Figure 5.

Electrophysiological analysis of control or NDUFS-4 patient iNs upon AMG treatment

(A) Scheme for electrophysiological analysis of iNs.

(B) Micrographs depicting the morphology of iNs before patch-clamp.

(C and D) Representative traces of action potential upon increasing step depolarization in control (left) or patient (right) reprogrammed neurons following AMG treatment (D) or without (C).

(E and F) Boxplots depicting the proportion of iNs spiking at least 1 (E) or 3 (F) action potentials in the indicated conditions. Each dot represents an independent batch culture. Control Ngn2+Bcl2_noAMG: n = 25 cells; control Ngn2+Bcl2_AMG: n = 25; patient Ngn2+Bcl2_noAMG: n = 15; control Ngn2+Bcl2_AMG: n = 16.

(G) Example of spontaneous synaptic activity recorded from a patient-Ngn2-Bcl2-AMG-treated neuron.

(H) Boxplot depicting the proportion of iNs showing a spontaneous synaptic activity. Each dot represents a biological replicate. Same number of cells as in (E) and (F).

(I–M) Boxplots showing input resistance (I), action potential threshold (J), action potential duration (K), action potential amplitude (L), and rise-to-fall ratio (M) in different conditions. Each dot represents one cell. Same number of cells as in (E) and (F).