The DOI matches its Crossref record; 2 of the 2 references it cites were found in a registry or an index.
The Short Apical Membrane Half-life of Rescued ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Results from Accelerated Endocytosis of ΔF508-CFTR in Polarized Human Airway Epithelial Cells
Open accessAbstract
The most common mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene in individuals with cystic fibrosis, DeltaF508, causes retention of DeltaF508-CFTR in the endoplasmic reticulum and leads to the absence of CFTR Cl(-) channels in the apical plasma membrane. Rescue of DeltaF508-CFTR by reduced temperature or chemical means reveals that the DeltaF508 mutation reduces the half-life of DeltaF508-CFTR in the apical plasma membrane. Because DeltaF508-CFTR retains some Cl(-) channel activity, increased expression of DeltaF508-CFTR in the apical membrane could serve as a potential therapeutic approach for cystic fibrosis. However, little is known about the mechanisms responsible for the short apical membrane half-life of DeltaF508-CFTR in polarized human airway epithelial cells. Accordingly, the goal of this study was to determine the cellular defects in the trafficking of rescued DeltaF508-CFTR that lead to the decreased apical membrane half-life of DeltaF508-CFTR in polarized human airway epithelial cells. We report that in polarized human airway epithelial cells (CFBE41o-) the DeltaF508 mutation increased endocytosis of CFTR from the apical membrane without causing a global endocytic defect or affecting the endocytic recycling of CFTR in the Rab11a-specific apical recycling compartment.
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Citations
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
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
Cited by
Bomberger, J. M., MacEachran, D. P., Coutermarsh, B. A., Ye, S., O'Toole, G. A., & Stanton, B. A. (2009) · PLoS Pathogens · doi:10.1371/journal.ppat.1000382 · Cited by 614
Self-citationCFTR Function and Prospects for Therapy
Riordan, J. R. (2008) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.75.103004.142532 · Cited by 610
New insights into cystic fibrosis: molecular switches that regulate CFTR
Guggino, W. B., & Stanton, B. A. (2006) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm1949 · Cited by 432
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Metadata
- Authors
- Swiatecka-Urban, A., Brown, A., Moreau-Marquis, S., Renuka, J., Coutermarsh, B., Barnaby, R., Karlson, K. H., Flotte, T. R., Fukuda, M., Langford, G. M., & Stanton, B. A.
- Venue
- Journal of Biological Chemistry
- Publisher
- Elsevier BV
- Published
- 2005-11
- Volume
- 280
- Issue
- 44
- Pages
- 36762-36772
- ISSN
- 0021-9258 (ISSN-L)
- OpenAlex
- W2115617146
- Cited by
- 184
- References
- 2
- FWCI
- 8.63
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Swiatecka-Urban, A., Brown, A., Moreau-Marquis, S., Renuka, J., Coutermarsh, B., Barnaby, R., Karlson, K. H., Flotte, T. R., Fukuda, M., Langford, G. M., & Stanton, B. A. (2005). The Short Apical Membrane Half-life of Rescued ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Results from Accelerated Endocytosis of ΔF508-CFTR in Polarized Human Airway Epithelial Cells. Journal of Biological Chemistry. https://doi.org/10.1074/jbc.m508944200
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