Figure 1
SnRNP composition and assembly in mice spinal cords are not significantly affected by hFUS overexpression. (a) RT-qPCR quantification of the indicated snRNAs in lysates from spinal cords of end-stage hFUS+/+ mice. Means ± SE were normalized for the snRNA levels in control hFUS−/− mice. At least 6 animals for each genotype were used. Values significantly different from relative controls are indicated with an asterisk when P ≤ 0.05, and two asterisks when P ≤ 0.01. (b) Expression levels of Gemin2, Sm B/B’, SMN, FUS and β-actin were assayed by Western blot on spinal cord lysates from 40 days-old mice of the indicated genotypes. The panel is representative of at least n = 4 independent experiments. Full-length blots are presented in Supplementary Figure 6. (c) The expression levels of FUS (endogenous and exogenous) and Sm B/B’ in hFUS+/+ mice were calculated by densitometric analysis of the bands from Western blots as in (b), normalised to β-actin levels and expressed as fold increases over the hFUS−/− control animals. Means ± SD are shown from n = 4 independent experiments. Values significantly different from relative controls are indicated with an asterisk when P ≤ 0.05. (d) RT-qPCR quantification of snRNAs in the Y12 anti-Sm immunoprecipitates from extracts of hFUS−/− and hFUS+/+ mice. Unrelated IgGs were used in control immunoprecipitates. Values were normalized for the RNA in the input extracts and are reported as mean ± SD. Values significantly different from relative controls are indicated with an asterisk when P ≤ 0.05, and two asterisks when P ≤ 0.01.
