Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes
- 20 December 1991
- journal article
- research article
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 254 (5039) , 1797-1799
- https://doi.org/10.1126/science.1722350
Abstract
The cystic fibrosis transmembrane conductance regulator (CFTR) is associated with expression of a chloride conductance that is defective in cystic fibrosis (CF). Xenopus oocytes injected with RNA coding for CFTR that contained mutations in the first nucleotide binding fold (NBF1) expressed chloride currents in response to raising adenosine 3',5'-monophosphate (cAMP) with forskolin and 3-isobutyl-1-methylxanthine (IBMX). The mutant CFTRs were less sensitive than wild-type CFTR to this activating stimulus, and the reduction in sensitivity correlated with the severity of cystic fibrosis in patients carrying the corresponding mutations. This demonstration provides the basis for detailed analyses of NBF1 function and suggests potential pharmacologic treatments for cystic fibrosis.Keywords
This publication has 25 references indexed in Scilit:
- Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channelCell, 1991
- Phosphorylation-regulated CI− channel in CHO cells stably expressing the cystic fibrosis geneNature, 1991
- Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion SelectivityScience, 1991
- Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductanceCell, 1991
- Generation of cAMP-Activated Chloride Currents by Expression of CFTRScience, 1991
- Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cellsNature, 1990
- A frame-shift mutation in the cystic fibrosis geneNature, 1990
- Identification of the Cystic Fibrosis Gene: Chromosome Walking and JumpingScience, 1989
- Identification of the Cystic Fibrosis Gene: Genetic AnalysisScience, 1989
- Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNAScience, 1989