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Summary. CFTR modulator therapy targets the protein defective in CF and boosts its function, but the drug must match mutation pathobiology. Ivacaftor, a CFTR potentiator, was the first modulator approved in 2012, with impressive improvement in lung function and other measures of disease in patients with gating and other residual function mutations (∼10% of CF patients).
Jul 17, 2014 · Once the CaCC is fully developed (<5 min), CFTR inh -172 (A) or GlyH-101 (B) were perfused at increasing concentrations (1, 5, 10 µM). The membrane potential was held at −40 mV and currents were elicited by a train of 12 voltage steps (400-ms duration) between −100 and +120 mV in +20 mV increments.
Jun 2, 2022 · Singh AK, Fan Y, Balut C, Alani S, Manelli AM, Swensen AM, et al. Biological characterization of F508delCFTR protein processing by the CFTR Corrector ABBV-2222/GLPG2222. J Pharmacol Exp Ther. 2020 ...
Mar 26, 2001 · Cystic fibrosis (CF) is a multisystem disease affecting epithelia of the respiratory tract, exocrine pancreas, intestine, hepatobiliary system, and exocrine sweat glands. Morbidities include recurrent sinusitis and bronchitis, progressive obstructive pulmonary disease with bronchiectasis, exocrine pancreatic deficiency and malnutrition, pancreatitis, gastrointestinal manifestations (meconium ...
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Apr 5, 2020 · CFTR testing is not recommended as routine screening for men with other categories of infertility. While available CFTR panels include 30 to 96 of the most common variants, complete gene sequencing should be considered if there is a high index of suspicion in a high-risk couple (e.g., partner is CFTR mutation carrier).
May 21, 2022 · Introduction. Cystic fibrosis (CF) is an autosomal recessive monogenic disease that affects more than 90,000 people worldwide. It constitutes the most common life-limiting genetic disease in the Caucasian population. 1 –3 The clinical manifestations of CF are caused by a defect in the cystic fibrosis transmembrane regulator (CFTR) protein, a chloride channel that is widely distributed on ...
