181 6 iPSC-derived liver organoids as a tool to study Medium Chain Acyl-CoA Dehydrogenase deficienc Figure 5. CoA biosynthesis and metabolism in Mat-EHOs. (A) Schematic depiction of the hypothesized effect of MCADD on CoA pools and compensatory mechanism from peroxisomes. In green, the targets analyzed in C-D. (B) Total coenzyme A pools in control (grey) and MCADD (red) organoids. Organoids were grown in glucose-free medium supplemented with BSA-palmitate and L-carnitine. Data represents 5 biological replicates ± SEM. (C) Relative gene expression of genes involved in CoA biosynthesis. Data represents 7 biological replicates for the control group and 5 for MCADD ± SEM. (*P<0.05, **P<0.01, two-tailed unpaired t test). (D) Relative gene expression of genes involved in CoA metabolism. For C and D, organoids were grown in glucose-free medium supplemented with BSA and L-carnitine. Data represents 7 biological replicates for the control group and 5 for MCADD ± SEM. (*P<0.05, **P<0.01, two-tailed unpaired t test).
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