Fig. 1

ISR marker protein expression is affected in VWMD fibroblasts and astrocytes. a-f Effect of MG132 stress on protein expression of the ISR markers, p-eIF2α (normalised to eIF2α), GADD34 or CHOP, at 0, 24 or 48 h incubation period. Protein levels were quantified by western blot, normalised to total protein, and are shown relative to control at time 0. Individual data points are shown from VWMD1 patient EIF2B5^R113H/A403V; VWMD6 patient EIF2B2^G200V/E213G or their non-disease controls VWMD1 Control EIF2B5^R113/A403; VWMD6 Control EIF2B2^G200V/E213, with mean ± SEM, n = 3. Significant differences between pooled disease and control groups were identified by two-way ANOVA followed by Holm-Sidak post hoc test, * p < 0.05, ** p < 0.01, *** p < 0.001. Representative blots shown in (Figure S4B)