Figure 2.

Plp2 deficiency leads to increased susceptibility to ER stress. (A) WT and KO MEFs cell death assay. Data are shown as mean ± SEM; n = 6; ***P < 0.001. (B) WT and KO MEFs at passage 5 were treated with Fas ligand (2 µg/ml), Thap (10 mm) or TUN (2 µM) for 24 h. Total cells (adherent and floating) were harvested for western blot. (C) Human primary fibroblasts from two males who carry the PLP2-(−113C>A) allele and one normal control (WT) were exposed to TUN (20 µg/ml) and Thap (10 µM) for 48 h. 200× light microscopy. (D) Human primary fibroblasts from two males who carry PLP2-(−113C>A) allele and three normal males (WT) were treated with VP-16 (200 µM), Thap (10 µM) and Fas ligand (0.1 µg) + cycloheximide (20 µg/ml) for 48 h. The range was indicated with the error bar (***P < 0.001). (E) Flp-In Trex 293-PLP2 cells were cultured in medium with or without doxycyclin (1 µg/ml) for 24 h. Western blotted with an antibody specific for PLP2. (F) Flp-In Trex 293-PLP2 cells cultured in medium with (blue, PLP2 over-expressing) or without doxycycline (red, non-PLP2 over-expressing) were incubated in media containing various apoptotic inducing agents at the indicated doses for 24 h. Data are shown as mean ± SEM; n = 3; *P < 0.05; **P < 0.01.