Fig. 3.

Examination of PERK expression in the brain of 22q11.2DS model mouse and iPSCs. (a) Schematic diagram of preparing brain slices from adult WT and Del(3.0Mb)/+ mice. SN = substantial nigra. (b–e) Immunoblot analysis using area A and area B. Yellow arrows indicate the section used for quantification. b and c: PERK and TH were simultaneously detected, with GAPDH as the internal standard. d: Double detection of PERK and TH. e: Decreased expression of SNAP29, one of the factors in the 22q11.2 deletion region. (f–h) Quantification of the TH/GAPDH, PERK/GAPDH and PERK/TH ratios in the area A of section no. 3. f, g and h correspond to b, c and d, respectively. Quantitative detection by immunoblot was performed by Odyssey. Immunoblots were independently performed four (f and g) or six (h) times. The numbers indicated in the bars represent relative values (WT = 100). The bars represent means ± SEs. ***p < 0.001. (i) Immunoblotting for PERK, ATF6, IRE1 and β-actin using proteins extracted from iPSCs. (j) Quantification of the PERK/β-actin, ATF6/β-actin and IRE1/β-actin ratios. Two independent experiments were performed (in summary, Control: n = 6; 22DS: n = 12). The value of Control1 is 1. Each plot represents the value of each line. The bars represent means ± SEs.