Figure 1

CF MDMs have a hyperinflammatory response to LPS. After 48 h pretreatment with vehicle or modulators (ivacaftor [30 nM] and lumacaftor [3 µM], VX), CF (n = 7) and non-CF (TNFα n = 5; IL-6, IL-8, and IL-1β n = 6) MDMs were exposed to vehicle or LPS for 24 h before supernatants were collected for ELISA for (a) IL-6, (b) IL-8, (c) IL-1β, and (d) TNFα. (‘*’ indicates P < 0.05, ‘**’ indicates P < 0.01). No-LPS controls were analyzed using non-parametric Kruskal–Wallis test with Dunn’s test for multiple comparisons. LPS-treated non-CF and CF groups were compared using unpaired t-tests. LPS-treated CF and LPS-treated CF with modulator pretreatment were compared using paired Wilcoxon rank sum tests. (e) Representative DIC microscopy images of non-CF (n = 2) and CF (n = 3) monocytes after differentiation with M-CSF for 1 and 7 days (d1, d7).