only be successfully established beyond five passages from a subset of the BD-patient iPSC lines. Of the successfully FACS-purified NPC lines expressing CD184+, all had characteristic NESTIN+ cell morphology, proliferated in a monolayer in the presence of EGF and bFGF, and upon mitogen removal differentiated into MAP2+ and βIII-tubulin+ (Tuj1+) neurons capable of firing action potentials (Fig. 2B, C; Sup. Fig. 8). Thus, as summarized in the lineage diagram shown in Figure 2D, we were able to generate expandable (greater than five passages), FACS-immunopurified, CXCR4+ NPC lines from all six of the parental iPSC lines and only one of three iPSC lines from each BD-affected son within the Family-811 quartet.
