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

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

Living with Water Stress: Evolution of Osmolyte Systems

1982 • 3,735 citations

Update on Key Emerging Challenges in Cystic Fibrosis

2014 • 3,606 citations

Identification of the Cystic Fibrosis Gene: Chromosome Walking and Jumping

1989 • 3,180 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

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

1993 • 1,432 citations

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

1992 • 1,230 citations

Mechanism of protein stabilization by glycerol: preferential hydration in glycerol-water mixtures

1981 • 1,170 citations

Increased thermal stability of proteins in the presence of sugars and polyols

1979 • 746 citations

Liposome-mediated CFTR gene transfer to the nasal epithelium of patients with cystic fibrosis

1995 • 700 citations

Cellular localization and cell cycle regulation by a temperature-sensitive p53 protein.

1991 • 544 citations

Defective protein folding as a basis of human disease

1995 • 540 citations

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

1996 • 518 citations

Renal medullary organic osmolytes

1991 • 516 citations

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

1991 • 498 citations

Influence of molecular and chemical chaperones on protein folding

1996 • 496 citations

Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations

1996 • 482 citations

Thermodynamic and kinetic examination of protein stabilization by glycerol

1981 • 473 citations

Detailed Analysis of Structures and Formulations of Cationic Lipids for Efficient Gene Transfer to the Lung

1996 • 442 citations

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

1996 • 417 citations

Non–invasive liposome–mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice

1993 • 400 citations

Molecular basis of osmotic regulation

1995 • 371 citations

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

1994 • 359 citations

Protons, osmolytes, and fitness of internal milieu for protein function

1986 • 340 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

Chemical chaperones interfere with the formation of scrapie prion protein.

1996 • 297 citations

Accumulation of p53 in a mutant cell line defective in the ubiquitin pathway.

1994 • 274 citations

Repeat administration of an adenovirus vector encoding cystic fibrosis transmembrane conductance regulator to the nasal epithelium of patients with cystic fibrosis.

1996 • 263 citations

The cystic fibrosis mutation (ΔF508) does not influence the chloride channel activity of CFTR

1993 • 187 citations

Correcting temperature-sensitive protein folding defects.

1997 • 180 citations

Phosphatase inhibitors activate normal and defective CFTR chloride channels.

1994 • 153 citations

Alternate stimulation of apical CFTR by genistein in epithelia

1996 • 146 citations

Inhibition of heat shock (stress) protein induction by deuterium oxide and glycerol: Additional support for the abnormal protein hypothesis of induction

1989 • 143 citations

Increased Thermal Stability of Collagen in the Presence of Sugars and Polyols1

1983 • 142 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

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

1995 • 120 citations

OSMOTIC-REMEDIAL MUTANTS. A NEW CLASSIFICATION FOR NUTRITIONAL MUTANTS IN YEAST

1964 • 100 citations

Intracellular protein trafficking defects in human disease

1992 • 96 citations

A1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells.

1992 • 90 citations

Accumulation of p53 in a Mutant Cell Line Defective in the Ubiquitin Pathway

1994 • 87 citations

Folding intermediates are involved in genetic diseases?

1995 • 75 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

Activation of endogenous deltaF508 cystic fibrosis transmembrane conductance regulator by phosphodiesterase inhibition.

1996 • 55 citations

cAMP- and Ca2+-independent Activation of Cystic Fibrosis Transmembrane Conductance Regulator Channels by Phenylimidazothiazole Drugs

1996 • 52 citations

Stimulation by Alkylxanthines of Chloride Efflux in CFPAC-1 Cells Does Not Involve A1 Adenosine Receptors

1995 • 37 citations

Protection against heat-induced cell killing by polyols in vitro.

1983 • 27 citations

Protection of heat induced cytoxicity by glycerol

1981 • 21 citations