Our study demonstrates that the physiological maturation of oligodendrocytes from OPCs derived from human pluripotent stem cells is similar to that described for rodent models. Electrophysiological profiling of oligodendrocytes from OPCs derived from C9ORF72 patients indicates that, despite the presence of RNA foci pathology, these cell populations display maturation changes in intrinsic membrane properties and AMPAR populations similar to those seen in control lines.
