Fig. 7

Overexpression of UBQLN1 reduces MN loss and TDP-43 pathology in mice expressing the P497S mutant UBQLN2 transgene. a Cresyl violet staining of the grey matter regions of the SC for the different genotypes (a–d). The magnified areas highlighted by the boxed regions are shown beneath each of them (e–h). b Quantification of the number of MN per section counted by stereology for the different genotypes (at least 3 animals for each genotype). **P < 0.01, ****P < 0.0001. c Immunofluorescence staining of TDP-43 and DAPI staining of the lumbar region of the SC for the different genotypes. Note TDP-43 staining is displaced from the nucleus to the cytoplasm in the P497S mutant Tg animal, but is restored to its typical location in the double Tg animals. The P497S section had more MN than typical and was used to better illustrate the TDP-43 pathology in the mice