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. 2016 Aug 30;25(21):4771–4786. doi: 10.1093/hmg/ddw304

Figure 5.

Figure 5

Enhanced ER stress in SOD1G37R female mice overexpressing CHGBL413 transgene. (A) Enhanced ER stress due to oxidized SOD1G93A by overexpression of CHGBP413L and CHGBH230R variants in cultured cells. After exposure to H2O2, the extranuclear fractions of Neuro2a cells co-transfected with FLAG-SOD1 and HA-CHGB were immunoblotted. Densitometry of the ER stress markers were analyzed. The values (mean ±SEM, N = 3) represent the ratio compared to control (Lane 1). # P < 0.05, ##P < 0.01 in post-ANOVA Turkey test. (B, C) Accumulation of misfSOD1 (C4F6 antibody) (B) and Bip (C) in motor neurons of SOD1G37R;CHGBL413 mice especially females at 345 days of age. Numbers of motor neurons high (arrows) or low (arrow heads) expressing misfSOD1 (B) and Bip (C) signals in five lumbar cord sections from each mice (N = 3-4) was analyzed. Data are mean ±SEM. Post-ANOVA Turkey test was used in each sex (same for all figures). (D) Enhanced Bip expression due to misfSOD1 accumulation (C4F6 antibody) in motor neurons of SOD1G37R;CHGBL413 mice especially females at 345 days of age. Scale bars, 100 µm. (E) High expression of Bip in SOD1G37R;CHGBL413 female mice and CHGB proteins in SOD1G37R;CHGBL413 and SOD1G37R;CHGBP413 female mice at 300 days of age. The cytosolic/microsomal fractions of spinal cords were immunoblotted and the immunoprecipitates with anti-SOD1 antibody were immunoblotted. Densitometry of Bip were analyzed. The panels have been spliced for the IP-SOD1 results of male and female samples because results were obtained from two distinct immunoprecipitation experiments for male and female samples. The values (mean ±SEM, N = 3) represent the ratio compared to control (G37R male or non-Tg (NTg) mice) (see also Supplementary Material, Fig. S5A).