Fig. 8

iPSC-derived motor neurons transduced with arginine-rich DPRs exhibit reduced nucleolus liquidity upon mutation or genetic loss of SETX. a. Here we see representative images of iPSC-MNs of the indicated genotypes transduced with lentivirus encoding NCL-mCherry and a GR(30) lentivirus vector, shown prior to photobleaching (left) and at two time points post-nucleolus photobleaching. Scale bars = 5 μM. b Fluorescence intensity measured over the first 28 s post-nucleolus photobleaching (“initial recovery”). c Percent fluorescence recovery per minute following nucleolus photobleaching over the entire period of recovery (400 s). Results are shown for n = 3 biological replicates, for n = 9–13 iPSC motor neurons per biological replicate, yielding analysis of 38–40 motor neurons per genotype. ANOVA with post-hoc Tukey test; ***P < 0.001, ****P < 0.0001. Error bars = s.e.m.