Fig. 2
From: Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation
hFUS transgene corrects abnormal levels of FUS protein in Fus∆NLS mice. A: Immunoblot analysis of FUS protein in total extracts from Fus+/+ (+/+) and FusΔNLS/+ (∆/+) brains with or without hFUS transgene and of FusΔNLS/∆NLS mice (∆/∆) with hFUS transgene at 22 months of age. Representative results using different antibodies revealing total FUS, the C-terminal (C-ter) NLS, mouse FUS, and human FUS. Vinculin was used as loading controls. Note that these immunoblots were performed on different membranes to avoid cross reaction between different antibodies and one representative Vinculin blot is shown. Uncropped western blots and their corresponding Vinculin western blots are provided in Source data. B: Quantification of immunoblotting experiments of panel A. Quantification of total, C-ter, mouse, human and ADMA-FUS protein levels in cytoplasmic and nuclear fractions of the indicated genotypes. N = 4–8. *p < 0.05, ***p < 0.001vs Fus+/+, #, p < 0.05 and ###, p < 0.001 vs. indicated genotype by ANOVA followed by Tukey. C: Immunoblot analysis of FUS protein subcellular localization in cortex of Fus+/+ (+/+) and FusΔNLS/+ (∆/+) mice with or without hFUS transgene and of FusΔNLS/∆NLS mice (∆/∆) with hFUS transgene at 1 month of age. Representative results using different antibodies revealing total FUS, the C-terminal (C-ter) NLS, mouse FUS, human FUS and asymmetrically dimethylated arginine FUS (ADMA-FUS). SOD1 and HDAC1 are used as loading controls for cytoplasmic and nuclear protein extracts fractions, respectively. Note that these immunoblots were performed on different membranes to avoid cross reaction between different antibodies. Uncropped western blots and corresponding stain free gels are provided in Source data. D: Quantification of western blotting experiments of panel C. Quantification of total, C-ter, mouse, human and ADMA-FUS protein levels in cytoplasmic and nuclear fractions of the indicated genotypes. N = 4–8. *p < 0.05, ***p < 0.001vs Fus+/+, #, p < 0.05 and ###, p < 0.001 vs. indicated genotype by ANOVA followed by Tukey