Fig. 4

Neural differentiation of PBD-ZSD-derived iPSCs. PDB_PEX1ms1 patient-derived iPSCs were immunostained and imaged during the process of differentiation into motor neurons. Images depicting the expression of a PAX6 in the neural progenitor cells, b ChAT in neurons, c TuJ1 in neurons, and d GFP transgene under the control of the HB9 enhancer in transfected motor neurons. e, f Whole cell patch clamp recording from candidate neurons derived from PBD-ZSD patient PBD_PEX1fs1 iPSCs. e Voltage-clamp records at a single patient cell, showing currents recorded at different voltages. The shapes of the curves are typical for a cell expressing sodium and potassium channels. Holding potential was −70 mV and the voltages of the steps range from −60 mV up to +60 mV, in 10-mV steps. f Current-clamp records at a single patient cell in response to current injection. Current injection triggers action potential firing, as expected for neurons