Results 181 to 190 of about 11,406 (228)

Comparison of the Effect of CFTR Modulators <i>elexacaftor</i>/<i>tezacaftor</i>/<i>ivacaftor</i> and <i>lumacaftor</i>/<i>ivacaftor</i> via Serum Human Epididymis Protein 4 Concentration in <i>p.Phe508del-CFTR</i> Homozygous Cystic Fibrosis Patients. [PDF]

J Clin Med
Pócsi M, Fila L, Péterfia C, Halász A, Szanto TG, Mészáros B, Major J, Laki I, Szabó H, Panyi G, Balogh I, Amaral MD, Macek M, Nagy B.   +13 more
europepmc   +1 more source

Fibrose quística: etiopatogénese e novas estratégias terapêuticas [PDF]

, 2013
Ariana Helena Ribeiro de Barros
core  

Organoid-guided synergistic treatment of minimal function CFTR mutations with CFTR modulators, roflumilast and simvastatin: a personalised approach. [PDF]

Eur Respir J
Spelier S, de Winter-de Groot K, Keijzer-Nieuwenhuijze N, Liem Y, van der Ent K, Beekman J, Kamphuis LS.   +6 more
europepmc   +1 more source

Erratum: Inflammatory Activity of Epithelial Stem Cell Variants from Cystic Fibrosis Lungs Is Not Resolved by CFTR Modulators. [PDF]

Am J Respir Crit Care Med
europepmc   +1 more source

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Unmasking hereditary hemochromatosis with CFTR modulator therapy

Journal of Clinical Images and Medical Case Reports, 2022
Cystic Fibrosis (CF) is a genetic condition affecting the ability to excrete chloride, resulting in multi-system organ damage. Liver disease is one co-morbidity that affects 20-40% of people with CF. This case describes a 16-year-old male with CF (F508Del and Q493X mutations) who was started one lexacaftor/tezacaftor/ifacaftortriple CF Transmembrane ...
Richard Wong, DO, Roberts, Abigail, Michelle Condren, PharmD   +2 more
openaire   +1 more source

CFTR Modulators: Current Status and Evolving Knowledge

Seminars in Respiratory and Critical Care Medicine, 2022
AbstractIn the past decade, the medical management of people with cystic fibrosis (pwCF) has changed with the development of small molecules that partially restore the function of the defective CF transmembrane conductance regulator (CFTR) protein and are called CFTR modulators.
Lucile, Regard, Clémence, Martin, Jennifer, Da Silva, Pierre-Régis, Burgel   +3 more
openaire   +2 more sources

WNK1 and WNK4 modulate CFTR activity

Biochemical and Biophysical Research Communications, 2007
The cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-gated chloride channel. WNK kinases are widely expressed modulators of ion transport. WNK1 and WNK4, two WNK kinases that are mutated in familial hyperkalemic hypertension (FHHt), are co-expressed with CFTR in several organs, raising the possibility that WNK kinases might alter ...
Chao-Ling, Yang, Xuehong, Liu, Alex, Paliege, Xiaoman, Zhu, Sebastian, Bachmann, David C, Dawson, David H, Ellison   +6 more
openaire   +2 more sources

CFTR Modulator Therapies for Cystic Fibrosis

Pediatric Allergy, Immunology, and Pulmonology, 2015
The cloning of cystic fibrosis transmembrane conductance regulator (CFTR) set into motion a cascade of discoveries that have helped to reveal the underlying pathophysiologic basis of cystic fibrosis (CF). This discovery and the knowledge that followed have also provided the opportunity to target this basic defect, with the hope of reversing or ...
Aaron T, Trimble, Scott H, Donaldson
openaire   +2 more sources

The impact of CFTR modulators on bone health

Pediatric Pulmonology, 2022
We are hopeful that CTFR modulation does have a positive impact of bone health, but we have concerns about this study design being used to support the hypothesis.
Elizabeth, Clarke, Fiona, Moore, Andrew, Gallagher, Stephen, Thomson   +3 more
openaire   +2 more sources