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Fig. 4 | Acta Neuropathologica Communications

Fig. 4

From: The ESCRT-III protein VPS4, but not CHMP4B or CHMP2B, is pathologically increased in familial and sporadic ALS neuronal nuclei

Fig. 4

Overexpression of a dominant negative VPS4 increases nuclear POM121 spots but does not restore their distribution in C9orf72 and sALS iPSNs. a, c Maximum intensity projections from SIM imaging of VPS4 (a) and POM121 (c) in nuclei isolated from control, C9orf72, and sALS iPSNs overexpressing GFP or GFP tagged VPS4 variants. Overexpression as indicated on left, genotype and antibody as indicated on top. Arrows (c) indicate uneven distribution of POM121 observed following overexpression of dominant negative VPS4 (VPS4E228Q) in ALS nuclei. b, d Quantification of VPS4 (b) and POM121 (d) spots. n = 4 control, 4 C9orf72, and 4 sALS iPSC lines, 50 GFP + nuclei per line/overexpression. Two-way ANOVA with Tukey’s multiple comparison test was used to calculate statistical significance. *p < 0.05, **p < 0.01, ****p < 0.0001. Scale bar = 5 μm

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