

Cholangiocarcinomas express fas ligand and disable the fas receptor

1999 • 69 citations

Vasoactive intestinal polypeptide is a potent regulator of bile secretion from rat cholangiocytes

1999 • 67 citations

Ca²⁺‐activated Cl⁻ channels can substitute for CFTR in stimulation of pancreatic duct bicarbonate secretion

2000 • 63 citations

Role of sodium/hydrogen exchanger isoform NHE3 in fluid secretion and absorption in mouse and rat cholangiocytes

2001 • 61 citations

Expression of aquaporin-4 water channels in rat cholangiocytes

2000 • 60 citations

Hepatic artery malformations associated with a primary defect in intrahepatic bile duct development

2003 • 58 citations

Correction of CFTR malfunction and stimulation of Ca²⁺ -activated Cl⁻ channels restore HCO 3− secretion in cystic fibrosis bile ductular cells

2002 • 56 citations

Tauroursodeoxycholate inhibits human cholangiocarcinoma growth via Ca²⁺-, PKC-, and MAPK-dependent pathways

2004 • 55 citations

New insights into cholangiocyte physiology

1997 • 53 citations

Insulin inhibits secretin-induced ductal secretion by activation of PKC alpha and inhibition of PKA activity

2002 • 51 citations

Purinergic regulation of acid/base transport in human and rat biliary epithelial cell lines

1998 • 51 citations

Molecular identification and functional characterization of Mdr1a in rat cholangiocytes

2000 • 49 citations

Signal transduction by nitric oxide in cellular stress responses.

2002 • 46 citations

Cystic Disease in Women: Clinical Characteristics and Medical Management

2003 • 44 citations

Characterization of recombinant monoclonal IgA anti–PDC-E2 autoantibodies derived from patients with PBC

2002 • 43 citations

Capillary Endothelial Thrombomodulin Expression and Fibrin Deposition in Rats with Continuous and Bolus Lipopolysaccharide Administration

2003 • 41 citations

Tissue‐based stem cells: ABC transporter proteins take centre stage

2003 • 37 citations

Gastrin reverses established cholangiocyte proliferation and enhanced secretin-stimulated ductal secretion of BDL rats by activation of apoptosis through increased expression of Ca²⁺-dependent PKC isoforms

2003 • 30 citations

Characterization of ion transport mechanisms involved in bombesin-stimulated biliary secretion in rat cholangiocytes

1999 • 29 citations

Biliary tract malformations

2003 • 28 citations

Stimulation of bile duct epithelial secretion by glybenclamide in normal and cholestatic rat liver.

1998 • 27 citations

Bile acid-independent bile flow is differently regulated by glucagon and secretin in humans after orthotopic liver transplantation

1997 • 25 citations

Antibodies and primary biliary cirrhosis – piecing together the jigsaw

2002 • 16 citations

Chronic Cholestasis Associated with Turner’s Syndrome: 12 Years of Clinical and Histopathological Follow-Up

2003 • 12 citations

Ursodeoxycholate further increases bile-duct cell proliferative response induced by partial bile-duct ligation in rats

2004 • 10 citations