Fig. 1: PolyQ expansion promotes TBP to preferentially form aggregates in the cerebellum. | Nature Communications

Fig. 1: PolyQ expansion promotes TBP to preferentially form aggregates in the cerebellum.

From: Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17

Fig. 1

a A schematic diagram of AAV plasmids expressing human TBP with different polyQ repeat lengths. b Western blot analysis of HEK293 cells transfected with AAV-TBP plasmids confirming the expression of TBP with different polyQ repeats (13Q, 44Q, 68Q, and 105Q). Vinculin was used as a loading control. c A diagram of stereotaxic injection of AAV-TBP into the cerebellum, striatum, and prefrontal cortex in 3-month-old wild-type mice. d–f TBP immunofluorescent staining of the cerebellum (d), striatum (e), and prefrontal cortex (f) from AAV-TBP-injected mouse brains. Anti-1TBP18 antibody was used. Enlarged images in the boxed areas were also presented. Red arrows indicate aggregates. Scale bar = 20 μm. g Quantification of the accumulated TBP aggregates in the cerebellum, striatum, and prefrontal cortex. One-way ANOVA followed with Tukey’s multiple comparisons test was performed; cerebellum, F = 1491; striatum, F = 311.6; prefrontal cortex, F = 354.6, ****P < 0.0001. n = 4 mice per group, three images per brain region from each mouse were used to count. Data are represented as mean ± SEM. Source data and full blots are provided as a Source Data file.

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