The DOI matches its Crossref record; 2 of the 2 references it cites were found in a registry or an index.
Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes
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.
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Cites
Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
Riordan, J. R., Rommens, J. M., Kerem, B.-S., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Lok, S., Plavsic, N., Chou, J.-L., et al. (1989) · Science · doi:10.1126/science.2475911 · Cited by 7,418
Self-citationIdentification of the Cystic Fibrosis Gene: Genetic Analysis
Kerem, B.-S., Rommens, J. M., Buchanan, J. A., Markiewicz, D., Cox, T. K., Chakravarti, A., Buchwald, M., & Tsui, L.-C. (1989) · Science · doi:10.1126/science.2570460 · Cited by 4,142
Cited by
ABC Transporters: From Microorganisms to Man
Higgins, C. F. (1992) · Annual Review of Cell Biology · doi:10.1146/annurev.cb.08.110192.000435 · Cited by 3,985
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
Welsh, M. J., & Smith, A. E. (1993) · Cell · doi:10.1016/0092-8674(93)90353-r · Cited by 1,485
Degradation of CFTR by the ubiquitin-proteasome pathway
Ward, C. L., Omura, S., & Kopito, R. R. (1995) · Cell · doi:10.1016/0092-8674(95)90240-6 · Cited by 1,304
Structure and Function of the CFTR Chloride Channel
SHEPPARD, D. N., & WELSH, M. J. (1999) · Physiological Reviews · doi:10.1152/physrev.1999.79.1.s23 · Cited by 1,018
New concepts of the pathogenesis of cystic fibrosis lung disease
Boucher, R. C. (2004) · European Respiratory Journal · doi:10.1183/09031936.03.00057003 · Cited by 640
Metadata
- Authors
- Drumm, M. L., Wilkinson, D. J., Smit, L. S., Worrell, R. T., Strong, T. V., Frizzell, R. A., Dawson, D. C., & Collins, F. S.
- Venue
- Science
- Published
- 1991-12-20
- Volume
- 254
- Issue
- 5039
- Pages
- 1797-1799
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2080423498
- Cited by
- 496
- References
- 2
- FWCI
- 40.62
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes. https://app.cochat.ai/papers/10.1126/science.1722350
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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. https://doi.org/10.1126/science.1722350
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