Results 11 to 20 of about 71,874 (264)

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
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CFTR mutations altering CFTR fragmentation [PDF]

Biochemical Journal, 2012
Most CF (cystic fibrosis) results from deletion of a phenylalanine (F508) in the CFTR {CF transmembrane-conductance regulator; ABCC7 [ABC (ATP-binding cassette) sub-family C member 7]} which causes ER (endoplasmic reticulum) degradation of the mutant. Using stably CFTR-expressing BHK (baby-hamster kidney) cell lines we demonstrated that wild-type CTFR ...
TOSONI, KENDRA, Michelle Stobbart, D.i.a.n.e. .M. Cassidy, VENERANDO, ANDREA, PAGANO, MARIO ANGELO PRIMO, Simão Luz, M.a.r.g.a.r.i.d.a. .D. Amaral, Karl Kunzelmann, PINNA, LORENZO, C.a.r.l.o.s. .M. Farinha, Anil Mehta   +10 more
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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

Cftr [PDF]

The Journal of General Physiology, 2001
Some studies of CFTR imply that channel activation can be explained by an increase in open probability (Po), whereas others suggest that activation involves an increase in the number of CFTR channels (N) in the plasma membrane. Using two-electrode voltage clamp, we tested for changes in N associated with activation of CFTR in Xenopus oocytes using a ...
Xuehong Liu, Stephen S. Smith, Fang Sun, David C. Dawson   +3 more
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CFTR structure [PDF]

Journal of Cystic Fibrosis, 2018
Structural studies of the cystic fibrosis transmembrane conductance regulator (CFTR) protein are critical to understand molecular mechanisms involved in gating of the apical anion channel as well as the way in which the gating is regulated, especially by the regulatory region (R region).
Isabelle, Callebaut, P Andrew, Chong, Julie D, Forman-Kay   +2 more
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CFTR and TNR-CFTR expression and function in the kidney [PDF]

Biophysical Reviews, 2014
The cystic fibrosis transmembrane conductance regulator (CFTR) is abundantly expressed in the kidney. CFTR mRNA is detected in all nephron segments of rats and humans and its expression is higher in the renal cortex and outer medulla than in the inner medulla.
Jackson, Souza-Menezes, Geórgia, da Silva Feltran, Marcelo M, Morales   +2 more
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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

Mechanisms of endothelial cell dysfunction in cystic fibrosis [PDF]

, 2017
Although cystic fibrosis (CF) patients exhibit signs of endothelial perturbation, the functions of the cystic fibrosis conductance regulator (CFTR) in vascular endothelial cells (EC) are poorly defined.
Anile, Marco, Cianci, Eleonora, Del Porto, Paola, Dell'Elba, Giuseppe, Di Silvestre, Sara, Evangelista, Virgilio, Guarnieri, Simone, Lanuti, Paola, Marchisio, Marco, Mari, Veronica C., Mariggiã², Maria A., Moretti, Paolo, Mucilli, Felice, Pandolfi, Assunta, Patruno, Sara, Piccoli, Antonio, Plebani, Roberto, Prioletta, Marco, Recchiuti, Antonio, Romano, Mario, Sacchetti, Silvio, Totani, Licia, Venuta, Federico   +22 more
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New era of cystic fibrosis: full mutational analysis and personalized therapy [PDF]

, 2017
Despite its apparently simple genetics, cystic fibrosis (CF) is a rather complex genetic disease. A lot of variability in the steps of the path from the cystic fibrosis transmembrane conductance regulator (CFTR ) gene to the clinical manifestations ...
Lucarelli, Marco
core   +1 more source

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