Fig. 1
From: Mutant UBQLN2P497H in motor neurons leads to ALS-like phenotypes and defective autophagy in rats
Restricted expression of the ALS-linked UBQLN2P497H mutation in motor neurons of rats. a A graph showing the tetracycline-regulated expression system used in this study. The transgene ChATtTA binds to the TRE promoter to induce the expression of mutant human UBQLN2P497H. The presence of Dox suppresses the expression of the transgene. b-c Immunoblotting shows that human UBQLN2 was expressed in ChATtTA/UBQLN2P497H (P497H) bigenic rats (TG: T1- T6) but not in control (ChATtTA) rats (non-TG: W1-W6). rUB2: endogenous UBQLN2, hUB2: human UBQLN2. The * indicates unknown bands. The data are reported as the mean ± standard deviation (n = 3). d-i Immunofluorescence staining shows that human UBQLN2 is substantially expressed in the spinal motor neurons of P497H but not ChATtTA rats. In addition, the accumulation of UBQLN2 promoted choline acetyltransferase (ChAT, a marker of motor neurons) to progressively form inclusions, which colocalized with UBQLN2 inclusions. In (d), a chart shows the quantification of the accumulated UBQLN2 in motor nuclei (n = 3). In (i), the arrows point to the ChAT inclusions. m: month. Scale bars: 200 μm (d); 50 μm (e-i)