Inactivation of PERK and ATF6α in neurons led to death and abnormal hindlimb clasping reflex in mice. (A) PCR analysis showed that spliced XBP1 (XBP1s) mRNA was dramatically increased in the brain of Double KO mice (lane 4) compared to WT mice (lane 1), PERK KO mice (lane 2), and ATF6 KO mice (lane 3) at PID30. (B) Body weight of WT mice (N = 8 mice), PERK KO mice (N = 11 mice), ATF6 KO mice (N = 14 mice), and Double KO mice (N = 13 mice). (C) Survival curve of WT mice, PERK KO mice, ATF6 KO mice, and Double KO mice. N = 20 mice. (D) Double KO mice developed hindlimb clasping reflexes compared to WT mice, PERK KO mice, and ATF6 KO mice. (E) The percentage of WT mice (N = 9 mice), PERK KO mice (N = 9 mice), ATF6 KO mice (N = 9 mice), and Double KO mice (N = 15 mice) developed hindlimb clasping reflex.