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. 2023 Sep 7;32(24):3323–3341. doi: 10.1093/hmg/ddad146

Figure 6.

Figure 6

Summary of GSL and ceramide fatty acid abundance changes in iPSCs and NCCs of wildtype and GM3SD variants. The relative abundance of the major GSL types detected in iPSCs and NCCs demonstrate relatively minor differences in iPSCs but more significant changes upon differentiation to NCCs. (A) As WT iPSCs differentiate to NCCs, decreases in the relative abundance of LacCer, Gb3, and other Globo-, and Lacto-series GSLs are mirrored by increases in GM3 and other Ganglio-series GSLs. Differentiation of GM3SD variant iPSCs to NCCs is accompanied by maintenance of LacCer as a major GSL and by complete lack of Ganglio-series GSLs that depend on ST3GAL5 activity. (B) The relative abundance of the ceramide fatty acids of LacCer were quantified. Although their iPSC fatty acid profiles are quite different, as WT and p.Arg288Ter cells differentiate to NCCs, relatively minor changes are observed, especially comparing the shortest (C16:0) to the longest (C24:0, C26:0) fatty acids. The fatty acid profile of p.Glu355Lys iPSCs is similar to WT iPSCs but as the p.Glu355Lys cells differentiate to NCCs, the relative abundance of the shortest chains (C16:0) expands at the expense of the longest chain fatty acids (C24:0, C26:0).