Fig. 6

EIF4H is downregulated in C9+ ALS/FTD. a. Western immunoblots were used to define changes in eIF4B or eIF4H protein levels from 5 independent control or 4 independent C9+ derived fibroblast cell lines. Data are relative to controls. Quantification of total protein was done after normalizing to loading, using GAPDH. Phospho-eIF4B quantification was further normalized to total eIF4B. Statistics: unpaired student t-tests. Shown: each data point represents 1 cell line with mean ± SEM; the mean data from 2 independent protein preparations is shown per line. b. RNA levels of EIF4B or EIF4H were assessed by qPCR in human cerebellar tissue from healthy individuals or ALS/FTD patients with (C9+) or without (C9-) the G4C2 expansion in C9orf72. n = 22 (healthy), 46 (C9- ALS/FTD), 66 (C9+ ALS/FTD). Statistics: one-way ANOVAs with Dunn’s multiple comparison correction. Shown: individual data points representing 1 individual with mean ± SEM. Cell line details: Additional file 3: Table S3. Patient details: Additional file 4: Table S4. p-values **** < 0.0001, *** < 0.001, ** < 0.01, * < 0.05, no significance > 0.05