In Vitro Characterization of Motor Neurons and Purkinje Cells Differentiated from Induced Pluripotent Stem Cells Generated from Patients with Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay

Figure 4

Assessment by Western blot analysis of DRP1 and sacsin expression levels in MNs and Purkinje cells differentiated from healthy and ARSACS iPSCs cultured in 2D and 3D. Expression levels of DRP1 and sacsin were assessed by Western blot analysis on MNs and Purkinje cells differentiated from 3 healthy control iPSCs (C1, C2, C3) and 3 ARSACS iPSCs (A1, A2, A3) cultured in 2D and 3D, as well as on fibroblasts. DRP1 and sacsin were, respectively, normalized against actin and vinculin to control for equal loading quantification. A statistical analysis was performed using 1-way ANOVA nonparametric test, ().