Figure 5.

Hemizygous Mecp2^308/y male iPS-derived neurons show deficits in action potential characteristics, excitability and voltage-activated Na⁺ currents. (a) Neuronally differentiated male ♂ WT (Mecp2^+/y; WT ♂) and mutant (Mecp2^308/y; 308) iPS-derived cells express MAP2. Immunofluorescence analysis using a C-terminal antibody unable to detect the C-terminal truncated MeCP2³⁰⁸ protein reveals that mutant iPS cell line 308 #1 is negative for MeCP2 expression (left). In contrast, WT ♂ #11 iPS-derived neurons express MeCP2 in all MAP2+ cells (right). Scale bars: 10 μm. (b) Histogram shows average action potential amplitude for total iPS-derived cell lines of WT ♂ (grey, n=53 neurons) and 308 (black, n=88 neurons). (c) Average action potential rise time from WT ♂ iPS-derived neurons and 308 iPS-derived neurons. (d) Average input resistances of WT ♂ and 308 iPS-derived neurons. (e) The plot shows average numbers of action potentials evoked by a series of depolarizing current steps from WT ♂ and 308 iPS-derived neurons. (f) A plot of average current–voltage relationships in WT ♂ compared with 308 iPS-derived neurons. ^*P<0.05, ^**P<0.01 and ^***P<0.001.