Expression of hTDP-43ΔNLS results in dramatic motor impairments, weight loss, and death in rNLS8 mice. Onset of a hindlimb clasping, b tremor, and progressive declines in c wirehang and d rotarod performance in rNLS8 mice, n = 10 controls, n = 14 bigenic. e rNLS8 mice also showed significant weight loss relative to controls beginning 3 weeks after Dox removal, n = 19 per group. f Kaplan–Meier survival curve for rNLS8 mice and littermate controls shows a marked decrease in survival time in rNLS8 mice, n = 19 per group