175 6 iPSC-derived liver organoids as a tool to study Medium Chain Acyl-CoA Dehydrogenase deficienc Figure 2. MCADD EHO organoids display an MCADD-characteristic phenotype in culture. (A) Representative immunoblot images illustrating the lack of MCAD protein in MCADD organoids; data represents 2 MCADD lines and 2 control lines. (B) Intracellular triglyceride levels in control and MCADD organoids. On the left organoids were stimulated with bovine serum albumin (BSA). The graph on the right depicts the values after treating the organoids with 0.5mM of BSA-Palmitate; data represents the mean of 6 biological replicates ± SEM (Unpaired two-tailed t-test) (C) Oxygen consumption rate in organoids measured at state 3 (stimulated with ADP, octanoyl carnitine and malate) and uncoupled (state U); Data represents the mean of 5 biological replicates ± SEM (Unpaired two-tailed t-test). (D) Upper panel: medium chain acyl-carnitines measured in the organoids incubated with palmitate and L-carnitine for 24 hours (C6, C8 and C10) and C8/C10 ratio; data represents 6 biological replicates from independent cultures ± SEM. Lower panel: Medium-chain acylcarnitine measured in supernatant (C6, C8 and C10) and C8/ C10 ratio. Data represents 6 biological replicates from independent cultures ± SEM. (*P<0.05, **P<0.01, ***P<0.001, ****P<0.0001 one-tailed unpaired t test). Maturation of EHOs into Mat-EHOs increases peroxisome abundance Although peroxisomes are specialized in their ability to oxidize very-longchain, branched and dicarboxylic fatty acids, they are also capable of oxidizing
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