Organoid cultures of MELAS neural cells reveal hyperactive Notch signaling that impacts neurodevelopment

摘要

, MELAS iPSCs express OCT4 and NANOG mRNA transcripts equivalent to that expressed by wild-type BJ-iPS and isogenic control c-MELAS iPSCs. Schematic of differentiation of iPSCs into neural progenitors (by day 10) and eventually into motor neurons (by day 28). , Immunostaining analyses of day 10 neural progenitors derived from BJ-iPS, c-MELAS and MELAS iPSCs, demonstrating efficient derivation of neural progenitors expressing SOX1, Ki67 and Nestin. Quantification of cells coexpressing SOX1 and Ki67 in respective cultures. Scale bars indicate 100 μm.