Article
Correction of lung inflammation in a F508del CFTR murine cystic fibrosis model by the sphingosine-1-phosphate lyase inhibitor LX2931.
American journal of physiology. Lung cellular and molecular physiology - 1 Nov 2016
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
Abstract excerpt
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 Cftrtm1EUR F508del CFTR mutant mice. This correlates with enhanced infiltration by inducible nitric oxide synthase (iNOS)-expressing granulocytes, B cells, and T cells. Furthermore, the ratio of...
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