Altered cytoskeleton dynamics in patient-derived iPSC-based model of PCDH19 clustering epilepsy.

Quantification of fluorescence intensity provided evidence of significantly increased levels of acetylated-tubulin in PCDH19mut cortical neurons when compared to control ones (** p = 0.0076, unpaired t -test, mean ± SEM, n = 9 images, N = 3 independent experiments), whereas the levels of STOP were slightly increased though such difference did not reach statistical significance ( p = 0.3600, unpaired t -test, mean ± SEM, n = 9 images, N = 3 independent experiments) ( Figure 3C ). 3.4 FRAP analyses show impairments of PCDH19mut neurites’ terminals To further investigate the details of MTs polymerization on living cells, we performed FRAP experiments using a permeant specific microtubule probe, the SiR-tubulin, to study the tubulin dynamics in control and PCDH19mut neurons, by monitoring the fluorescence recovery rate in a bleached region using time-lapse confocal microscopy.

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