Article
Alterations in blood leukocytes of G551D-bearing cystic fibrosis patients undergoing treatment with ivacaftor.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jan 2016
Bratcher Preston E, Rowe Steven M, Reeves Ginger, Roberts Tambra, Szul Tomasz, Harris William T, Tirouvanziam Rabindra, Gaggar Amit
Abstract excerpt
BACKGROUND: Ivacaftor improves clinical outcome by potentiation of mutant G551D CFTR. Due to the presence of CFTR in monocytes and polymorphonuclear neutrophils (PMNs), we hypothesized that ivacaftor may impact leukocyte activation. METHODS: We examined blood leukocytes from G551D CF subjects prior to and at one and six months after receiving ivacaftor. Blood leukocytes from ivacaftor-naïve G551D, F508del, and...
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