Modulating Cftr activity restores lumen size in myo1d MZ mutant embryos. a, b Knockdown of myo1d in TgBAC(Cftr-GFP) embryos did not affect CFTR apical GFP localization (n?=?6 control MO, n?=?6 myo1d MO). c?e Activation of Cftr function in the absence of myo1d MZ mutant expanded lumen area. Representative Z-stack image showing that Forskolin and IBMX (FIBMX) increased KV lumen in WT and myo1d MZ embryos (c). Treating with ouabain, an inhibitor of Na+/K+ ATPase decreased the KV lumen area in WT and myo1d mutant embryos. Numeration of lumen area with treatments are shown in (WT-DMSO: n?=?8, WT-ouabain: n?=?10, WT-FIBMX: n?=?4, myo1d MZ-DMSO): n?=?19, myo1d MZ-ouabain: n?=?7, myo1d MZ-FIBMX: n?=?12) (d). Cardiac looping defects were increased in WT embryos treated with FIBMX or ouabain but did not increase in myo1d MZ mutants (e). Unpaired t test and one-way ANOVA were conducted. *p?<?0.05, ***p?<?0.001, ****p?<?0.0001 represents statistical significance. ns not significant. Data shown are mean?±?SEM. L lumen, scale bar?=?15?µm (a), 50?µm (c)
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