Fig. 1

TDP-43 immunohistochemistry of cerebellum sections of wt and Npc1 ^−/− PN11 mice (a). At higher magnification (bottom panels), immunohistochemistry revealed a decrease of TDP-43 nuclear expression in GNs of both external granule layer (EGL) and internal granule layer (IGL), and in Purkinje cells (arrows) of Npc1 ^−/− mice compared to wt. b Western blot analysis of TDP-43 protein levels in nuclear and cytoplasmic cerebellar fractions, identified by Sp1 and β-III-tubulin, respectively. Histograms indicate nuclear TDP-43 abundance (mean ± SEM), determined by densitometry of protein bands obtained in at least 3 independent experiments (having at least three replicates for each data point) taking Sp1 as internal reference. ** indicates P < 0.01 in Student’s t-test