Results 21 to 30 of about 81,604 (317)

Mucin glycosylation and sulphation in airway epithelial cells is not influenced by cystic fibrosis transmembrane conductance regulator expression [PDF]

, 2005
Abnormalities in mucus properties and clearance make a major contribution to the pathology of cystic fibrosis (CF). Our aim was to test the hypothesis that the defects in CF mucus are a direct result of mutations in the CF transmembrane conductance ...
Dell, Anne, Evans, Joanne, Harris, Ann, Leir, Shih-Hsing, Morris, Howard R, Palmai-Pallag, Timea, Parry, Simon   +6 more
core   +1 more source

CFTR Inhibitors

Current Pharmaceutical Design, 2013
The cystic fibrosis transmembrane conductance regulator (CFTR) protein is a cAMP-regulated Cl- channel whose major function is to facilitate epithelial fluid secretion. Loss-of-function mutations in CFTR cause the genetic disease cystic fibrosis. CFTR is required for transepithelial fluid transport in certain secretory diarrheas, such as cholera, and ...
Verkman, Alan S, Synder, David, Tradtrantip, Lukmanee, Thiagarajah, Jay R, Anderson, Marc O   +4 more
openaire   +4 more sources

CFTR

, 2021
CFTR
openaire   +2 more sources

FUNCIÓN PULMONAR EN FIBROSIS QUÍSTICA

Neumología Pediátrica, 2022
La fibrosis quística (FQ) es una enfermedad hereditaria autosómica recesiva, causada por la mutación del gen que codifica la proteína CFTR (cystic fibrosis transmembrane conductance regulator), afecta varios órganos, pero la enfermedad pulmonar es la ...
Hortensia Barrientos
doaj   +1 more source

Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype phenotype correlations [PDF]

, 1996
We have generated a mouse carrying the human G551D mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR) by a one-step gene targeting procedure.
Alton, Ewfw, Delaney, S. J., Dorin, J. R., Farley, R., Hume, D. A., Lamb, D., Lovelock, P. K., Lunn, D. P., Porteous, D. J., Smith, S. N., Thomson, S. A., Wainwright, B. J.   +11 more
core   +2 more sources

Stimulation of salivary secretion in vivo by CFTR potentiators in Cftr+/+ and Cftr−/− mice [PDF]

Journal of Cystic Fibrosis, 2008
Physiologically, salivary secretion is controlled by cholinergic and adrenergic pathways but the role of ionic channels in this process is not yet clearly understood. In cystic fibrosis (CF), most exocrine glands failed to response to beta-adrenergic agonists.To determine the implication of CFTR in this process, we measured in vivo the salivary ...
Noel, S, Strale, PO, Dannhoffer, L, Wilke, Martina, de Jonge, Hugo, Rogier, C, Mettey, Y, Becq, F   +7 more
openaire   +3 more sources

Pharmacological Responses of the G542X-CFTR to CFTR Modulators

Frontiers in Molecular Biosciences, 2022
Cystic fibrosis (CF) is a lethal hereditary disease caused by loss-of-function mutations of the chloride channel cystic fibrosis transmembrane conductance regulator (CFTR). With the development of small-molecule CFTR modulators, including correctors that facilitate protein folding and expression and potentiators that promote channel activity, about 90%
Xinxiu Fang, Jiunn-Tyng Yeh, Tzyh-Chang Hwang, Tzyh-Chang Hwang   +3 more
openaire   +3 more sources

Sinus Disease Grading on Computed Tomography Before and After Modulating Therapy in Adult Patients with Cystic Fibrosis

Journal of the Belgian Society of Radiology, 2022
Objectives: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy effects on respiratory function, pulmonary exacerbations and quality of life have been well documented. However, CFTR modulator therapy effects on sinus disease have
Corrado Tagliati, Stefano Pantano, Giuseppe Lanni, Davide Battista, Matteo Marcucci, Marco Fogante, Giulio Argalia, Enrico Paci, Gabriella Lucidi Pressanti, Mingliang Ying, Pietro Ripani   +10 more
doaj   +1 more source

Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation [PDF]

, 2014
Restoration of BECN1/Beclin 1-dependent autophagy and depletion of SQSTM1/p62 by genetic manipulation or autophagy-stimulatory proteostasis regulators, such as cystamine, have positive effects on mouse models of human cystic fibrosis (CF). These measures
Amin R, Andrea Venerando, Angela Sepe, Anil Mehta, Anna Bossi, Anna Zolin, Antonella Tosco, Boyle MP BS, Carlo A Leone, Daniela De Stefano, Donaldson S., Fabiola De Gregorio, Fischer H, Gianni Bona, Gibson LE, Giuseppe De Rosa, Guido Kroemer, Illek B, Jayaraman S, Kerem B, Kerem Eithan HMO, Laura Salvadori, Legssyer R, Lorenzo A Pinna, Luigi Maiuri, Lukacs G, Maria C Maiuri, Massimo Pettoello-Mantovani, Masvidal L, Pharmaceuticals Cylene, Pharmaceuticals Cylene, Rosa Grassia, Speranza Esposito, Standardization of Spirometry, Stefano Guido, Stern M, Valeria R Villella, Valeria Raia, van Doorninck JH, Villella VR   +39 more
core   +4 more sources

Development of CFTR structure [PDF]

Frontiers in Pharmacology, 2012
Cystic fibrosis is a lethal genetic disease caused by lack of functional cystic fibrosis transmembrane conductance regulator (CFTR) proteins at the apical surface of secretory epithelia. CFTR is a multidomain protein, containing five domains, and its functional structure is attained in a hierarchical folding process.
Patrick, Anna E., Thomas, Philip J.
openaire   +3 more sources