Chloride conductance expressed by ΔF508 and other mutant CFTRs in xenopus oocytes

Mitchell L. Drumm, Daniel J. Wilkinson, Lisa S. Smit, Roger T. Worrell, Theresa V. Strong, Raymond A. Frizzell, David C. Dawson, Francis S. Collins

Research output: Contribution to journalArticle

394 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1797-1799
Number of pages3
JournalScience
Volume254
Issue number5039
DOIs
StatePublished - Jan 1 1991

ASJC Scopus subject areas

  • General

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    Drumm, M. L., Wilkinson, D. J., Smit, L. S., Worrell, R. T., Strong, T. V., Frizzell, R. A., Dawson, D. C., & Collins, F. S. (1991). Chloride conductance expressed by ΔF508 and other mutant CFTRs in xenopus oocytes. Science, 254(5039), 1797-1799. https://doi.org/10.1126/science.1722350