Results 11 to 20 of about 2,418 (178)

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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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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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
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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 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.
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The first report on the association of celiac disease and cystic fibrosis in a tertiary care center in Saudi Arabia

International Journal of Pediatrics & Adolescent Medicine, 2022
Introduction: Celiac disease (CD) has been described before in Saudi Arabia (SA) to be at the range of 1% –2% in the general population, but the association of celiac disease and cystic fibrosis (CF) has never been described before in the Middle East ...
Hanaa Banjar, Ahmed Bawazir, Firas Ghomraoui, Khalid Alotaibi, Abduallah Alotaibi, Sarah Alotaibi, Reham Sayyari, Khalid Alsaleem   +7 more
doaj   +1 more source

Analysis of polymorphic variants of CFTR (rs 113993960), IL-4 (rs 2243250), PRSS1 (rs 111033565), SPINK1 (rs ID 6690) and TNF-α (rs 1800629) Genes in Patients with Edematous Pancreatitis Living in Northern Bukovyna region

Galician Medical Journal, 2016
The occurrence of gene mutations affecting the formation of acute pancreatitis or exacerbation of chronic pancreatitis differs in different populations and ethnic groups. The objective of the research was to study the incidence of CFTR (rs 113 993 960),
Sergei Ivashchuk, Larysa Sydorchuk
doaj   +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
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