Expression of cystic fibrosis transmembrane conductance regulator in liver tissue from patients with cystic fibrosis

Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA

1989 • 7,327 citations

Identification of the Cystic Fibrosis Gene: Genetic Analysis

1989 • 4,105 citations

Identification of the Cystic Fibrosis Gene: Chromosome Walking and Jumping

1989 • 3,180 citations

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

1990 • 1,756 citations

Degradation of CFTR by the ubiquitin-proteasome pathway

1995 • 1,285 citations

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

1992 • 1,230 citations

Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion Selectivity

1991 • 1,108 citations

Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)

1992 • 937 citations

Multiple proteolytic systems, including the proteasome, contribute to CFTR processing

1995 • 885 citations

The Relation between Genotype and Phenotype in Cystic Fibrosis — Analysis of the Most Common Mutation (ΔF₅₀₈)

1990 • 724 citations

Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation

1991 • 689 citations

Submucosal glands are the predominant site of CFTR expression in the human bronchus

1992 • 644 citations

Glycerol Reverses the Misfolding Phenotype of the Most Common Cystic Fibrosis Mutation

1996 • 518 citations

Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes

1991 • 498 citations

Genetic determination of exocrine pancreatic function in cystic fibrosis.

1992 • 437 citations

One-Second Needle Biopsy of the Liver

1958 • 386 citations

Mislocalization of ΔF508 CFTR in cystic fibrosis sweat gland

1992 • 354 citations

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

1993 • 347 citations

The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment.

1993 • 335 citations

Natural history of liver disease in cystic fibrosis

1999 • 306 citations

ΔF508 CFTR protein expression in tissues from patients with cystic fibrosis

1999 • 265 citations

Molecular regulation of hepatic fibrogenesis

1998 • 249 citations

Phosphatidylinositol 3-kinase is required for platelet-derived growth factor's actions on hepatic stellate cells

1997 • 202 citations

Analysis of risk factors for the development of liver disease associated with cystic fibrosis

1994 • 194 citations

A two-year prospective study of the effect of ursodeoxycholic acid on urinary bile acid excretion and liver morphology in cystic fibrosis-associated liver disease

1998 • 171 citations

Hepatic changes in young infants with cystic fibrosis: Possible relation to focal biliary cirrhosis

1975 • 153 citations

A distinctive type of biliary cirrhosis of the liver associated with cystic fibrosis of the pancreas; recognition through signs of portal hypertension.

1956 • 151 citations

A DISTINCTIVE TYPE OF BILIARY CIRRHOSIS OF THE LIVER ASSOCIATED WITH CYSTIC FIBROSIS OF THE PANCREAS

1956 • 137 citations

Cotranslational Ubiquitination of Cystic Fibrosis Transmembrane Conductance Regulator in Vitro

1998 • 132 citations

Differential Localization of the Cystic Fibrosis Transmembrane Conductance Regulator in Normal and Cystic Fibrosis Airway Epithelium

1992 • 128 citations

Expression of platelet-derived growth factor in newly formed cholangiocytes during experimental biliary fibrosis in rats

1999 • 128 citations

Focal adhesion kinase and phospholipase C gamma involvement in adhesion and migration of human hepatic stellate cells

1997 • 125 citations

Liver and Biliary Problems in Cystic Fibrosis

1998 • 118 citations

Increased expression of intercellular adhesion molecule 1 on bile ducts in primary biliary cirrhosis and primary sclerosing cholangitis

1991 • 118 citations

The Molecular Basis for Disease Variability in Cystic Fibrosis

1996 • 117 citations

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

1993 • 116 citations

Bile-duct destruction and collagen deposition: A prominent ultrastructural feature of the liver in cystic fibrosis

1992 • 108 citations

The Pathological Changes in the Liver in Cystic Fibrosis of the Pancreas

1957 • 86 citations

Genotype analysis for ΔF508, G551D and R553X mutations in children and young adults with cystic fibrosis with and without chronic liver disease

1992 • 85 citations

Clinical denouement and mutation analysis of patients with cystic fibrosis undergoing liver transplantation for biliary cirrhosis

1995 • 77 citations

Alkaline phosphatase-fast red, a new fluorescent label

1990 • 70 citations

Morphological findings in the liver of children with cystic fibrosis: A light and electron microscopical study

1986 • 67 citations

Meconium Ileus and Its Equivalent as a Risk Factor for the Development of Cirrhosis

1989 • 53 citations

Expression of delta F508 cystic fibrosis transmembrane conductance regulator protein and related chloride transport properties in the gallbladder epithelium from cystic fibrosis patients

1999 • 47 citations

Expression of cystic fibrosis transmembrane conductance regulator in human gallbladder epithelial cells.

1995 • 43 citations

Genotype-phenotype correlation in cystic fibrosis patients.

1996 • 35 citations

Meconium Ileus and Its Equivalent as a Risk Factor for the Development of Cirrhosis

1989 • 33 citations

Immunocytochemical analysis reveals differences between the subcellular localization of normal and ΔPhe508 recombinant cystic fibrosis transmembrane conductance regulator

1992 • 28 citations