Fig. 1
From: Loss of CREST leads to neuroinflammatory responses and ALS-like motor defects in mice
The ALS Mutation Q388X Leads to Instability of CREST Protein In Vitro and In Vivo. (a and b) Protein levels of WT and Q388X mutant form of CREST in primary cortical neurons infected with lentivirus expressing HA tagged CREST WT or Q388X cDNA respectively, treated with cycloheximide (CHX, 20μg/ml) for 0h, 3h, 6h, 12h, measured by immunoblot a and quantification b Relative expression represents the ratios of HA (upper panel) and Actin (lower panel) band intensities determined by Image J. c The schematic illustration for partial alignment of human CREST protein with mouse CERST protein, showing the conserved position of ALS mutation (Q388X) and the corresponding site in mouse CREST protein (Q394X). (d) Strategies of the constructing CREST knockout (KO) and CREST Q394X mutation mice using CRISPR/Cas9 technology. (e) Body size of a Crest −/− mouse compared to Crest +/− and WT littermates at P14. (f and g) Endogenous protein levels of CREST in the frontal cortices (f) and the lumbar spinal cords (g) from 6-month-old Q394X mice and WT littermates (n = 6, each phenotype). Error bars represent SEM. *p < 0.05, **p < 0.01, Student’s t test