Results 41 to 50 of about 4,639 (201)

Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression. [PDF]

, 2014
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane regulator (CFTR) that result in reduced anion conductance at the apical membrane of secretory epithelia.
Apaja, Pirjo M., Avramescu, Radu G., Bagdany, Miklos, Borot, Florence, Finkbeiner, Walter E., Hegedus, Tamas, Lukacs, Gergely L., Perdomo, Doranda, Phuan, Puay-Wah, Szollosi, Daniel, Veit, Guido, Verkman, Alan S., Wu, Yu-Sheng   +12 more
core   +1 more source

Paediatrics: messages from Munich. [PDF]

, 2015
The aim of this article is to describe paediatric highlights from the 2014 European Respiratory Society (ERS) International Congress in Munich, Germany.
Albuali, Alexopoulos, Andersson, Anton-Pacheco, Arigliani, Arumugam, Balfour-Lynn, Baraldi, Barikbin, Bekhof, Benseler, Berankova, Bilton, Bon, Boon, Boudewyns, Brinkman, Casas, Chiu, Constant, Conway, Dassios, DeBoer, Duijts, Elborn, Engelkes, Engelkes, Everard, Fainardi, Fouzas, Frankenberger, Gadomski, Gilchrist, Goksör, Goussard, Goutaki, Gray, Grosse-Onnebrink, Hatziagorou, Henckel, Hirst, Honková, Iglesias, Iglesias, Ingham, Ioan, Jedynak-Wasowicz, Jung, Kirkby, Kobbernagel, Koschlig, Krivec, Loza, Luciani, Lukkarinen, Madkour, Maguire, Mandadzhieva, Marshall, Maurer, Meurling, Moreno, Moreno, Mulder, Murray, Murray, Mustafayev, Mutti, Naehrig, Ohn, Olsson, Ong, Onland, Parekh, Pie tropaoli, Popovic, Pramana, Ramsey, Rosenow, Rossor, Rubio, Schoegler, Schoegler, Schultz, Serio, Smit, Smyth, Stern, Stokholm, Su, Svedberg, Thouvenin, Tiwari, Umutoglu, Uyan, Van Bragt, Van der Schee, Waddingham, Yacob, Yammine   +99 more
core   +11 more sources

The TAK1→IKKβ→TPL2→MKK1/MKK2 Signaling Cascade Regulates IL-33 Expression in Cystic Fibrosis Airway Epithelial Cells Following Infection by Pseudomonas aeruginosa [PDF]

, 2016
In cystic fibrosis (CF), chronic respiratory infections result in an exaggerated and uncontrolled inflammatory response that ultimately lead to a decrease in pulmonary function.
Raquel Farias, Simon Rousseau
core   +2 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

Efficient, non‐toxic anion transport by synthetic carriers in cells and epithelia [PDF]

, 2016
Transmembrane anion transporters (anionophores) have potential for new modes of biological activity, including therapeutic applications. In particular they might replace the activity of defective anion channels in conditions such as cystic fibrosis ...
A Vargas Jentzsch, Anthony P. Davis, AP Davis, AS Verkman, AV Koulov, B Shen, BA McNally, BC Pressman, BD de Grenu, BW Ramsey, CA Lipinski, CE Wainwright, CJ Dutton, CW Jiang, DA Stoltz, David N. Sheppard, DN Sheppard, DP Wallace, EWFW Alton, FM Ashcroft, G McLachlan, GR Cutting, GW Gokel, H Li, H Valkenier, H Valkenier, Hazel A. Sparkes, Hennie Valkenier, Hongyu Li, JA Cooper, James A. Cooper, JL Sessler, JL Sessler, JP Clare, JR Broughman, JR Broughman, JT Davis, JT Davis, LJV Galietta, Luke W. Judd, LVJ Galietta, MA Mall, N Busschaert, N Busschaert, Ondřej Jurček, PA Gale, Peter R. Brotherhood, PR Brotherhood, S Hussain, Sabir Hussain, SJ Edwards, SJ Moore, SK Ko, V Sidorov, X Li   +54 more
core   +2 more sources

Role of the SLC26A9 chloride channel as disease modifier and potential therapeutic target in cystic fibrosis [PDF]

, 2018
The solute carrier family 26, member 9 (SLC26A9) is an epithelial chloride channel that is expressed in several organs affected in patients with cystic fibrosis (CF) including the lungs, the pancreas, and the intestine. Emerging evidence suggests SLC26A9
Balázs, Anita, Mall, Marcus A.
core   +1 more source

Impact of cystic fibrosis transmembrane conductance regulator modulator therapies on liver stiffness and liver enzymes: An observational perspective single‐center cohort study

JPGN Reports, EarlyView.
Abstract Objectives The efficacy of cystic fibrosis transmembrane conductance regulator (CFTR)‐modulator therapies in preventing or ameliorating cystic fibrosis liver disease (CFLD) by correcting CFTR in cholangiocytes is not well‐documented. This study aimed to assess liver function during CFTR‐modulators.
Laura Giugliano, Elisabetta Augustoni, Antonio Pizzol, Anna Opramolla, Cristina Chiadò, Anna Zagaria, Irene Esposito, Giulia Roberto, Alice Ponte, Andrea Evangelista, Paola Quarello, Michele Pinon, Pier Luigi Calvo   +12 more
wiley   +1 more source

Improved Quality of Life in Children With Cystic Fibrosis Who Received Transmembrane Conductance Regulator Modulators

Acta Paediatrica, EarlyView.
ABSTRACT Aim Children with cystic fibrosis (CF) face substantial daily treatment burdens and the effects of transmembrane conductance regulator modulators on these have not been sufficiently described. We evaluated changes in treatment burden after elexacaftor tezacaftor ivacaftor (ETI) was initiated.
Marcus Svedberg, Frida Lundqvist, Huda Abdulahi Östrand, Christina Krantz, Per Ekman, Henrik Imberg   +5 more
wiley   +1 more source

Anion-transport mechanism of a triazole-bearing derivative of prodigiosine: a candidate for cystic fibrosis therapy [PDF]

, 2018
Cystic fibrosis (CF) is a genetic lethal disease, originated from the defective function of the CFTR protein, a chloride and bicarbonate permeable transmembrane channel.
Baroni, Debora, Caci, Emanuela, Capurro, Valeria, Cossu, Claudia, Fiore, Michele, García Valverde, María, Moran, Óscar, Quesada Pato, Roberto   +7 more
core   +2 more sources

Challenges to Assessing the Prevalence of Cystic Fibrosis in the Caribbean

Pediatric Pulmonology, Volume 61, Issue 5, May 2026.
ABSTRACT Cystic fibrosis (CF) is likely underdiagnosed in Caribbean populations due to non‐representative cystic fibrosis transmembrane conductance regulator (CFTR) variant screening panels, limited newborn screening programs, and structural healthcare barriers.
Krystal L. Rivera‐Figueroa, Cole L. Martin, Christina A. Le, Wilfredo De Jesús Rojas, Leandra Cordero Oñate, Stephen G. Aller   +5 more
wiley   +1 more source