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 5

Formation of NFM aggregates in neurites from MNs and Purkinje cells differentiated from ARSACS iPSCs cultured in 3D. Cells were seeded onto the sponge containing fibroblasts during a total in vitro differentiation of 53 days for Purkinje cells and 74 days for MNs. The 3D model was imaged with a view from above after staining of cells for NFM in green (a–h). Neurites elongated from MNs and Purkinje cells differentiated from the healthy control into MNs (a) or Purkinje cells (e) and were compared to 3 ARSACS iPSC cell lines (A1, A2, A3) differentiated into MNs (b–d) or Purkinje cells (f–h). NFM accumulation was highlighted in white squares enlarged in the bottom right corner of (a)–(h). Scale bars: 20 μm.