Fig. 5
From: Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation
hFUS transgene activates autoregulatory splicing in Fus∆NLS/+ spinal cord. A-D: RT-qPCR results for endogenous Fus mRNA deleted of exon 7 (A), endogenous Fus mRNA retaining intron 6 (B), endogenous Fus mRNA retaining intron 7 (C) and exogenous FUS mRNA retaining intron 7 (D) in spinal cord at 1 month (left) or 22 months (right) of age. Note that the hFUS transgene activates autoregulatory exon 7 skipping as well as retentions of introns 6 and 7 in endogenous mRNA and retention of intron 7 in exogenous mRNA at 1 and 22 months of age. N = 4–8. *, p < 0.05, **p < 0.01, ***p < 0.001 vs. Fus+/+, #, p < 0.05, ###, p < 0.001 vs. indicated genotype by ANOVA followed by Tukey. E-H: representative gel electrophoresis of RT-PCR assays identifying RNA species with or without intron 6 retention (E, G, upper panel), with or without intron 7 retention (E, G, middle panel), or with or without exon 7 skipping (E, G, lower panel) in spinal cord at 1(E) or 22 (G) months of age. We did not detect exon 7 skipped mRNA using these assays. Panels F and H show the percentage of intron 6 or 7 retention (intron + band intensity divided by the sum of intensities of intron + and intron – bands, multiplied by 100), for 1 month (F) or 22 months (H) of age. N = 4–8. ***p < 0.001 vs. Fus+/+, #, p < 0.05, ##, p < 0.01, ###, p < 0.001 vs. indicated genotype by ANOVA followed by Tukey