Nitric oxide has no beneficial effects on ion transport defects in cystic fibrosis human nasal epithelium

2000 ◽  
Vol 441 (1) ◽  
pp. 133-137 ◽  
Author(s):  
Claudia Rückes-Nilges ◽  
H. Lindemann ◽  
Thorsten Klimek ◽  
Hiltrud Glanz ◽  
Wolf-Michael Weber
Keyword(s):  
Nitric Oxide ◽  
Cystic Fibrosis ◽  
Ion Transport ◽  
Nasal Epithelium ◽  
Beneficial Effects ◽  
Human Nasal Epithelium

scholarly journals Assessing the residual CFTR gene expression in human nasal epithelium cells bearing CFTR splicing mutations causing cystic fibrosis

2013 ◽  
Vol 22 (6) ◽  
pp. 784-791 ◽  
Author(s):  
Laia Masvidal ◽  
Susana Igreja ◽  
Maria D Ramos ◽  
Antoni Alvarez ◽  
Javier de Gracia ◽  
...  
Keyword(s):  
Gene Expression ◽  
Cystic Fibrosis ◽  
Cftr Gene ◽  
Nasal Epithelium ◽  
Human Nasal Epithelium ◽  
Splicing Mutations ◽  
Epithelium Cells

Effect of nitric oxide on epithelial ion transports in noncystic fibrosis and cystic fibrosis human proximal and distal airways

2012 ◽  
Vol 303 (7) ◽  
pp. L617-L625 ◽  
Author(s):  
Sabine Blouquit-Laye ◽  
Luc Dannhoffer ◽  
Camille Braun ◽  
Anh-Tuan Dinh-Xuan ◽  
Edouard Sage ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Cystic Fibrosis ◽  
Ion Transport ◽  
Soluble Guanylate Cyclase ◽  
Short Circuit ◽  
Primary Cultures ◽  
Airway Epithelia ◽  
Ussing Chambers ◽  
Intracellular Cgmp ◽  
Bronchial Cells

The airways of patients with cystic fibrosis (CF) exhibit decreased nitric oxide (NO) concentrations, which might affect airway function. The aim of this study was to determine the effects of NO on ion transport in human airway epithelia. Primary cultures of non-CF and CF bronchial and bronchiolar epithelial cells were exposed to the NO donor sodium nitroprusside (SNP), and bioelectric variables were measured in Ussing chambers. Amiloride was added to inhibit the Na+channel ENaC, and forskolin and ATP were added successively to stimulate cAMP- and Ca2+-dependent Cl−secretions, respectively. The involvement of cGMP was assessed by measuring the intracellular cGMP concentration in bronchial cells exposed to SNP and the ion transports in cultures exposed to 1H-[1,2,4]oxadiazolo-[4,3-a]quinoxalin-1-one, an inhibitor of the soluble guanylate cyclase (ODQ), or to 8Z, a cocktail of 8-bromo-cGMP and zaprinast (phosphodiesterase 5 inhibitor). SNP decreased the baseline short-circuit current ( Isc) and the changes in Iscinduced by amiloride, forskolin, and ATP in non-CF bronchial and bronchiolar cultures. The mechanism of this inhibition was studied in bronchial cells. SNP increased the intracellular cGMP concentration ([cGMP]i). The inhibitory effect of SNP was abolished by 2-phenyl-4,4,5,5-tetramethylimidazoline-1-oxyl-3-oxide, an NO scavenger (PTIO) and ODQ and was partly mimicked by increasing [cGMP]i. In CF cultures, SNP did not significantly modify ion transport; in CF bronchial cells, 8Z had no effect; however, SNP increased the [cGMP]i. In conclusion, exogenous NO may reduce transepithelial Na+absorption and Cl−secretion in human non-CF airway epithelia through a cGMP-dependent pathway. In CF airways, the NO/cGMP pathway appears to exert no effect on transepithelial ion transport.

Cystic fibrosis and non-cystic-fibrosis human nasal epithelium show analogous Na + absorption and reversible block by phenamil

1997 ◽  
Vol 434 (1) ◽  
pp. 19-24 ◽  
Author(s):  
Ulrike Blank ◽  
Claudia Rückes ◽  
Wolfgang Clauss ◽  
Thomas Hofmann ◽  
Hermann Lindemann ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Nasal Epithelium ◽  
Human Nasal Epithelium

scholarly journals Interleukin-13 stimulates production of nitric oxide in cultured human nasal epithelium

2018 ◽  
Vol 54 (3) ◽  
pp. 200-204 ◽  
Author(s):  
Johnny L. Carson ◽  
Michelle Hernandez ◽  
Ilona Jaspers ◽  
Katherine Mills ◽  
Luisa Brighton ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Nasal Epithelium ◽  
Interleukin 13 ◽  
Human Nasal Epithelium

scholarly journals Single-Dose Lentiviral Mediated Gene Therapy Recovers CFTR Function in Cystic Fibrosis Knockout Rats

2021 ◽  
Vol 12 ◽  
Author(s):  
Nicole Reyne ◽  
Patricia Cmielewski ◽  
Alexandra McCarron ◽  
Juliette Delhove ◽  
David Parsons ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Gene Therapy ◽  
Ion Transport ◽  
Rat Model ◽  
Nasal Epithelium ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Epitope Tag ◽  
Cf Transmembrane Conductance Regulator ◽  
First Time

Cystic fibrosis (CF) is a genetic disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, resulting in defective ion transport in the airways. Addition of a functioning CFTR gene into affected airway cells has the potential to be an effective treatment for lung disease. The therapeutic efficacy of airway gene transfer can be quantified in animal models by assessing ion transport in the treated nasal epithelium using the nasal potential difference (PD) measurement technique. The nasal PD technique is routinely used in CF mice, however when applied to a recently developed CF rat model those animals did not tolerate the initial nasal PD assessment, therefore the procedure was firstly optimised in rats. This study evaluated the effect of lentiviral (LV)-mediated CFTR airway gene delivery on nasal PD in a CFTR knockout rat model. LV gene vector containing the CFTR gene tagged with a V5 epitope tag (LV-V5-CFTR) was delivered to the nasal epithelium of CF rats, and one week later nasal PD was analysed. This study demonstrated for the first time that LV-V5-CFTR treatment produced a mean correction of 46% towards wild-type chloride response in treated CF rats. Transduced cells were subsequently identifiable using V5 immunohistochemical staining. These findings in the nose validate the use of airway gene therapy for future lung based experiments.

Minor Role of Cl– Secretion in Non-Cystic Fibrosis and Cystic Fibrosis Human Nasal Epithelium

1999 ◽  
Vol 9 (1) ◽  
pp. 1-10 ◽  
Author(s):  
Claudia Rückes-Nilges ◽  
Ulrike Weber ◽  
Hermann Lindemann ◽  
Gerd Münker ◽  
Wolfgang Clauss ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Nasal Epithelium ◽  
Minor Role ◽  
Human Nasal Epithelium

scholarly journals Apical CFTR Expression in Human Nasal Epithelium Correlates with Lung Disease in Cystic Fibrosis

PLoS ONE ◽  
2013 ◽  
Vol 8 (3) ◽  
pp. e57617 ◽  
Author(s):  
Marit Arianne van Meegen ◽  
Suzanne Willemina Julia Terheggen-Lagro ◽  
Kirsten Judith Koymans ◽  
Cornelis Korstiaan van der Ent ◽  
Jeffrey Matthijn Beekman
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Nasal Epithelium ◽  
Human Nasal Epithelium
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