Fig. 3. Selective neuronal loss is also caused by endogenously expressed mutant TBP.

a Western blotting analysis of TBP expression in the cerebellum, striatum, and prefrontal cortex from 3-month-old SCA17 knock-in (KI) and wild-type (WT) mice. Vinculin was used as a loading control. b Immunofluorescent staining of Purkinje neurons with anti-calbindin antibody in the cerebellum of 5-month-old WT and KI mice. Scale bar = 100 μm. c Western blotting analysis of calbindin and NeuN levels in the cerebellum of 5-month-old WT and KI mice. The densitometric ratios of calbindin to vinculin and NeuN to vinculin were normalized to WT and analyzed with Student’s t test, t = 6.305, **P = 0.0032, n = 3 mice per group. d Western blotting of DARPP32 levels in the striatum of 5-month-old WT and KI mice. β-Actin was used as a loading control. The densitometric ratios of DARPP32 to β-actin were normalized to WT and analyzed with Student’s t test, t = 5.872, **P = 0.0011, n = 4 mice per group. e Immunofluorescent staining with anti-DARPP32 antibody in the striatum of 5-month-old WT and KI mice. Scale bar = 50 μm. Quantitative assessment of the number of medium spiny neurons labeled by DARPP32 per image field are also presented, t = 2.301, *P = 0.0352, n = 3 mice per group, three images from each mouse were used to count. f Western blotting of NeuN levels in the prefrontal cortex of 5-month-old WT and KI mice. The densitometric ratios of NeuN to vinculin were normalized to WT and analyzed with Student’s t test. n = 3 mice per group. Data are represented as mean ± SEM. Source data and full blots are provided as a Source Data file.