Fig. 2

G1208D-CFTR can be partially rescued by exposure to low temperature or using VX-809. a A representative blot showing the expression level and maturation status of G1208D-CFTR in HEK293 cells cultured at normal condition (37 °C) or at a low temperature (28 °C). WT-CFTR- and vector-transfected HEK293 cells were cultured at 37 °C and used as controls. b Exposure to low temperature (28 °C) promoted the maturation efficiency (1.3-fold) of G1208D-CFTR. The data were quantified from blots as represented in (a) using Image J software. *P <0.05, n = 3. c Exposure to low temperature (28 °C) increased the total CFTR protein level of G1208D (1.3-fold). The data were quantified from blots as represented in (a) using Image J software and normalized to their loading control, β-actin, respectively. *P <0.05, n = 3. d A representative blot showing the expression level and maturation status of G1208D-CFTR in HEK293 cells treated with VX-809 (5 μM) or DMSO (solvent control for VX-809). WT-CFTR- and vector-transfected HEK293 cells were used as controls. e VX-809 increased the total CFTR protein level of G1208D-CFTR (1.5-fold). The data were quantified from blots as represented in (d) using Image J software and normalized to their loading control, β-actin, respectively. *P <0.05, n = 3. f Representative iodide efflux traces of G1208D-CFTR in HEK-293 cells treated with VX-809 (5 μM) or DMSO. PKA activating agonists (10 μM forskolin, 100 μM IBMX, and 200 μM cpt-cAMP) were used to activate CFTR channel function. g The maximal iodide efflux rate of G1208D-CFTR increased 1.5-fold with VX-809 treatment compared to DMSO-treated controls. The data were quantified from experiments as represented in (f) and normalized to the total protein concentrations of cell lysates for each sample, respectively. *P <0.05, n = 9–11