Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in Cftr(-/-) mice.

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Physiology of the Gastrointestinal Tract.

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

1990 • 1,756 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

An Animal Model for Cystic Fibrosis Made by Gene Targeting

1992 • 921 citations

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

1991 • 495 citations

Defective acidification of intracellular organelles in cystic fibrosis

1991 • 490 citations

Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia.

1991 • 373 citations

Defective Epithelial Chloride Transport in a Gene-Targeted Mouse Model of Cystic Fibrosis

1992 • 316 citations

Altered intestinal chloride transport in cystic fibrosis

1988 • 205 citations

Association of a nonsense mutation (W1282X), the most common mutation in the Ashkenazi Jewish cystic fibrosis patients in Israel, with presentation of severe disease.

1992 • 202 citations

Synergistic action of cyclic adenosine monophosphate- and calcium-mediated chloride secretion in a colonic epithelial cell line.

1985 • 181 citations

Chloride secretory response of cystic fibrosis human airway epithelia. Preservation of calcium but not protein kinase C- and A-dependent mechanisms.

1989 • 160 citations

Intracellular Cl- activity and cellular Cl- pathways in cultured human airway epithelium

1989 • 150 citations

Failure to induce secretion in jejunal biopsies from children with cystic fibrosis.

1988 • 136 citations

Chloride secretory response to extracellular ATP in human normal and cystic fibrosis nasal epithelia

1992 • 134 citations

Shunt resistance and ion permeabilities in normal and cystic fibrosis airway epithelia

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Abnormal epithelial transport in cystic fibrosis jejunum

1991 • 64 citations

Carbachol induces oscillations of membrane potassium conductance in a colonic cell line, T84

1990 • 63 citations

Sodium- and chloride-conductive pathways in cultured mouse tracheal epithelium

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In vivo evidence of altered chloride but not potassium secretion in cystic fibrosis rectal mucosa

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(Dys)regulation of epithelial chloride channels

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Ducts of the rat pancreas in agarose matrix culture

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