Figure 5.

F508del-CFTR correction in CF-KM4 cells restored histamine ER Ca²⁺ release compared to non CF MM39 cells. A mRNA amplification of 3 isoforms of IP₃R by real time PCR in MM39 and CF-KM4 cells. B Example of line-scan images acquired in MM39 cells and in uncorrected or corrected CF-KM4 cells in absence of extracellular Ca²⁺. These cells were incubated 2 h at 37°C with 100 μM miglustat or 100 μM NB-DGJ. C Average of the line-scan images in A expressed as normalized fluorescence in each conditions. D Histogram of the normalized area under curve of intensity profile of Ca²⁺ response extracted from A in various experimental conditions as indicated. E Mean of amplitude of Ca²⁺ response in each experimental condition. Results are presented as mean ± SEM and the number of experiments is noted on each bar graph. *** P < 0.001; ns, non significant difference.