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

Figure 5

Impaired proliferation and abnormal differentiation of ERLIN2 p.V71A-mutant NSCs. (a) The dot plot showed the significantly enriched categories of KEGG of the differentially expressed genes in the p.Val71Ala based on the WT. (b) The western blot showed that the protein expression levels of t-Erk1/2 and p-Erk1/2 in the MAPK signaling pathway were significantly downregulated (, experiments, ). (c) Cell proliferation was detected using the BeyoClick™ EDU-488 kit. The green fluorescence indicates cells that have the ability to proliferate, the blue fluorescence indicates the nucleus, and the bar chart shows the percentage of proliferated cells (, images, ). (d) The heat map demonstrates differentially expressed genes related to motor neuron differentiation in the p.Val71Ala group compared to the WT group, iPS-GDL as the WT, and iPS-GJH and iPS-YCY as the p.Val71Ala.

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