Fig. 6.

Lowering STAU1 restores PCP2 expression in SCA2 cells. a Normal [ATXN2-Q22/22] and SCA2 [ATXN2-Q22/52] LBCs were electroporated with STAU1 siRNA and STAU1 levels were determined by western blotting 4 days post-electroporation. b Quantification of three independent experiments of the blot in a. c Matched cultures were used for qRT-PCR analyses to measure PCP2 transcript levels. STAU1 depletion is associated with increased PCP2 transcript levels in SCA2 LBCs. PCP2 mRNA is unchanged upon STAU1 knockdown in normal LBCs. STAU1 silencing does not alter ATXN2 transcript steady-state levels in normal or SCA2 LBCs. d–i ATXN2 siRNA lowers STAU1 levels and increases PCP2 expression in SCA2 cells. d SCA2 LBCs [ATXN2-Q22/52] were electroporated with siATXN2 and evaluated by western blotting. e Quantification of three independent experiments of the blot in d. f Matched cultures were used for qRT-PCR analyses to measure PCP2 transcript levels, demonstrating PCP2 expression increased with siATXN2 dose. g–i As for d–f but using SCA2 FBs [ATXN2-Q22/42]. β-Actin and GAPDH RNA were used as internal controls for western blot and qRT-PCR analyses, respectively. Abundances relative to β-Actin determined densitometrically. Data are mean ± SD, ns = P > 0.05, **P < 0.01, ***P < 0.001, Student t-test