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Fig. 3 | Cell & Bioscience

Fig. 3

From: Pharmacological activation of ATF6 remodels the proteostasis network to rescue pathogenic GABAA receptors

Fig. 3

AA147 and AA263 reduce the degradation of the α1(D219N) subunit. A Effect of AA147 (5 µM, 24 h) and AA263 (5 µM, 24 h) on the degradation of the α1(D219N) subunit in HEK293T cells stably expressing α1(D219N)β2γ2 GABAA receptors using cycloheximide (CHX)-chase analysis. B Effect of AA147 (5 µM, 24 h) and AA263 (5 µM, 24 h) on steady-state protein levels of ERAD factors, including GRP94, VCP, HRD1, and SEL1L in HEK293T cells stably expressing α1(D219N)β2γ2 GABAA receptors. Quantification of the normalized band intensities is shown on the right (n = 3). C Effect of AA147 (5 µM, 24 h) and AA263 (5 µM, 24 h) on the interactions between α1(D219N) subunits and ERAD factors in HEK293T cells expressing α1(D219N)β2γ2 receptors. Quantification of the ratio of the target proteins and α1(D219N) post immunoprecipitation (IP) is on the right (n = 3). Each data point is reported as mean ± SEM. For statistical analysis, two-tailed Student’s t-test was used in (A), whereas one-way ANOVA followed by post-hoc Tukey test was used in B and C. *, p < 0.05; **, p < 0.01; ***, p < 0.001

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