Results 81 to 90 of about 4,681 (114)
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Matrine modulates HSC70 levels and rescues ΔF508‐CFTR
Journal of Cellular Physiology, 2012AbstractCystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP‐dependent Cl− channel located in the plasma membrane, and its malfunction results in cystic fibrosis (CF), the most common lethal genetic disease in Caucasians. Most CF patients carry the deletion of Phe508 (ΔF508 mutation); this mutation prevents the delivery of the CFTR to ...
BASILE, ANNA +7 more
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Impact of interruption of CFTR modulator therapies
Journal of Cystic FibrosisNovel drug therapy targeting the defective cystic fibrosis transmembrane conductance regulator protein has the potential to significantly enhance the quality of life for numerous patients with cystic fibrosis. However, in some countries social insurance does not pay for modulators because these drugs are extremely expensive.
Burcu, Capraz Yavuz +12 more
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CFTR Modulators for the Gastroenterologist
Journal of Pediatric Gastroenterology and Nutrition, 2023Sabina, Sabharwal, Sarah Shrager, Lusman
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Expanding CFTR Modulator Testing to Carriers of CFTR Variants
Annals of the American Thoracic Society, 2021Preston E, Bratcher, Pamela L, Zeitlin
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CFTR modulates lung secretory cell proliferation and differentiation
American Journal of Physiology-Lung Cellular and Molecular Physiology, 2000We have permanently reversed the lethal phenotype in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR)-deficient (knockout) mouse after in utero gene therapy with an adenovirus containing the cftr gene. The gene transfer targeted somatic stem cells in the developing lung and intestine, and these epithelial surfaces demonstrated ...
J E, Larson +4 more
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CFTR is a modulator of airway inflammation
American Journal of Physiology-Lung Cellular and Molecular Physiology, 2007although it has been 17 years since the identification of the cystic fibrosis transmembrane ion receptor (CFTR) gene and protein, it remains enigmatic how abnormalities in CFTR can cause chronic and persistent pulmonary infection and inflammation that lead to bronchiectasis and end-stage ...
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CFTR Modulator Therapies in Cystic Fibrosis
2017Cystic fibrosis (CF), the most common life-shortening autosomal recessive disorder in Caucasians, results from mutations in the gene that encodes the cystic fibrosis transmembrane-conductance regulator (CFTR) protein. Nearly 2000 CFTR mutations have been identified as being associated with disease, with known effects of a given mutation on protein ...
David R. Spielberg +2 more
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PULMONOLOGY: CFTR modulators for cystic fibrosis
Journal of the American Academy of Physician Assistants, 2013Erica, Bertoncini, Dawn, Colomb-Lippa
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Simplifying CFTR modulation therapy in cystic fibrosis
Archives of Disease in Childhood, 2023openaire +2 more sources