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The impact of CFTR modulators on bone health
Pediatric Pulmonology, 2022We 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 +3 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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WNK1 and WNK4 modulate CFTR activity
Biochemical and Biophysical Research Communications, 2007The 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 +6 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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CFTR Modulator Therapies for Cystic Fibrosis
Pediatric Allergy, Immunology, and Pulmonology, 2015The 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
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Splicing Modulation as a Modifier of the CFTR Function
2006A significant fraction of CF-causing mutations affects pre-mRNA splicing. These mutations can generate both aberrant and correct transcripts, the level of which varies among different patients. An inverse correlation was found between this level and disease severity, suggesting a role for splicing regulation as a genetic modifier.
Malka, Nissim-Rafinia, Batsheva, Kerem
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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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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 Modulator Therapy for Cystic Fibrosis
New England Journal of Medicine, 2017Cystic fibrosis is a disease of abnormal ion transport through epithelium that results in progressive lung disease as well as the involvement of other organs including the pancreas, gut, and liver. Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), and inheritance is autosomal ...
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Modulators of CFTR. Updates on clinical development and future directions
European Journal of Medicinal Chemistry, 2021Anita Golec +2 more
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