Skip to main content
Fig. 1 | Molecular Neurodegeneration

Fig. 1

From: Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation

Fig. 1

hFUS transgene rescues lethality and motor deficits in Fus∆NLS mice. A: Scheme of the breeding strategy. B: Representative genotyping results of 5 mice at 1 month of age. C-D: Kaplan Meier survival curve of the different genotypes either homozygous (C) or heterozygous for the ∆NLS mutation (D). Note that all Fus∆NLS/∆NLS mice die at birth, unless carrying a hFUS transgene. *, p < 0.05 Log Rank test; ***, p < 0.001 Log rank test. E: Age-dependent changes in the mean hanging time (s) and holding impulse (Ns) in the four-limb wire inverted grid test in Fus+/+ (+/+), and FusΔNLS/+ (∆/+) mice with or without hFUS transgene. N = 10–28 per group. Mixed effect analysis, with 3 factors (Age, ∆NLS genotype and hFUS genotype). P < 0.001 for ∆NLS genotype, P < 0.001 for age, P < 0.001 for hFUS genotype. A significant protective interaction is observed between ∆NLS and hFUS genotypes (p = 0.0216, and p = 0.0366). Only 4 groups out of 5 are shown here for clarity. The whole dataset is shown in Fig S1. F: Hindlimb grip strength in female and male mice. Mixed effect analysis, with 3 factors (Age, ∆NLS genotype and hFUS genotype). For female mice, P < 0.001 for ∆NLS genotype, p = ns for age, p = ns for hFUS genotype. A significant protective interaction is observed between ∆NLS and hFUS genotypes (p = 0.0131). For male mice, P < 0.001 for ∆NLS genotype, p = ns for age, p = ns for hFUS genotype. No significant protective interaction is observed between ∆NLS and hFUS genotypes (p = 0.0512)

Back to article page