A small molecule neutrophil elastase inhibitor, KRP-109, inhibits cystic fibrosis mucin degradation

Abstract Background Neutrophil elastase (NE) rapidly degrades gel-forming airway mucins in cystic fibrosis (CF) sputum. We hypothesized that KRP-109, a small molecule NE inhibitor, would inhibit CF mucin degradation in vitro. Methods Sputa were collected from CF patients ( n = 5) chronically or inte...

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Veröffentlicht in:	Journal of cystic fibrosis 2016-05, Vol.15 (3), p.325-331
Hauptverfasser:	Chillappagari, Shashi, Müller, Christian, Mahavadi, Poornima, Guenther, Andreas, Nährlich, Lutz, Rosenblum, Jonathan, Rubin, Bruce K, Henke, Markus O
Format:	Artikel
Sprache:	eng
Schlagworte:	Airway Obstruction - metabolism Airway Obstruction - physiopathology Benzoxazines - metabolism Cystic fibrosis Cystic Fibrosis - complications Cystic Fibrosis - metabolism Humans Leukocyte Elastase - antagonists & inhibitors Leukocyte Elastase - metabolism Mucin Mucins - analysis Mucins - metabolism Mucociliary Clearance Protease and anti-protease balance Pseudomonas aeruginosa - isolation & purification Pseudomonas aeruginosa - physiology Pseudomonas Infections - diagnosis Pseudomonas Infections - etiology Pulmonary/Respiratory Pyrrolidines - metabolism Respiratory Tract Infections - diagnosis Respiratory Tract Infections - etiology Small molecule inhibitors Spectrophotometry - methods Sputum - metabolism Sputum - microbiology
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