Function and Composition of Pulmonary Surfactant and Surfactant-Derived Fatty Acid Profiles Are Altered in Young Adults With Cystic Fibrosis

Essential genome of Pseudomonas aeruginosa in cystic fibrosis sputum

“…Notably, SCFM lacks several molecules and polymers known to be present in CF sputum including DNA (33,34), lipids (35)(36)(37), N-acetyl glucosamine (GlcNAc) (38), and mucin (39,40). To more closely approximate the contents of natural CF sputum we supplemented SCFM with concentrations of salmon sperm DNA, GlcNAc, bovine maxillary mucin, and dioleoylphosphatidylcholine (DOPC) similar to those observed in CF sputum to create SCFM2 (Dataset S8) (33)(34)(35)(36)(37)(38)(39)(40). We then investigated how closely SCFM2 approximates the nutritional environment of authentic CF sputum using Tn-seq as a test of the utility of this medium for in vitro experimentation.…”

Significance of surfactant proteins in the diagnosis of therapeutic diseases

“…Показано, что уровень SP-D в БАЛЖ больных муковисцидозом отрицательно коррелировал с марке-рами нейтрофильного воспаления [43], а уровень SP-D в сыво-ротке крови больных муковисцидозом повышен по сравнению с таковым у здоровы лиц [44].…”

Immune Dysfunction in Cystic Fibrosis

“…Defensins Impaired activity Goldman MJ et al 1997, Bals R et al 2001 Surfactant proteins Decreased or inactive Hartl D, Griese M 2006, Meyer KC et al 2000, Noah TL et al 2003 Antioxidants Reduced glutathione availability in airways Gao L et al 1999, Roum JH et al 1993, Day BJ 2005, Childers M et al 2007 Moss RB et al1996, Mueller C et al2010 IL-6 4 Increased secretion Black HR et al 1998, Becker MN et al 2004, Andersson C et al 2007, Vandivier RW et al 2009 Increased secretion Bonfield TL et al 1995, Black HR et al 1998, Becker MN et al 2004, Vandivier RW et al 2009 Altered secretion Bonfield TL et al 1995, Moss RB et al 1996, Armstrong DS et al 2005 Increased secretion Mueller C et al2010 IL-17 8 Increased secretion McAllister F et al2005, Tan HL et al2011 TNF- 9 Increased secretion Bonfield TL et al 1995, Andersson C et al 2007 Increased secretion Brennan S et al 2009 MCP-1 11 Increased secretion Brennan S et al 2009 Notes: 1 IFN-( interferon-gamma); 2 IL-1(interleukin-1 beta), 3 IL-4 (interleukin-4), 4 IL-6 (interleukin-6), 5 IL-8 (interleukin-8), 6 IL-10 (interleukin-10), 7 IL-13 (interleukin-13), [8][9][10][11][12][13][14][15][16][17], 9 TNF-...…”

“…These include the collectins and surfactant proteins , Noah TL 2003, Meyer KC 2000, defensins (Goldman MJ 1997, Bals 2001, glutathione (Gao TJ 1999, Kogan I 2003, Roum JH 1993, Hudson VM 2001 and antiproteases such as secretory leukoprotease inhibitor (SLPI) and tissue inhibitor of metalloproteinase 1 (TIMP-1) (Gaggar 2007, Cantin AM 1991, Vandivier 2002. Initially, it was thought that defensins in the CF lung were impaired due to the altered salt concentration in the CF airway (Goldmann 1997).…”

“…The Levels of -defensin were even found to be similar in bronchial brushings in CF and non CF patients (Dauletbaev N 2002). Surfactant proteins, besides their surface-tension regulating properties, also have immuno-modulatory and anti-inflammatory functions were shown to be degraded , Noah TL 2003 and structurally altered in CF (Meyer KC 2000). Glutathione, a critical component of the antioxidant defense system in the lung, was found to be reduced in the CF lung (Roum JH 1993).…”