ArticleProceedings of the National Academy of Sciences of the United States of America1997
In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium.
Article in Proceedings of the National Academy of Sciences of the United States of America, 1997. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
19 citing papers in PubMed, 97 citations in OpenAlex.
- A W1282X cystic fibrosis mouse allows the study of pharmacological and gene-editing therapeutics to restore CFTR function.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025Article
- Electrochemical measurement of membrane cholesterol correlates with CFTR function and is HDAC6-dependent.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2019Article
- Improved Growth Patterns in Cystic Fibrosis Mice after Loss of Histone Deacetylase 6.Scientific reports · 2017Article
- Marked repression of CFTR mRNA in the transgenic Cftr(tm1kth) mouse model.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2014Article
- PDE5 Inhibitors as Potential Tools in the Treatment of Cystic Fibrosis.Frontiers in pharmacology · 2012Article
- Activation of the cystic fibrosis transmembrane conductance regulator by the flavonoid quercetin: potential use as a biomarker of ΔF508 cystic fibrosis transmembrane conductance regulator rescue.American journal of respiratory cell and molecular biology · 2010Article
- Compartmentalized cyclic adenosine 3',5'-monophosphate at the plasma membrane clusters PDE3A and cystic fibrosis transmembrane conductance regulator into microdomains.Molecular biology of the cell · 2010Article
- INO-4995 therapeutic efficacy is enhanced with repeat dosing in cystic fibrosis knockout mice and human epithelia.American journal of respiratory cell and molecular biology · 2010Article
- Role of oxygen availability in CFTR expression and function.American journal of respiratory cell and molecular biology · 2008Article
- Generation of a conditional null allele for Cftr in mice.Genesis (New York, N.Y. : 2000) · 2008Article
- Processing and function of CFTR-DeltaF508 are species-dependent.Proceedings of the National Academy of Sciences of the United States of America · 2007Article
- Targeted therapy for cystic fibrosis: cystic fibrosis transmembrane conductance regulator mutation-specific pharmacologic strategies.Molecular diagnosis & therapy · 2006Review
- Defective respiratory amiloride-sensitive sodium transport predisposes to pulmonary oedema and delays its resolution in mice.The Journal of physiology · 2004Article
- The human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70.The Biochemical journal · 2002Article
- Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings.The Journal of clinical investigation · 2001Article
- In vivo alterations of IFN regulatory factor-1 and PIAS1 protein levels in cystic fibrosis epithelium.The Journal of clinical investigation · 2000Article
- What happens to deltaF508 in vivo?The Journal of clinical investigation · 1999Article
- DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.The Journal of clinical investigation · 1999Article
- Inducible nitric oxide synthase expression is reduced in cystic fibrosis murine and human airway epithelial cells.The Journal of clinical investigation · 1998Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
The gene causing cystic fibrosis (CF) encodes the CF transmembrane conductance regulator (CFTR), a cAMP-regulated chloride channel. Mutations in this gene result in reduced transepithelial chloride permeability across tissues affected in CF. Consequently, restoring chloride permeability to these tissues may prove therapeutic. Here we report that a combination of forskolin, an adenylate cyclase activator, and milrinone, an inhibitor of class III phosphodiesterases, increases the magnitude of the potential difference across nasal epithelium of mice homozygous for the most common CF mutation, delta F508, while neither drug alone has a significant effect on potential difference. Transgenic mice lacking CFTR do not respond to the milrinone/forskolin combination, indicating that the effect in delta F508 mice requires CFTR. These results suggest that, by pharmacological means, at least partial CFTR-mediated electrolyte transport can be restored in vivo to CF tissues expressing delta F508.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.