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SPX-101 Is a Novel Epithelial Sodium Channel-targeted Therapeutic for Cystic Fibrosis That Restores Mucus Transport.
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Osmotic stimuli increase clearance of mucus in patients with mucociliary dysfunction.
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New pulmonary therapies directed at targets other than CFTR.
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Therapeutic options for hydrating airway mucus in cystic fibrosis.
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Mucus clearance and lung function in cystic fibrosis with hypertonic saline.
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Mendelian randomization analysis of two samples to determine the impact of immune cells on bronchitis.
Medicine (Baltimore). 2024 Nov 29;103(48):e40541. doi: 10.1097/MD.0000000000040541.
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Recent Advances in the Development of Novel Drug Candidates for Regulating the Secretion of Pulmonary Mucus.
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Nebulized hypertonic saline in noncystic fibrosis bronchiectasis: a comprehensive review.
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2
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
N Engl J Med. 2015 Jul 16;373(3):220-31. doi: 10.1056/NEJMoa1409547. Epub 2015 May 17.
3
Duration of action of hypertonic saline on mucociliary clearance in the normal lung.
J Appl Physiol (1985). 2015 Jun 15;118(12):1483-90. doi: 10.1152/japplphysiol.00404.2014. Epub 2015 Apr 24.
4
The Relationship of Mucus Concentration (Hydration) to Mucus Osmotic Pressure and Transport in Chronic Bronchitis.
Am J Respir Crit Care Med. 2015 Jul 15;192(2):182-90. doi: 10.1164/rccm.201412-2230OC.
5
Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation.
J Cyst Fibros. 2014 Dec;13(6):674-80. doi: 10.1016/j.jcf.2014.09.005. Epub 2014 Sep 26.
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Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.
Sci Transl Med. 2014 Jul 23;6(246):246ra97. doi: 10.1126/scitranslmed.3008889.
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Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.
Sci Transl Med. 2014 Jul 23;6(246):246ra96. doi: 10.1126/scitranslmed.3008680.
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Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure.
J Clin Invest. 2014 Jul;124(7):3047-60. doi: 10.1172/JCI73469. Epub 2014 Jun 2.

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