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. 2024 Jul 22;88:101995. doi: 10.1016/j.molmet.2024.101995

Figure 3.

Figure 3

SFD iRPE cells display dysregulated central carbon metabolism A, Fibroblasts from SFD patients with the S204C mutation were de-differentiated into iPSCs and then differentiated into iPSC-RPE cells (iRPE). Cells were expanded until passage 3 and then cultured for a minimum 30 days. Relative intracellular and extracellular (from conditioned media) metabolites were quantified with GC/MS. B–N, Relative abundance of total metabolite levels were compared between SFD iRPE (SFD) and Crispr corrected controls (Control) (n = 25 for all metabolites except for αKG where n = 18). O, Extracellular glucose concentration in conditioned media was quantified after 24 h using an enzymatic assay (n = 6). P, relative extracellular metabolites levels featuring metabolites that were decreased after 24 h compared to time 0 (i.e. consumed). P. metabolites that were increased after 24 h compared with time 0 (i.e. produced) (n = 25). Statistics performed were two-tailed unpaired Mann–Whitney. ∗p < 0.05, ∗∗p < 0.01, ∗∗∗p < 0.001, ∗∗∗∗p < 0.0001. TCA cycle schematic was generated using BioRender. Graphs were made with GraphPad Prism.