Results 171 to 180 of about 1,459,532 (261)

Editorial: Real-world experience with CFTR modulator therapy. [PDF]

Front Pharmacol, 2023
Tümmler B, Burgel PR.
europepmc   +1 more source

Monosodium glutamate‐mediated Ca2+‐dependent intestinal epithelial ion transports in health and IBS‐D in male mice

Physiological Reports, Volume 14, Issue 11, June 2026.
Abstract Despite the ubiquitous use of monosodium glutamate (MSG) as a food additive, its specific effects on intestinal health and disease remain largely unclear. Here we found mucosal application of MSG induced an upward intestinal short‐circuit current (Isc) in the murine ileum, which was significantly reduced by extracellular Cl−, Na+, and Ca2 ...
Yunxian Wang, Fenglan Chu, Yanhua Gong, Ruihong Guo, Hui Dong   +4 more
wiley   +1 more source

Modulator therapies for cystic fibrosis

, 2019
\ua9 2019 Elsevier Ltd Cystic fibrosis (CF) is a life-limiting genetic disease that arises from defects in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and protein. This causes abnormal epithelial ion transport. CF is a multisystem
Gardner AI, Brodlie M, Parameswaran MC, Gonzalez-Ciscar A, Socas A, Abidin NZ, Haq IJ   +6 more
core  

Modulator Therapy for Cystic Fibrosis: An Exploration of Current Research

, 2020
Developing a drug therapy that addresses the root cause of cystic fibrosis (CF) by increasing CFTR protein levels has long been a research challenge. After genetic therapy failed because a suitable delivery system could not be found, researchers began ...
Rombocos, Jessalyn
core  

Increased aminotransferases do not necessarily mean hepatotoxicity of CFTR modulator therapy. [PDF]

Clin Exp Hepatol, 2023
Woynarowski M, Sapiejka E, Jóźwiak M, Wierzbicka-Rucińska A.   +3 more
europepmc   +1 more source

Molecular and functional characterisation of rare CFTR variants

Cystic fibrosis (CF) is caused by mutations in the CFTR gene. The CFTR gene encodes for the CFTR protein, which is an ion channel and indirectly responsible for fluid transport in various organs.
Lefferts, Juliet Willemijn
core  

CFTR modulator response plots colored by AlphaMissense pathogenicity score reveals little predictive capabilities of AM in theratyping.

A. Basal mature CFTR (C band) to immature CFTR (B band) trafficking (C-B ratio) in percent WT versus modulator enhanced C/B ratio in percent WT from the Bihler et al. study [33]. Error bars were excluded for clarity.
Jonathan P. Schlebach (1326678), Kathryn E. Oliver (17847260), Lars Plate (1265706), Jens Meiler (112401), Eli Fritz McDonald (7382066)   +4 more
core   +1 more source

Impact of CFTR modulator concentrations on clinical response in cystic fibrosis. [PDF]

Eur Respir J
Chalamalla AR, Baker E, Ryan KJ, Dowell A, Natt JR, Zemanick ET, Konstan MW, Mayer-Hamblett N, Acosta EP, Guimbellot JS, CHEC-SC investigators.   +10 more
europepmc   +1 more source

Predictive capacity of paediatric nasal epithelial cells in sequential CFTR modulator therapy. [PDF]

Thorax
Fawcett LK, Chew ZA, Schneider-Futschik EK, Allan KM, Patel HR, Jaffe A, Waters S.   +6 more
europepmc   +1 more source

Elexacaftor/tezacaftor/ivacaftor efficacy in intestinal organoids with rare CFTR variants in comparison to CFTR-F508del and CFTR-wild type controls

Cystic fibrosis is a life-shortening genetic disease caused by pathological variants of the cystic fibrosis transmembrane conductance regulator gene. The CFTR modulator therapy elexacaftor, tezacaftor and ivacaftor (ETI) rescues CFTR protein function and
Muilwijk, Danya, van der Ent, Cornelis K., Bierlaagh, Marlou C., Alghisi, Federico, Niemöller, Paul J., Verburg, Tibo F., Beekman, Jeffrey M., Viscomi, Carla, Cutrera, Renato, Lefferts, Juliet W., Kroes, Suzanne, Fiocchi, Alessandro G., Lucidi, Vincenzina, Boni, Alessandra, Keijzer-Nieuwenhuijze, Natascha D.A., Ciciriello, Fabiana, Cristiani, Luca   +16 more
core