Fig. 7.

GGGGCC repeat expansions exacerbate susceptibility to inhibition of the autophagy pathway. Cell viability of human neurons after exposure to chloroquine (a) and 3-methyladenine (3-MA) (b) for 24 h. Values are expressed as a percentage of the untreated cells (control) (n = 3 independent cultures) (a, b). Caspase-3-like activity after exposure to 5 mM 3-MA for 24 h (c). In all panels, values are mean ± SEM. *p < 0.05, **p < 0.01, ***p < 0.001 (One-way ANOVA). Representative Western blot showing p62 protein levels in neurons of two non-carriers (PGRN S116X: lines 1 and 26, and control: lines 17 and 20) and two repeat expansions carriers (carrier 1: lines 5 and 6, and carrier 2: lines 1 and 11) (d)