Novel pharmacologic therapies for cystic fibrosis

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

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

Cystic Fibrosis Airway Epithelia Fail to Kill Bacteria Because of Abnormal Airway Surface Fluid

1996 • 1,042 citations

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

1995 • 885 citations

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

1991 • 689 citations

CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP

1995 • 647 citations

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

1996 • 518 citations

Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins.

1994 • 482 citations

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

1996 • 482 citations

The Cystic Fibrosis Transmembrane Conductance Regulator

1993 • 438 citations

In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.

1997 • 386 citations

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

1994 • 359 citations

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

1998 • 335 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

Effects of the flavonoids quercetin and apigenin on hemostasis in healthy volunteers: results from an in vitro and a dietary supplement study

1998 • 273 citations

Effect of Deleting the R Domain on CFTR-Generated Chloride Channels

1991 • 271 citations

Gene redundancy and pharmacological gene therapy: Implications for X-linked adrenoleukodystrophy

1998 • 242 citations

Genistein potentiates wild-type and delta F508-CFTR channel activity

1997 • 205 citations

Correcting temperature-sensitive protein folding defects.

1997 • 180 citations

Regulation of Epithelial Sodium Channels by the Cystic Fibrosis Transmembrane Conductance Regulator

1996 • 165 citations

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

1995 • 144 citations

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

1996 • 139 citations

Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis.

1991 • 139 citations

Direct action of genistein on CFTR

1997 • 139 citations

The Molecular Chaperone Hsc70 Assists the in VitroFolding of the N-terminal Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator

1997 • 121 citations

Functional activation of the cystic fibrosis trafficking mutant delta F508-CFTR by overexpression

1995 • 120 citations

On the induction of fetal hemoglobin by butyrates: in vivo and in vitro studies with sodium butyrate and comparison of combination treatments with 5-AzaC and AraC

1989 • 118 citations

Flavonoids stimulate Cl conductance of human airway epithelium in vitro and in vivo

1998 • 113 citations

Triple-barrel Organization of ENaC, a Cloned Epithelial Na+ Channel

1996 • 100 citations

Partial restoration of cAMP-stimulated CFTR chloride channel activity in ΔF508 cells by deoxyspergualin

1998 • 86 citations

Direct Activation of Cystic Fibrosis Transmembrane Conductance Regulator Channels by 8-Cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-Diallyl-8-cyclohexylxanthine (DAX)

1998 • 75 citations

The cystic fibrosis transmembrane conductance regulator. Overexpression, purification, and characterization of wild type and delta F508 mutant forms of the first nucleotide binding fold in fusion with the maltose-binding protein.

1993 • 74 citations

8-Cyclopentyl-1,3-dipropylxanthine and Other Xanthines Differentially Bind to the Wild-Type and ΔF508 Mutant First Nucleotide Binding Fold (NBF-1) Domains of the Cystic Fibrosis Transmembrane Conductance Regulator

1997 • 69 citations

Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells

1995 • 57 citations

Butyrate derivatives. New agents for stimulating fetal globin production in the beta-globin disorders.

1994 • 56 citations

A1 Receptor Antagonist 8-Cyclopentyl-1,3-dipropylxanthine Selectively Activates Chloride Efflux from Human Epithelial and Mouse Fibroblast Cell Lines Expressing the Cystic Fibrosis Transmembrane Regulator .DELTA.F508 Mutation

1995 • 56 citations

Strategies for correcting the delta F508 CFTR protein-folding defect.

1997 • 55 citations

Extracellular ATP and UTP Induce Chloride Secretion in Nasal Epithelia of Cystic Fibrosis Patients and Normal Subjects in vivo

1992 • 53 citations

Phosphorylation-dependent Block of Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel by Exogenous R Domain Protein

1996 • 52 citations

Cystic fibrosis transmembrane conductance regulator activation by cAMP-independent mechanisms

1998 • 41 citations

Kinetic Interconversion of Rat and Bovine Homologs of the α Subunit of an Amiloride-sensitive Na+ Channel by C-terminal Truncation of the Bovine Subunit

1996 • 31 citations

II. Regulation of CFTR by small molecules including HCO 3 −

1998 • 30 citations

Similar levels of mRNA from the W1282X and the delta F508 cystic fibrosis alleles, in nasal epithelial cells.

1994 • 28 citations

The Final Stage of Gene Expression: Chaperones and the Regulation of Protein Fate

1997 • 21 citations

Effect of IBMX and alkaline phosphatase inhibitors on Cl⁻secretion in G551D cystic fibrosis mutant mice

1998 • 17 citations

Deleted Work

1955 • 0 citations