Correction of lung inflammation in a F508del CFTR murine cystic fibrosis model by the sphingosine-1-phosphate lyase inhibitor LX2931

Progressive lung disease with early onset is the main cause of mortality and morbidity in cystic fibrosis patients. Here we report a reduction of sphingosine-1-phosphate (S1P) in the lung of unchallenged Cftr F508del CFTR mutant mice. This correlates with enhanced infiltration by inducible nitric ox...

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Veröffentlicht in:	American journal of physiology. Lung cellular and molecular physiology 2016-11, Vol.311 (5), p.L1000-L1014
Hauptverfasser:	Veltman, Mieke, Stolarczyk, Marta, Radzioch, Danuta, Wojewodka, Gabriella, De Sanctis, Juan B, Dik, Willem A, Dzyubachyk, Oleh, Oravecz, Tamas, de Kleer, Ismé, Scholte, Bob J
Format:	Artikel
Sprache:	eng
Schlagworte:	Aldehyde-Lyases - antagonists & inhibitors Aldehyde-Lyases - metabolism Animals Biological Transport - drug effects Body Weight - drug effects Cystic Fibrosis - diagnostic imaging Cystic Fibrosis - drug therapy Cystic Fibrosis - pathology Cystic Fibrosis Transmembrane Conductance Regulator Cytokines - metabolism Dendritic Cells - drug effects Dendritic Cells - metabolism Disease Models, Animal Enzyme Inhibitors - pharmacology Enzyme Inhibitors - therapeutic use Epithelial Cells - drug effects Epithelial Cells - metabolism Imidazoles - pharmacology Imidazoles - therapeutic use Lipopolysaccharides - pharmacology Lung - drug effects Lung - metabolism Lysophospholipids - metabolism Mice, Inbred C57BL Mucin 5AC - metabolism Mutation - genetics Myeloid Cells - drug effects Myeloid Cells - metabolism N-Formylmethionine Leucyl-Phenylalanine - pharmacology Oximes - pharmacology Oximes - therapeutic use Pneumonia - diagnostic imaging Pneumonia - drug therapy Pneumonia - pathology Salivary Glands - drug effects Salivary Glands - metabolism Sphingosine - analogs & derivatives Sphingosine - metabolism X-Ray Microtomography
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