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Sildenafil (Viagra) corrects DeltaF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis

Dormer, Robert Leslie, Harris, Ceinwen Menai, Clark, Zoe Elizabeth, Pereira, Malcolm Martin Claude, Doull, Iolo John Manley, Norez, C., Becq, F. and McPherson, Margaret Ann 2005. Sildenafil (Viagra) corrects DeltaF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis. Thorax 60 (1) , pp. 55-9. 10.1136/thx.2003.019778

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Abstract

Background: Most patients with cystic fibrosis (CF) have a ?F508 mutation resulting in abnormal retention of mutant gene protein (?F508-CFTR) within the cell. This study was undertaken to investigate ?F508-CFTR trafficking in native cells from patients with CF with the aim of discovering pharmacological agents that can move ?F508-CFTR to its correct location in the apical cell membrane. Method: Nasal epithelial cells were obtained by brushing from individuals with CF. CFTR location was determined using immunofluorescence and confocal imaging in untreated cells and cells treated with sildenafil. The effect of sildenafil treatment on CFTR chloride transport function was measured in CF15 cells using an iodide efflux assay. Results: In most untreated CF cells ?F508-CFTR was mislocalised within the cell at a site close to the nucleus. Exposure of cells to sildenafil (2 hours at 37

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Subjects: R Medicine > R Medicine (General)
ISSN: 0040-6376
Last Modified: 18 Oct 2017 08:22
URI: http://orca.cf.ac.uk/id/eprint/387

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