ΔF508 CFTR protein expression in tissues from patients with cystic fibrosis

The Metabolic and Molecular Bases of Inherited Disease

1995 • 12,086 citations

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

1989 • 7,327 citations

Therapy of CF-Patients with Amitriptyline and Placebo - a Randomised, Double-Blind, Placebo-Controlled Phase IIb Multicenter, Cohort-Study

2013 • 2,504 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

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

1995 • 885 citations

Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

1990 • 706 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

Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA

1993 • 515 citations

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

1991 • 498 citations

Efficiency of gene transfer for restoration of normal airway epithelial function in cystic fibrosis

1992 • 431 citations

Chemical chaperones correct the mutant phenotype of the ΔF508 cystic fibrosis transmembrane conductance regulator protein

1996 • 417 citations

Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP.

1994 • 397 citations

The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells.

1993 • 375 citations

Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator.

1994 • 359 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

Regulation of Plasma Membrane Recycling by CFTR

1992 • 339 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

A Pilot Clinical Trial of Oral Sodium 4-Phenylbutyrate (Buphenyl) in Δ F508-Homozygous Cystic Fibrosis Patients

1998 • 335 citations

Localization of the cystic fibrosis transmembrane conductance regulator in pancreas.

1991 • 296 citations

A mouse model for the delta F508 allele of cystic fibrosis.

1995 • 294 citations

Localization of cystic fibrosis transmembrane conductance regulator mRNA in the human gastrointestinal tract by in situ hybridization.

1994 • 266 citations

Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2.

1991 • 245 citations

Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia.

1992 • 240 citations

Mouse homologues of the human AZF candidate gene RBM are expressed in spermatogonia and spermatids, and map to a Y chromosome deletion interval associated with a high incidence of sperm abnormalities

1998 • 230 citations

Generation and characterization of a ΔF508 cystic fibrosis mouse model

1995 • 210 citations

Altered intestinal chloride transport in cystic fibrosis

1988 • 205 citations

Methods for the isolation of intact epithelium from the mouse intestine

1981 • 201 citations

Membrane Trafficking of the Cystic Fibrosis Gene Product, Cystic Fibrosis Transmembrane Conductance Regulator, Tagged with Green Fluorescent Protein in Madin-Darby Canine Kidney Cells

1998 • 157 citations

A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction.

1996 • 148 citations

Mutant (δF508) Cystic Fibrosis Transmembrane Conductance Regulator Cl− Channel Is Functional When Retained in Endoplasmic Reticulum of Mammalian Cells

1995 • 144 citations

Limited proteolysis as a probe for arrested conformational maturation of ΔF508 CFTR

1998 • 143 citations

A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo.

1996 • 140 citations

Delta F508-CFTR channels: kinetics, activation by forskolin, and potentiation by xanthines

1996 • 139 citations

An osmotic mechanism for exocytosis from dissociated chromaffin cells.

1984 • 137 citations

Rapid endocytosis of the cystic fibrosis transmembrane conductance regulator chloride channel.

1994 • 136 citations

Host Cell‐Specific Folding and Assembly of the Neuronal Nicotinic Acetylcholine Receptor α7 Subunit

1997 • 134 citations

Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype.

1994 • 134 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

Biochemical and biophysical identification of cystic fibrosis transmembrane conductance regulator chloride channels as components of endocytic clathrin-coated vesicles.

1994 • 116 citations

Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis.

1995 • 106 citations

CFTR and differentiation markers expression in non-CF and delta F 508 homozygous CF nasal epithelium.

1995 • 103 citations

Vasoactive intestinal peptide, forskolin, and genistein increase apical CFTR trafficking in the rectal gland of the spiny dogfish, Squalus acanthias. Acute regulation of CFTR trafficking in an intact epithelium.

1998 • 103 citations

Higher Proportion of Intact Exon 9 CFTR mRNA in Nasal Epithelium Compared with Vas Deferens

1997 • 101 citations

A unique subset of rat and human intestinal villus cells express the cystic fibrosis transmembrane conductance regulator

1995 • 100 citations

CFTR-dependent membrane insertion is linked to stimulation of the CFTR chloride conductance

1996 • 99 citations

A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis

1998 • 98 citations

In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant ΔF508 in murine nasal epithelium

1997 • 98 citations

Subcellular localization of CFTR to endosomes in a ductal epithelium

1994 • 87 citations

Distinct Functional Properties of Rab3A and Rab3B in PC12 Neuroendocrine Cells

1996 • 77 citations

Heterotrimeric G proteins, vesicle trafficking, and CFTR Cl- channels

1994 • 75 citations

CFTR: Development of high- affinity antibodies and localization in sweat gland

1991 • 72 citations

Mice Deficient in Lysosomal Acid Phosphatase Develop Lysosomal Storage in the Kidney and Central Nervous System

1997 • 69 citations

Maturation and Function of Cystic Fibrosis Transmembrane Conductance Regulator Variants Bearing Mutations in Putative Nucleotide-Binding Domains 1 and 2

1991 • 69 citations

Effect of anion transport inhibition on mucus secretion by airway submucosal glands

1997 • 68 citations

Decreased expression of the CFTR protein in remodeled human nasal epithelium from non-cystic fibrosis patients.

1995 • 63 citations

Isolation of transporting plasma membrane vesicles from bovine tracheal epithelium

1983 • 54 citations

Activation of ΔF508 CFTR in an epithelial monolayer

1998 • 54 citations

ATP-stimulated electrolyte and mucin secretion in the human intestinal goblet cell line HT29-C1.16E

1994 • 53 citations

Cellular differentiation regulates expression of Cl- transport and cystic fibrosis transmembrane conductance regulator mRNA in human intestinal cells

1991 • 51 citations

Tissue-specific in vivo transcription start sites of the human and murine cystic fibrosis genes

1998 • 44 citations

Constitutive Mucin Secretion Linked to CFTR Expression

1994 • 43 citations

Vector-Specific Complementation Profiles of Two Independent Primary Defects in Cystic Fibrosis Airways

1998 • 42 citations

Immunological localization of cystic fibrosis candidate gene products

1991 • 37 citations

A novel model for the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator

1997 • 35 citations

Cyclic AMP and Chloride-dependent Regulation of the Apical Constitutive Secretory Pathway in Colonic Epithelial Cells

1996 • 34 citations

An antibody against a cftr-derived synthetic peptide, incorporated into living submandibular cells, inhibits beta-adrenergic stimulation of mucin secretion

1992 • 33 citations

[47] Characterization of polyclonal and monoclonal antibodies to cystic fibrosis transmembrane conductance regulator

1998 • 27 citations

Coupled secretion of chloride and mucus in skin of Xenopus laevis: possible role for CFTR

1994 • 27 citations

Cellular localization of cystic fibrosis transmembrane regulator protein in piglet and mouse intestine

1996 • 25 citations

Targeting of CFTR protein is linked to the polarization of human pancreatic duct cells in culture

1998 • 23 citations

Defective ATP‐dependent mucin secretion by cystic fibrosis pancreatic epithelial cells

1996 • 22 citations