SRP54 mutant induce ER-stress and autophagy. mRNA expression levels of (A) ATF4, CHOP, and spliced XBP1 for ER stress and (B) ULK1 for autophagy were examined by qRT-PCR related to PPIA and HPRT at days (D) 7, 10, and 14 of culture of control (n = 3-4) and patient (n = 2-4) cells. Mean ± SEM; unpaired t test, 2-tailed on controls vs patients combing all days, *P < .05. mRNA expression levels of (C) CHOP and (D) ULK1 were also checked after sorting CD36⁻CD15⁺CD11b⁻ and CD15⁺CD11b⁺CD36⁻ granulocytic cells between days 10 and 14. Controls (n = 3) and patients (n = 3); mean ± SEM; unpaired t test, 2-tailed on controls vs patients; *P < .05; **P < .01. (E) Using primary fibroblasts, autophagy was evaluated by the lipidation of LC3 (LC3-II) by western blot analysis in controls (n = 2) and SRP54-mutated patients (n = 8). β-Actin was used as a loading control. Fold induction of LC3-II/β-Actin was quantified using Image J software.