Disruption of Intracellular Calcium Homeostasis Leads to ERLIN2-Linked Hereditary Spastic Paraplegia in Patient-Derived Stem Cell Models

Figure 2

The differentiation from iPSC into motor neurons. (a) Schematic diagram of the process of motor neuron differentiation. iPSC = induced pluripotent stem cells; NSCs = neural stem cells; NPCs = neural progenitor cells; MN = motor neurons. (b) Various cell morphologies at the differentiation stage of motor neurons. (c) iPS-GDL as the WT, iPS-GJH as the p.Val71Ala, iPSC-induced NSCs were immunofluorescence stained with SOX2, and the number of SOX2-positive cells was calculated (, images, experiments, ). (d) iPSC-induced NPCs were immunofluorescence stained with HB9. The number of HB9-positive cells and dead cells was calculated (, images, experiments, ). (e) Immunofluorescence staining of motor neurons on day 21 of maturation showed that the number of MNs was smaller and the axon length of motor neurons was significantly shorter in the p.Val71Ala group than in the WT group. The green was β-III-tubulin, and the blue was DAPI (, images, experiments, ).

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