Fig. 3

Enhanced SOC entry in GMSLNs differentiated from HD iPSCs. a SOC currents amplitudes recorded in whole-cell experiments as a function of time after application of Tg (1 μM) to HD GMSLNs differentiated from iPSHD11, iPSHD22, and iPSHD34 lines, or WT GMSLNs differentiated from IPSRG2L, endo-iPS12, and hESM01 lines. Current amplitudes for all groups were measured every 10 s at a test potential of –80 mV. Each plot shows mean ± SEM. b Average current-voltage (I-V) curves of currents evoked by passive depletion of calcium stores with Tg (1 μM) in GMSNLNs differentiated from HD iPSCs or WT PSCs. The I-V curves were recorded after full development of the SOC currents. Each trace represents the average of several experiments. c, d Average current-voltage (I-V) curves of currents evoked by passive depletion of calcium stores with Tg (1 μM). The I-V curves were recorded after full development of the SOC currents. Each trace represents the average of several experiments. c WT GMSNLNs infected with mutant HTTQ138-1exon or control HTTQ15-1exon. d In HD GMSNLNs transfected with LNA(T) specifically blocking mHTT expression or control LNA(S). e Average SOC current amplitude in GMSNLNs differentiated from HD iPSCs or WT PSCs. f Average amplitude of SOC currents in GMSLNs differentiated from HD iPSCs (black symbols), WT iPSCs (red symbols), WT ESCs (light magenta symbols); in WT GMSNLNs infected with mutant HTT138Q-1exon (dark green symbols) or control HTT15Q-1exon (orange symbols); in HD GMSNLNs transfected with LNA(T) (olive symbols) or control LNA(S) (green symbols). e, f For all groups, current amplitude was determined at a test potential of –80 mV and plotted as the mean ± SEM (n = number of single cell experiments)