Cystic ibrosis: Molecular Biology and Therapeutic Implications

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

A TEST FOR CONCENTRATION OF ELECTROLYTES IN SWEAT IN CYSTIC FIBROSIS OF THE PANCREAS UTILIZING PILOCARPINE BY IONTOPHORESIS

1959 • 1,397 citations

Structural model of ATP-binding proteing associated with cystic fibrosis, multidrug resistance and bacterial transport

1990 • 1,149 citations

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

1991 • 1,108 citations

CYSTIC FIBROSIS OF THE PANCREAS AND ITS RELATION TO CELIAC DISEASE

1938 • 1,087 citations

In vivo transfer of the human cystic fibrosis transmembrane conductance regulator gene to the airway epithelium

1992 • 953 citations

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

1992 • 937 citations

Targeted integration of adeno-associated virus (AAV) into human chromosome 19.

1991 • 774 citations

Chloride impermeability in cystic fibrosis

1983 • 750 citations

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

1990 • 724 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

Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel

1991 • 658 citations

Increased Bioelectric Potential Difference across Respiratory Epithelia in Cystic Fibrosis

1981 • 578 citations

Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer

1990 • 576 citations

Phosphorylation-regulated CI− channel in CHO cells stably expressing the cystic fibrosis gene

1991 • 564 citations

Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation.

1986 • 561 citations

Generation of cAMP-Activated Chloride Currents by Expression of CFTR

1991 • 550 citations

Nucleoside triphosphates are required to open the CFTR chloride channel

1991 • 520 citations

Cystic fibrosis: a disease in electrolyte transport

1990 • 510 citations

Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance

1991 • 508 citations

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

1991 • 498 citations

Activation by Extracellular Nucleotides of Chloride Secretion in the Airway Epithelia of Patients with Cystic Fibrosis

1991 • 495 citations

Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

1983 • 493 citations

Defective acidification of intracellular organelles in cystic fibrosis

1991 • 490 citations

ABNORMAL ELECTROLYTE COMPOSITION OF SWEAT IN CYSTIC FIBROSIS OF THE PANCREAS

1953 • 488 citations

Positional cloning: Let's not call it reverse anymore

1992 • 485 citations

Congenital Bilateral Absence of the Vas Deferens

1992 • 362 citations

A Pilot Study of Aerosolized Amiloride for the Treatment of Lung Disease in Cystic Fibrosis

1990 • 348 citations

Expression and characterization of the cystic fibrosis transmembrane conductance regulator

1990 • 340 citations

Regulation of Plasma Membrane Recycling by CFTR

1992 • 339 citations

In vivo cell-specific expression of the cystic fibrosis transmembrane conductance regulator

1991 • 327 citations

Defective beta adrenergic response of cystic fibrosis sweat glands in vivo and in vitro.

1984 • 296 citations

Localization of the cystic fibrosis transmembrane conductance regulator in pancreas.

1991 • 296 citations

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

1991 • 271 citations

Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel.

1991 • 251 citations

Abnormal regulation of ion channels in cystic fibrosis epithelia

1990 • 236 citations

A Preliminary Study of Aerosolized Recombinant Human Deoxyribonuclease I in the Treatment of Cystic Fibrosis

1992 • 203 citations

Cl- channel activity in Xenopus oocytes expressing the cystic fibrosis gene.

1991 • 196 citations

Cystic Fibrosis Gene Mutation in Two Sisters with Mild Disease and Normal Sweat Electrolyte Levels

1991 • 117 citations

Correction of the Apical Membrane Chloride Permeability Defect in Polarized Cystic Fibrosis Airway Epithelia Following Retroviral-Mediated Gene Transfer

1992 • 97 citations

Biochemical characterization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis epithelial cells.

1992 • 94 citations

Localization and Induced Expression of Fusion Genes in the Rat Lung

1991 • 80 citations

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

1991 • 72 citations

Cyclic AMP-dependent protein kinase activation of cystic fibrosis transmembrane conductance regulator chloride channels in planar lipid bilayers.

1992 • 61 citations

The CF Gene Product as a Member of a Membrane Transporter (TM6-NBF) Super Family

1991 • 21 citations

Translationally regulated genes in higher eurkaryotes

1992 • 2 citations

Smith's anesthesia for infants and children, 5th ed. Edited by Etsuro K. Motoyama (editor) and Peter J. Davis (associate editor). St. Louis: C.V. Mosby Co., 1990, 948 pp

1991 • 2 citations

Selected abstract

1991 • 1 citations